Vohwinkel Syndrome vs. KID Syndrome
•Download as PPTX, PDF•
4 likes•2,621 views
Vohwinkel Syndrome vs. KID Syndrome, presented by Jennifer Armstrong. UCI Dermatology, Grand Rounds, March 30, 2011
Report
Share
Report
Share
Recommended
Neutrophilic dermatosis
1. Sweet's syndrome is an uncommon skin condition characterized by abrupt onset of tender red skin lesions and systemic symptoms. Histopathology shows a dense neutrophilic infiltrate without evidence of infection.
2. Treatment involves oral corticosteroids which provide excellent response within days to weeks. Recurrences may occur in about 30% of cases.
3. Pyoderma gangrenosum is a rare neutrophilic dermatosis presenting as painful cutaneous ulcers with undermined, irregular borders and purulent bases that may enlarge. About half of cases are associated with underlying systemic disease. Treatment focuses on controlling underlying conditions and using high-dose corticosteroids or immunosuppressants.
Nutrophilic dermatosis
This document discusses neutrophilic dermatoses, a spectrum of disorders characterized by neutrophilic infiltration of the skin without true vasculitis. Key points include:
- Common features include a neutrophilic vascular reaction, some cases having a reactive or systemic cause, disorders may coexist or occur sequentially in individuals.
- Classification includes disorders grouped by location of neutrophilic infiltrate (epidermal vs dermal).
- Pyoderma gangrenosum is discussed in depth, including its pathogenesis, associated diseases, diagnostic criteria, variants (classic, pustular, bullous etc.), investigations and histopathology. Treatment involves immunosuppressive therapy.
Dermo epidermal junction
This document discusses the dermo-epidermal junction (DEJ) and dermis. It describes the four layers of the basement membrane zone (BMZ) of the DEJ - the basal keratinocyte layer containing hemidesmosomes, the lamina lucida, lamina densa containing type IV collagen and laminins, and the lamina fibroreticularis containing anchoring fibrils made of type VII collagen. It also discusses the cells and extracellular matrix components of the dermis, including collagen, elastic fibers, proteoglycans, fibroblasts, macrophages, dendrocytes and mast cells. Disorders of the DEJ like epidermolysis bullosa are also mentioned.
Morphology of skin lesions tim
This document provides an overview of the approach to dermatologic diagnosis and the morphology of skin lesions. It discusses taking a thorough patient history and performing a physical exam, noting the four cardinal features of describing lesions - type, shape, arrangement, and distribution. Common skin lesions are defined, including macules, papules, plaques, nodules, vesicles, pustules, and others. Morphologic characteristics like color, size, texture, and distribution are important for diagnosis. A systematic approach including history, exam, and potential investigations is essential for evaluating skin conditions.
Approach to a patient with vesicobullous disorder
This document provides an approach to evaluating patients presenting with vesicobullous disorders. It begins by defining vesicles and bullae and then discusses the history, examination, and investigations for vesicobullous disorders in neonates, children, and adults. Key points covered include distinguishing features of various disorders based on onset, progression, distribution of lesions, associated symptoms, histopathology and immunological findings. The document provides a comprehensive overview of evaluating patients across different age groups presenting with this group of skin conditions.
Eczema
Eczema is a skin inflammation characterized by itching and dryness. It is caused by both external (exogenous) factors like irritants or allergens as well as internal (endogenous) factors. The pathology involves inflammation and thickening of the epidermis and dermis. Clinically it presents as redness, scaling, excoriation and thickening of the skin (lichenification). It most commonly affects infants/children and the elderly. Treatment involves reducing inflammation and preventing infection through topical corticosteroids, moisturizers and oral antihistamines.
Eosiniphil in dermatology
This document provides information on eosinophils and various eosinophilic conditions. It begins with basic facts about eosinophils as bone marrow-derived cells that normally make up 6% of white blood cells. It then discusses eosinophilic syndromes like hypereosinophilic syndrome and its subtypes. Other conditions discussed in detail include Well's syndrome, eosinophilic cellulitis, lymphocytic variant HES, myeloproliferative HES, granuloma faciale, erythema elevatum diutinum, eosinophilic pustular folliculitis, and recurrent cutaneous eosinophilic vasculitis. Clinical findings, histopathology, differential diagnoses
Cutaneous manifestations of diabetes mellitus
This document summarizes various cutaneous manifestations that can occur in patients with diabetes mellitus. It classifies them into vascular, metabolic, necrobiotic, bullous, infection, neuropathic, treatment related and miscellaneous categories. Some of the most common manifestations discussed include diabetic dermopathy, acanthosis nigricans, necrobiosis lipoidica, bullous diabeticorum, bacterial and fungal infections, lipodystrophy and various cutaneous complications related to diabetes treatment. Evaluation and management of these cutaneous signs are important for diabetes care and control.
Recommended
Neutrophilic dermatosis
1. Sweet's syndrome is an uncommon skin condition characterized by abrupt onset of tender red skin lesions and systemic symptoms. Histopathology shows a dense neutrophilic infiltrate without evidence of infection.
2. Treatment involves oral corticosteroids which provide excellent response within days to weeks. Recurrences may occur in about 30% of cases.
3. Pyoderma gangrenosum is a rare neutrophilic dermatosis presenting as painful cutaneous ulcers with undermined, irregular borders and purulent bases that may enlarge. About half of cases are associated with underlying systemic disease. Treatment focuses on controlling underlying conditions and using high-dose corticosteroids or immunosuppressants.
Nutrophilic dermatosis
This document discusses neutrophilic dermatoses, a spectrum of disorders characterized by neutrophilic infiltration of the skin without true vasculitis. Key points include:
- Common features include a neutrophilic vascular reaction, some cases having a reactive or systemic cause, disorders may coexist or occur sequentially in individuals.
- Classification includes disorders grouped by location of neutrophilic infiltrate (epidermal vs dermal).
- Pyoderma gangrenosum is discussed in depth, including its pathogenesis, associated diseases, diagnostic criteria, variants (classic, pustular, bullous etc.), investigations and histopathology. Treatment involves immunosuppressive therapy.
Dermo epidermal junction
This document discusses the dermo-epidermal junction (DEJ) and dermis. It describes the four layers of the basement membrane zone (BMZ) of the DEJ - the basal keratinocyte layer containing hemidesmosomes, the lamina lucida, lamina densa containing type IV collagen and laminins, and the lamina fibroreticularis containing anchoring fibrils made of type VII collagen. It also discusses the cells and extracellular matrix components of the dermis, including collagen, elastic fibers, proteoglycans, fibroblasts, macrophages, dendrocytes and mast cells. Disorders of the DEJ like epidermolysis bullosa are also mentioned.
Morphology of skin lesions tim
This document provides an overview of the approach to dermatologic diagnosis and the morphology of skin lesions. It discusses taking a thorough patient history and performing a physical exam, noting the four cardinal features of describing lesions - type, shape, arrangement, and distribution. Common skin lesions are defined, including macules, papules, plaques, nodules, vesicles, pustules, and others. Morphologic characteristics like color, size, texture, and distribution are important for diagnosis. A systematic approach including history, exam, and potential investigations is essential for evaluating skin conditions.
Approach to a patient with vesicobullous disorder
This document provides an approach to evaluating patients presenting with vesicobullous disorders. It begins by defining vesicles and bullae and then discusses the history, examination, and investigations for vesicobullous disorders in neonates, children, and adults. Key points covered include distinguishing features of various disorders based on onset, progression, distribution of lesions, associated symptoms, histopathology and immunological findings. The document provides a comprehensive overview of evaluating patients across different age groups presenting with this group of skin conditions.
Eczema
Eczema is a skin inflammation characterized by itching and dryness. It is caused by both external (exogenous) factors like irritants or allergens as well as internal (endogenous) factors. The pathology involves inflammation and thickening of the epidermis and dermis. Clinically it presents as redness, scaling, excoriation and thickening of the skin (lichenification). It most commonly affects infants/children and the elderly. Treatment involves reducing inflammation and preventing infection through topical corticosteroids, moisturizers and oral antihistamines.
Eosiniphil in dermatology
This document provides information on eosinophils and various eosinophilic conditions. It begins with basic facts about eosinophils as bone marrow-derived cells that normally make up 6% of white blood cells. It then discusses eosinophilic syndromes like hypereosinophilic syndrome and its subtypes. Other conditions discussed in detail include Well's syndrome, eosinophilic cellulitis, lymphocytic variant HES, myeloproliferative HES, granuloma faciale, erythema elevatum diutinum, eosinophilic pustular folliculitis, and recurrent cutaneous eosinophilic vasculitis. Clinical findings, histopathology, differential diagnoses
Cutaneous manifestations of diabetes mellitus
This document summarizes various cutaneous manifestations that can occur in patients with diabetes mellitus. It classifies them into vascular, metabolic, necrobiotic, bullous, infection, neuropathic, treatment related and miscellaneous categories. Some of the most common manifestations discussed include diabetic dermopathy, acanthosis nigricans, necrobiosis lipoidica, bullous diabeticorum, bacterial and fungal infections, lipodystrophy and various cutaneous complications related to diabetes treatment. Evaluation and management of these cutaneous signs are important for diabetes care and control.
PID case- Blau syndrome final.pptx
1. A 3-year-old male child presented with joint pain, fever, and skin rash for 6 months. His mother had a similar history since childhood of inflammatory joint pain, skin lesions, and eye issues.
2. Examination found joint swelling and tenderness, skin papules, and the mother had hand deformities. Investigations for both were normal.
3. A differential diagnosis of Blau syndrome or familial early onset sarcoidosis was made given the familial pattern. Genetic testing found a pathogenic NOD2 variant consistent with Blau syndrome.
granulomatosis with polyangiitis (Wegener’s granulomatosis)
This document discusses Wegener's granulomatosis (now called granulomatosis with polyangiitis or GPA), a rare multisystem autoimmune disease characterized by necrotizing vasculitis and granulomatous inflammation that commonly involves the respiratory tract and kidneys. Key points include:
- GPA is associated with circulating ANCA antibodies and causes necrotizing inflammation of small to medium vessels.
- Common clinical manifestations involve the upper respiratory tract, lungs, and kidneys. Chest imaging often shows nodules/masses, cavities, ground glass opacities and consolidations.
- Diagnosis is based on clinical features, labs including positive ANCA, and biopsy
Bullous diseases
This document summarizes several bullous diseases:
1. It describes the locations and characteristics of vesicles and bullae. Vesicles can form within or under the epidermis or between the dermis and epidermis.
2. It then focuses on three main immunobullous diseases - pemphigus, pemphigoid, and linear IgA bullous disease. Pemphigus is caused by antibodies against desmoglein proteins and features flaccid blisters. Pemphigoid features tense blisters caused by antibodies against basement membrane proteins. Linear IgA bullous disease clinically resembles pemphigoid.
3. Dermatitis herpetiformis is described
Dyschromatosis and Reticulate pigmentary disorders
This document summarizes several rare pigmentary disorders including reticulate pigmentary disorders, dyschromatosis, and related conditions. Key points include:
- Reticulate pigmentary disorders involve freckle-like macules joined at margins in a net-like pattern, while dyschromatosis involves interspersed hyperpigmented and hypopigmented macules.
- Several conditions are classified including acral, flexural, generalized types like reticulate acropigmentation of Kitamura and dyschromatosis symmetrica hereditaria.
- Genetic mutations in genes like ADAM10, DSRAD, KRT5 are associated with these conditions. Histopathology often
Ocular Manifestations of Leprosy - EYE
This document discusses the ocular manifestations of leprosy. It begins by noting that leprosy continues to carry stigma that prevents patients from seeking eye care. It then outlines several common eye complications of leprosy, including loss of eyebrows and eyelashes, ectropion, entropion, lagophthalmos, reduced corneal sensation, corneal ulcers, scleritis, uveitis, cataracts, and glaucoma. For each complication, it describes signs, symptoms, risk factors, and treatment approaches. It emphasizes that eye complications can occur before, during, and after treatment for leprosy. The document concludes by stressing the importance of multidisciplinary care and lowering barriers to eye care in
Lichen planus ppt
Lichen planus is a chronic inflammatory disease that affects the skin and mucous membranes. Microscopically, it is characterized by a band-like lymphohistiocytic infiltrate at the dermo-epidermal junction, vacuolar alteration of basal keratinocytes, saw-toothed rete ridges, and wedge-shaped hypergranulosis. Clinically, it presents as pruritic, violaceous flat-topped papules and plaques, often with white Wickham striae. Variants include hypertrophic, atrophic, ulcerative, actinicus, and lichen planopilaris forms. It is important to differentiate lichen planus from other lichenoid
Atopic dermatitis
Atopic dermatitis is a chronic inflammatory skin disease associated with respiratory allergies. It is characterized by recurrent eczematous lesions and intense itch. Genetic factors like filaggrin mutations cause skin barrier defects allowing allergens and microbes to trigger immune responses. The disease involves type 2 immunity cytokines activating neurons to produce itch. Staphylococcus aureus colonization exacerbates inflammation. Clinical features include erythematous patches and plaques with lichenification in chronic cases.
Dermatological history and examination
This document provides an overview of dermatological history taking, physical examination, diagnostic testing, and differential diagnosis of skin diseases in dogs. It discusses taking a patient history, performing a physical exam to identify primary and secondary skin lesions, and collecting samples via skin scrapings, trichograms, tape stripping, fungal culture and cytology. A range of parasitic, bacterial, viral, fungal and other infectious and non-infectious dermatological conditions are described. Diagnostic tests include Wood's lamp exam, skin biopsies and bacterial culture. Treatment may involve topical therapies, systemic drugs or surgery.
Parry romberg syndrome
Parry Romberg syndrome is a rare neurocutaneous condition characterized by the progressive shrinkage and degeneration of tissues beneath the skin, usually on one side of the face but sometimes extending to other areas. It often presents with neurological, ocular, and oral signs and symptoms. While the exact cause is unknown, theories include infection, peripheral nerve inflammation, or autonomic nervous system dysfunction. Treatment aims to stop further progression and repair deformities through immunosuppression or reconstructive procedures once stabilization is achieved. Parry Romberg syndrome continues to challenge researchers due to its unknown origins and varied manifestations.
Epidermolysis bullosa complete
Epidermolysis bullosa (EB) is a group of genetic skin disorders characterized by skin fragility and blistering from minor mechanical trauma. There are several types of EB including EB simplex, junctional EB, dystrophic EB, and Kindler syndrome. The diagnosis is based on the level within the skin that blistering occurs, as determined by immunofluorescence mapping and transmission electron microscopy of a skin biopsy. EB is managed by avoiding blistering through protective padding, preventing infection of wounds, and treating specific complications involving the eyes, esophagus, hands/feet, and risk of skin cancers in some severe types.
Wood’s light in dermatology
Wood's lamp uses long-wave UV radiation to cause tissues to fluoresce, allowing diseases and conditions to be diagnosed. It emits radiation between 320-400nm, causing fluorophores in the skin like collagen to glow blue. Various conditions have distinctive fluorescence - vitiligo appears bright blue due to lack of melanin, while pityriasis versicolor glows yellow-gold due to a skin yeast. Wood's lamp is useful for diagnosing infections caused by fungi, bacteria, and viruses based on the fluorescent compounds they produce. It can also detect pre-cancerous and cancerous lesions treated with fluorescent precursors.
Nikolsky sign
Nikolsky's sign refers to the ability to induce peripheral extension of a blister by applying lateral pressure to the border of an intact blister. It is diagnostic of pemphigus and indicates disease activity, as it will be positive during active stages and negative once treatment begins. The sign is elicited by applying pressure with the thumb over a bony prominence, causing the upper epidermal layers to separate from the lower layers. Variants include marginal and direct Nikolsky's sign, as well as false-Nikolsky's sign seen in subepidermal blistering disorders where pressure pulls apart the blister roof rather than inducing new blistering.
Dermo epidermal junction
dermoepidermal junction is the main interconnecting supporting strucure binding the Epidermis and Dermis together
Pemphigus Disorders of skin
Pemphigus is a group of chronic autoimmune bullous diseases characterized by the presence of autoantibodies against desmosomal proteins. The main types are pemphigus vulgaris, pemphigus foliaceus, and pemphigus erythematosus. Pemphigus vulgaris presents with flaccid blisters and erosions especially in the oral cavity and skin folds. Pemphigus foliaceus presents with fragile superficial blisters that leave moist erosions and crusts mainly on the face and scalp. Diagnosis involves clinical features and detection of autoantibodies through laboratory tests. Nikolsky's sign, elicited by applying pressure to separate
Granuloma annulare 2.0
- A 7 year old female presented with a skin biopsy from her left gluteal region for a clinical diagnosis of granuloma annulare.
- Microscopic examination showed features consistent with granuloma annulare including hyperkeratosis, acanthosis, lymphocytic infiltration, and histiocytes surrounding degenerated collagen extending into the dermis.
- Granuloma annulare is a benign inflammatory dermatosis more common in females that involves skin and subcutaneous tissue, though the etiology is unknown.
Ganyang MCQ Dermatology
The document discusses various topics in dermatology including:
- Common skin lesions and their definitions.
- Side effects of topical steroids including atrophy, bruising, allergic contact dermatitis, and moon face.
- Characteristics of psoriasis such as it being inherited, common nail involvement, and methotrexate being used to treat severe cases.
- Investigations for contact dermatitis which includes patch testing.
- Patient education for contact dermatitis including avoiding allergens and wearing gloves.
- Causes of non-scarring hair loss such as alopecia areata, drugs, hypothyroidism, and trauma.
Chronic actinic dermatitis
Chronic actinic dermatitis is a long-term inflammatory skin disorder caused by sun exposure that presents as itchy eczematous patches on light exposed areas. It can develop from pre-existing conditions like eczema or photosensitivity due to drugs. The pathogenesis involves CD8+ T cells causing a delayed hypersensitivity reaction when normal skin proteins are altered by UV light. Diagnosis is based on clinical examination, reduced skin sensitivity to UV light on testing, and histology showing chronic eczema. Treatment focuses on absolute sun protection and topical or oral immunosuppressants depending on severity.
Molluscum contagiosum Made Extremely Simple
A brief description of a very common viral infection affecting children and adults. Molluscum Contagious is an infectious contagious disease. Useful information regarding the symptoms and treatment of the rash are available for medical students, doctors, dermatologists, ophthalmologists, gynaecologist, pediatricians and nurses. Helpful for studying for exams. Reference: Rooks, Textbook of Dermatology
Lichen planus
Lichen planus is a chronic inflammatory skin condition that causes itchy, purple-colored papules and plaques. It is thought to be an autoimmune reaction targeting skin cells. The lesions typically appear on the wrists, legs, and oral mucosa. On microscopy, distinctive saw-tooth shaped keratinocytes (Civatte bodies) and band-like inflammatory infiltrate are seen. Treatment involves topical corticosteroids and immunomodulators. While usually self-limiting, lichen planus can lead to scarring and has a small risk of malignant transformation, especially in oral lesions.
Diabetic retinopathy
This document describes diabetic retinopathy (DR), its classification, pathogenesis, risk factors, screening protocols, and treatment. DR is classified as non-proliferative DR (NPDR) or proliferative DR (PDR). NPDR is further divided into mild, moderate, severe, and very severe stages based on lesions seen. PDR is characterized by new blood vessel growth. Clinically significant macular edema (CSME) can occur and cause vision loss. Screening intervals depend on DR severity. Laser treatment is used for CSME, PDR, and sometimes severe NPDR to prevent vision loss complications like vitreous hemorrhage or retinal detachment. Good glucose and blood pressure control can delay DR progression.
Palmoplantar Keratodermas
PPKs are inherited or acquired heterogeneous group of skin conditions with chronic thick hyperkeratotic palms/soles
More Related Content
What's hot
PID case- Blau syndrome final.pptx
1. A 3-year-old male child presented with joint pain, fever, and skin rash for 6 months. His mother had a similar history since childhood of inflammatory joint pain, skin lesions, and eye issues.
2. Examination found joint swelling and tenderness, skin papules, and the mother had hand deformities. Investigations for both were normal.
3. A differential diagnosis of Blau syndrome or familial early onset sarcoidosis was made given the familial pattern. Genetic testing found a pathogenic NOD2 variant consistent with Blau syndrome.
granulomatosis with polyangiitis (Wegener’s granulomatosis)
This document discusses Wegener's granulomatosis (now called granulomatosis with polyangiitis or GPA), a rare multisystem autoimmune disease characterized by necrotizing vasculitis and granulomatous inflammation that commonly involves the respiratory tract and kidneys. Key points include:
- GPA is associated with circulating ANCA antibodies and causes necrotizing inflammation of small to medium vessels.
- Common clinical manifestations involve the upper respiratory tract, lungs, and kidneys. Chest imaging often shows nodules/masses, cavities, ground glass opacities and consolidations.
- Diagnosis is based on clinical features, labs including positive ANCA, and biopsy
Bullous diseases
This document summarizes several bullous diseases:
1. It describes the locations and characteristics of vesicles and bullae. Vesicles can form within or under the epidermis or between the dermis and epidermis.
2. It then focuses on three main immunobullous diseases - pemphigus, pemphigoid, and linear IgA bullous disease. Pemphigus is caused by antibodies against desmoglein proteins and features flaccid blisters. Pemphigoid features tense blisters caused by antibodies against basement membrane proteins. Linear IgA bullous disease clinically resembles pemphigoid.
3. Dermatitis herpetiformis is described
Dyschromatosis and Reticulate pigmentary disorders
This document summarizes several rare pigmentary disorders including reticulate pigmentary disorders, dyschromatosis, and related conditions. Key points include:
- Reticulate pigmentary disorders involve freckle-like macules joined at margins in a net-like pattern, while dyschromatosis involves interspersed hyperpigmented and hypopigmented macules.
- Several conditions are classified including acral, flexural, generalized types like reticulate acropigmentation of Kitamura and dyschromatosis symmetrica hereditaria.
- Genetic mutations in genes like ADAM10, DSRAD, KRT5 are associated with these conditions. Histopathology often
Ocular Manifestations of Leprosy - EYE
This document discusses the ocular manifestations of leprosy. It begins by noting that leprosy continues to carry stigma that prevents patients from seeking eye care. It then outlines several common eye complications of leprosy, including loss of eyebrows and eyelashes, ectropion, entropion, lagophthalmos, reduced corneal sensation, corneal ulcers, scleritis, uveitis, cataracts, and glaucoma. For each complication, it describes signs, symptoms, risk factors, and treatment approaches. It emphasizes that eye complications can occur before, during, and after treatment for leprosy. The document concludes by stressing the importance of multidisciplinary care and lowering barriers to eye care in
Lichen planus ppt
Lichen planus is a chronic inflammatory disease that affects the skin and mucous membranes. Microscopically, it is characterized by a band-like lymphohistiocytic infiltrate at the dermo-epidermal junction, vacuolar alteration of basal keratinocytes, saw-toothed rete ridges, and wedge-shaped hypergranulosis. Clinically, it presents as pruritic, violaceous flat-topped papules and plaques, often with white Wickham striae. Variants include hypertrophic, atrophic, ulcerative, actinicus, and lichen planopilaris forms. It is important to differentiate lichen planus from other lichenoid
Atopic dermatitis
Atopic dermatitis is a chronic inflammatory skin disease associated with respiratory allergies. It is characterized by recurrent eczematous lesions and intense itch. Genetic factors like filaggrin mutations cause skin barrier defects allowing allergens and microbes to trigger immune responses. The disease involves type 2 immunity cytokines activating neurons to produce itch. Staphylococcus aureus colonization exacerbates inflammation. Clinical features include erythematous patches and plaques with lichenification in chronic cases.
Dermatological history and examination
This document provides an overview of dermatological history taking, physical examination, diagnostic testing, and differential diagnosis of skin diseases in dogs. It discusses taking a patient history, performing a physical exam to identify primary and secondary skin lesions, and collecting samples via skin scrapings, trichograms, tape stripping, fungal culture and cytology. A range of parasitic, bacterial, viral, fungal and other infectious and non-infectious dermatological conditions are described. Diagnostic tests include Wood's lamp exam, skin biopsies and bacterial culture. Treatment may involve topical therapies, systemic drugs or surgery.
Parry romberg syndrome
Parry Romberg syndrome is a rare neurocutaneous condition characterized by the progressive shrinkage and degeneration of tissues beneath the skin, usually on one side of the face but sometimes extending to other areas. It often presents with neurological, ocular, and oral signs and symptoms. While the exact cause is unknown, theories include infection, peripheral nerve inflammation, or autonomic nervous system dysfunction. Treatment aims to stop further progression and repair deformities through immunosuppression or reconstructive procedures once stabilization is achieved. Parry Romberg syndrome continues to challenge researchers due to its unknown origins and varied manifestations.
Epidermolysis bullosa complete
Epidermolysis bullosa (EB) is a group of genetic skin disorders characterized by skin fragility and blistering from minor mechanical trauma. There are several types of EB including EB simplex, junctional EB, dystrophic EB, and Kindler syndrome. The diagnosis is based on the level within the skin that blistering occurs, as determined by immunofluorescence mapping and transmission electron microscopy of a skin biopsy. EB is managed by avoiding blistering through protective padding, preventing infection of wounds, and treating specific complications involving the eyes, esophagus, hands/feet, and risk of skin cancers in some severe types.
Wood’s light in dermatology
Wood's lamp uses long-wave UV radiation to cause tissues to fluoresce, allowing diseases and conditions to be diagnosed. It emits radiation between 320-400nm, causing fluorophores in the skin like collagen to glow blue. Various conditions have distinctive fluorescence - vitiligo appears bright blue due to lack of melanin, while pityriasis versicolor glows yellow-gold due to a skin yeast. Wood's lamp is useful for diagnosing infections caused by fungi, bacteria, and viruses based on the fluorescent compounds they produce. It can also detect pre-cancerous and cancerous lesions treated with fluorescent precursors.
Nikolsky sign
Nikolsky's sign refers to the ability to induce peripheral extension of a blister by applying lateral pressure to the border of an intact blister. It is diagnostic of pemphigus and indicates disease activity, as it will be positive during active stages and negative once treatment begins. The sign is elicited by applying pressure with the thumb over a bony prominence, causing the upper epidermal layers to separate from the lower layers. Variants include marginal and direct Nikolsky's sign, as well as false-Nikolsky's sign seen in subepidermal blistering disorders where pressure pulls apart the blister roof rather than inducing new blistering.
Dermo epidermal junction
dermoepidermal junction is the main interconnecting supporting strucure binding the Epidermis and Dermis together
Pemphigus Disorders of skin
Pemphigus is a group of chronic autoimmune bullous diseases characterized by the presence of autoantibodies against desmosomal proteins. The main types are pemphigus vulgaris, pemphigus foliaceus, and pemphigus erythematosus. Pemphigus vulgaris presents with flaccid blisters and erosions especially in the oral cavity and skin folds. Pemphigus foliaceus presents with fragile superficial blisters that leave moist erosions and crusts mainly on the face and scalp. Diagnosis involves clinical features and detection of autoantibodies through laboratory tests. Nikolsky's sign, elicited by applying pressure to separate
Granuloma annulare 2.0
- A 7 year old female presented with a skin biopsy from her left gluteal region for a clinical diagnosis of granuloma annulare.
- Microscopic examination showed features consistent with granuloma annulare including hyperkeratosis, acanthosis, lymphocytic infiltration, and histiocytes surrounding degenerated collagen extending into the dermis.
- Granuloma annulare is a benign inflammatory dermatosis more common in females that involves skin and subcutaneous tissue, though the etiology is unknown.
Ganyang MCQ Dermatology
The document discusses various topics in dermatology including:
- Common skin lesions and their definitions.
- Side effects of topical steroids including atrophy, bruising, allergic contact dermatitis, and moon face.
- Characteristics of psoriasis such as it being inherited, common nail involvement, and methotrexate being used to treat severe cases.
- Investigations for contact dermatitis which includes patch testing.
- Patient education for contact dermatitis including avoiding allergens and wearing gloves.
- Causes of non-scarring hair loss such as alopecia areata, drugs, hypothyroidism, and trauma.
Chronic actinic dermatitis
Chronic actinic dermatitis is a long-term inflammatory skin disorder caused by sun exposure that presents as itchy eczematous patches on light exposed areas. It can develop from pre-existing conditions like eczema or photosensitivity due to drugs. The pathogenesis involves CD8+ T cells causing a delayed hypersensitivity reaction when normal skin proteins are altered by UV light. Diagnosis is based on clinical examination, reduced skin sensitivity to UV light on testing, and histology showing chronic eczema. Treatment focuses on absolute sun protection and topical or oral immunosuppressants depending on severity.
Molluscum contagiosum Made Extremely Simple
A brief description of a very common viral infection affecting children and adults. Molluscum Contagious is an infectious contagious disease. Useful information regarding the symptoms and treatment of the rash are available for medical students, doctors, dermatologists, ophthalmologists, gynaecologist, pediatricians and nurses. Helpful for studying for exams. Reference: Rooks, Textbook of Dermatology
Lichen planus
Lichen planus is a chronic inflammatory skin condition that causes itchy, purple-colored papules and plaques. It is thought to be an autoimmune reaction targeting skin cells. The lesions typically appear on the wrists, legs, and oral mucosa. On microscopy, distinctive saw-tooth shaped keratinocytes (Civatte bodies) and band-like inflammatory infiltrate are seen. Treatment involves topical corticosteroids and immunomodulators. While usually self-limiting, lichen planus can lead to scarring and has a small risk of malignant transformation, especially in oral lesions.
Diabetic retinopathy
This document describes diabetic retinopathy (DR), its classification, pathogenesis, risk factors, screening protocols, and treatment. DR is classified as non-proliferative DR (NPDR) or proliferative DR (PDR). NPDR is further divided into mild, moderate, severe, and very severe stages based on lesions seen. PDR is characterized by new blood vessel growth. Clinically significant macular edema (CSME) can occur and cause vision loss. Screening intervals depend on DR severity. Laser treatment is used for CSME, PDR, and sometimes severe NPDR to prevent vision loss complications like vitreous hemorrhage or retinal detachment. Good glucose and blood pressure control can delay DR progression.
What's hot (20)
granulomatosis with polyangiitis (Wegener’s granulomatosis)
granulomatosis with polyangiitis (Wegener’s granulomatosis)
Dyschromatosis and Reticulate pigmentary disorders
Dyschromatosis and Reticulate pigmentary disorders
Viewers also liked
Palmoplantar Keratodermas
PPKs are inherited or acquired heterogeneous group of skin conditions with chronic thick hyperkeratotic palms/soles
Dermatology 5th year, 3rd lecture (Dr. Kazhan)
This document discusses bullous skin disorders, focusing on pemphigus vulgaris (PV). It defines PV as an autoimmune blistering disease caused by antibodies against desmoglein 3, resulting in fragile blisters and erosions on skin and mucous membranes. It describes the clinical presentation of PV including lesions, Nikolski's sign, and involvement of oral mucosa. It also covers pathogenesis, diagnosis via histology and immunofluorescence, and treatment primarily with corticosteroids and immunosuppressants to reduce blister formation and promote healing.
Erythroderma
Erythroderma is defined as the scaling erythematous dermatitis involving 90% or more of the cutaneous surface.
Also known as exfoliative dermatitis
Idiopathic exfoliative dermatitis – also known as the “red man syndrome”, is characterized by marked palmoplantar keratoderma, dermatopathic lymphadenopathy,increased IgE.
Increased skin perfusion leads to
Temperature dysregulation >
Resulting in skin loss and hypothermia >
High output state >
Cardiac failure
BMR raises to compensate for heat loss
Increased dehydration due to transpiration (similar to burns)
All lead to negative nitrogen balance and characterized by edema, hypoalbuminemia, loss of muscle mass.
Comedonal Acne Cure in 10 Easy Steps!
This document outlines 10 steps to cure comedonal acne based on the author's personal experience dealing with adult acne. It discusses choosing the right cleansers and pH level for skin, daily exfoliation, balancing oil production, using retinoids to increase cell turnover, providing moisture without heavy creams, addressing hormonal imbalances, using benzoyl peroxide to introduce oxygen into pores, extracting hardened bumps, considering chemical peels, and examining diet for food sensitivities behind breakouts. Additional resources are provided on the website for further acne treatment techniques.
Clamídia
A clamídia é causada pela bactéria Chlamydia trachomatis e pode danificar os órgãos sexuais, causar infertilidade e secreções no pénis. Apresenta poucos sintomas, mas pode causar secreções vaginais e ardores ao urinar. É tratada com antibióticos e requer exame dos parceiros sexuais.
5 Cures for Chlamydia You Should Know!
Chlamydia is a common sexually transmitted infection caused by the bacteria Chlamydia trachomatis. It can cause symptoms like painful urination and discharge but is often asymptomatic. While anyone can get chlamydia, those at highest risk are teens, people with multiple sex partners, and illicit drug users. Chlamydia is treated with antibiotics like azithromycin, doxycycline, or amoxicillin. Left untreated it can cause infertility in women. Prevention involves limiting partners, using condoms, and getting tested annually.
Viewers also liked (7)
Similar to Vohwinkel Syndrome vs. KID Syndrome
Approach to childhood poikiloderma.pptx
1. The document discusses several congenital and acquired childhood poikilodermas, including their clinical features, etiology, diagnostic criteria and genetic basis.
2. Major congenital poikilodermas discussed include Rothmund-Thomson syndrome, dyskeratosis congenita, hereditary sclerosing poikiloderma, and hereditary fibrosing poikiloderma.
3. Genodermatoses associated with both poikiloderma and photosensitivity include xeroderma pigmentosum and Kindler syndrome. Bloom syndrome and Clericuzio-type poikiloderma are also briefly summarized.
Corneal dystrophies
1. The document discusses various types of corneal dystrophies, including epithelial, stromal, and Descemet membrane dystrophies.
2. Corneal dystrophies are classified based on which layer of the cornea is affected and their clinical, pathological, and genetic characteristics. The International Committee for Classification of Corneal Dystrophies provides the current classification system.
3. Specific dystrophies discussed include epithelial basement membrane dystrophy, epithelial recurrent erosion dystrophy, Reis-Bucklers corneal dystrophy, Thiel-Behnke corneal dystrophy, lattice corneal dystrophy types 1 and 2, and granular corneal dystrophy type 1. Details are provided on inheritance patterns, genetic causes
palisades15.pdf
Neonatal herpes simplex virus infection can occur in newborns who are exposed to HSV during vaginal delivery. Risk of transmission is greater if the mother acquires a primary genital HSV infection during pregnancy. Lesions typically appear on the scalp within the first 2 weeks of life and are often grouped papulovesicles on an erythematous base. Use of fetal scalp monitors may disrupt the infant's skin barrier and increase risk of HSV infection. Prompt diagnosis and antiviral treatment are important to reduce mortality and morbidity.
Sailing through cornea part 2
This document discusses corneal dystrophies and degenerations. It notes key differences between dystrophies and degenerations such as dystrophies typically being bilateral and symmetrical, occurring earlier in life, and having a positive family history, while degenerations are usually unilateral or asymmetrical and occur later in life. It then covers the main types of corneal dystrophies including epithelial, Bowman's membrane, stromal, and endothelial dystrophies. Specific dystrophies are discussed in terms of their inheritance, clinical presentation, and treatment.
Congenital ichthyosis
The document discusses various types of ichthyosis, which are genetic skin disorders characterized by dry, thickened, scaly skin. It describes several specific types in detail, including ichthyosis vulgaris, X-linked ichthyosis, lamellar ichthyosis, and harlequin ichthyosis. Harlequin ichthyosis is the most severe form, where infants are born with thick armor-like plates covering their entire body, along with other abnormalities. Prenatal ultrasound findings and genetic testing are discussed which can help diagnose some severe types of ichthyosis in utero.
Darier disease
This document describes a case report of a 35-year-old female patient presenting with Darier disease. Darier disease is a rare autosomal dominant genoderatosis caused by mutations in the ATP2A2 gene. Clinically, it presents with hyperkeratotic papules in seborrheic areas and nail abnormalities. Histologically, it shows suprabasal clefts with acantholytic and dyskeratotic cells. The patient presented with lesions on the head, neck, trunk and extremities consistent with Darier disease, along with nail abnormalities. A biopsy was taken and microscopic findings were consistent with the diagnosis.
Corneal dystrophies
This document provides an overview of different types of corneal dystrophies, including their classification, clinical features, histopathology, and management. It discusses epithelial and subepithelial dystrophies, corneal dystrophies of Bowman's layer, stromal corneal dystrophies, and Descemet membrane and endothelial dystrophies. The key points are that corneal dystrophies are inherited, bilateral, and slowly progressive disorders that begin early in life and are characterized by corneal opacification without relationship to environmental factors. Diagnosis involves classification based on the anatomical layer affected and treatment typically involves managing symptoms although surgery may be needed if vision is impaired.
Keratoconus and Its management
This document discusses ectatic disorders of the cornea, focusing on keratoconus. It defines keratoconus as a non-inflammatory thinning of the cornea that results in a protrusion and irregular astigmatism. The document covers the cascade hypothesis of oxidative damage in keratoconus pathogenesis, classification systems for keratoconus severity, signs and symptoms, and non-surgical management approaches like spectacles, contact lenses, and RGP fitting philosophies. The goal of management is to eliminate irregularities and provide optimal vision correction while minimizing further corneal damage.
Seborrhiec Keratosis
This document describes a case report of a 62-year-old female patient who presented with an asymptomatic growth on her left ear for one and a half years. The growth was diagnosed as a seborrhoeic keratosis based on its clinical features and confirmed by histopathological examination. Seborrhoeic keratosis is a benign skin tumor that commonly affects elderly people and presents as a pigmented verrucous plaque. While typically cosmetic, it can sometimes cause functional impairment or itching. Histological examination revealed features consistent with seborrhoeic keratosis such as hyperkeratosis, papillomatosis, and melanin pigment.
corneal dystrophy.pptx
corneal dystrophy and degeneration are very important factor for visual control especially in elderly people. there are various various classification on corneal dystrophy., which are based on disease etiology and progression of disease. timely intervention is required to save the vision.
Syndromes of orofacial region mine
This is a very comprehensive seminar on Syndromes of Orofacial region, easy classification and easy understanding.
Enjoy reading !!
Dermatology 5th year, 5th lecture (Dr. Kazhan)
Connective tissue disorders involve inflammation of connective tissue that can lead to skin changes like hardening or scarring as well as arthritis and other organ abnormalities. Lupus is an autoimmune disease where the immune system attacks the body's own tissues, affecting women more than men. It can cause a butterfly-shaped rash on the face, mouth sores, and inflammation of organs. Discoid lupus causes red scaly patches on the skin, especially the face, that can lead to scarring and pigment changes. Sjogren's syndrome destroys exocrine glands causing dry eyes and mouth. It is associated with rheumatoid arthritis and has a higher risk of lymphoma. Behcet's disease involves recurring
CORNEAL-DYSTROPHIES-Nafiz(2).pptx
The document discusses various types of corneal dystrophies, classified according to the layer of the cornea affected. It focuses on Fuchs endothelial corneal dystrophy, describing its stages from central guttae to dense opacity and vascularization. Symptoms include decreased vision, pain from ruptured bullae. Evaluation involves slit lamp exam and pachymetry. Management includes medical therapy like lubricants or surgery like DSEK/DMEK. The dystrophy is often sporadic but some familial forms are linked to genes like COL8A2.
CORNEAL DYSTROPHIES AND ECTASIAS.pptx
The document discusses various types of corneal dystrophies and ectasias. It begins with an introduction and outlines the different categories. It then describes the clinical features, inheritance patterns, pathology and treatment for several specific dystrophies including:
- Epithelial basement membrane dystrophy
- Meesman corneal dystrophy
- Fuchs endothelial corneal dystrophy
- Lattice corneal dystrophy type 1
- Granular corneal dystrophy type 1
It focuses on classifying and explaining the clinical presentations of different dystrophies affecting the epithelium, Bowman's layer, stroma, and endothelium. Management strategies including medical treatments and surgical options are also reviewed.
Hutchinson-Gilford Progeria Syndrome
Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic condition characterized by features of accelerated aging. It is caused by a mutation in the LMNA gene which leads to production of a defective protein called progerin. Children with HGPS appear normal at birth but start exhibiting aging-related problems at a very early age. They experience severe failure to thrive, loss of body fat and hair, stiff joints, and cardiovascular complications leading to death usually in their teens. The only approved treatment is lonafarnib which works by inhibiting progerin production and has shown benefits in improving growth and reducing disease severity. A multidisciplinary care approach is needed to manage the various health issues associated with H
Phacomatosis
This document summarizes several neurocutaneous syndromes including von Hippel-Lindau disease, neurofibromatosis type 1 and 2, tuberous sclerosis complex, Sturge-Weber syndrome, and Bourneville's disease. It describes the characteristic clinical features of each syndrome such as café au lait spots, tumors, hamartomas and visual or neurological disturbances. Diagnosis is based on the presence of specific lesions and symptoms. Screening recommendations are provided for early detection and treatment of complications.
Systemic sclerosis..scleroderma
This document provides information on systemic sclerosis (SSc), a chronic autoimmune disease characterized by thickening and hardening of the skin, and involvement of internal organs. It can present as either limited or diffuse cutaneous forms. Key clinical features include Raynaud's phenomenon, skin thickening and tightening, digital pitting scars, and internal organ involvement such as interstitial lung disease, gastrointestinal issues, and renal crisis. Diagnosis involves clinical examination and the presence of autoantibodies. Differential diagnoses include other conditions presenting with similar skin or vascular changes. Complications can affect multiple organ systems and are a leading cause of mortality.
Opthalmic disease
This document summarizes various ophthalmic diseases that affect the eyelids, conjunctiva, cornea, anterior uvea, lens, and ocular fundus in dogs, cats, horses, and other domestic animal species. It describes common eyelid disorders like entropion and ectropion. It also discusses inflammation of the eyelids, nasolacrimal diseases, conjunctivitis, corneal diseases like ulcers and dystrophies, anterior uveitis, glaucoma, cataracts, inherited retinopathies like progressive retinal atrophy, and chorioretinitis. The summary provides an overview of the key ophthalmic conditions and diseases addressed in the document.
SYMPATHETIC OPHTHALMIA & VKH SYNDROME
Sympathetic ophthalmia and Vogt-Koyanagi-Harada disease are both rare granulomatous uveitides that can cause bilateral eye inflammation. Sympathetic ophthalmia occurs after trauma or surgery to one eye, while VKH disease is thought to be an autoimmune response against ocular and skin melanocytes. Both can lead to vision loss if not treated promptly with high-dose corticosteroids and immunosuppressants. Imaging like fluorescein angiography and optical coherence tomography are useful for monitoring disease activity and response to treatment.
Similar to Vohwinkel Syndrome vs. KID Syndrome (20)
Recently uploaded
Journal Article Review on Rasamanikya
Rasamanikya is a excellent preparation in the field of Rasashastra, it is used in various Kushtha Roga, Shwasa, Vicharchika, Bhagandara, Vatarakta, and Phiranga Roga. In this article Preparation& Comparative analytical profile for both Formulationon i.e Rasamanikya prepared by Kushmanda swarasa & Churnodhaka Shodita Haratala. The study aims to provide insights into the comparative efficacy and analytical aspects of these formulations for enhanced therapeutic outcomes.
Cell Therapy Expansion and Challenges in Autoimmune Disease
There is increasing confidence that cell therapies will soon play a role in the treatment of autoimmune disorders, but the extent of this impact remains to be seen. Early readouts on autologous CAR-Ts in lupus are encouraging, but manufacturing and cost limitations are likely to restrict access to highly refractory patients. Allogeneic CAR-Ts have the potential to broaden access to earlier lines of treatment due to their inherent cost benefits, however they will need to demonstrate comparable or improved efficacy to established modalities.
In addition to infrastructure and capacity constraints, CAR-Ts face a very different risk-benefit dynamic in autoimmune compared to oncology, highlighting the need for tolerable therapies with low adverse event risk. CAR-NK and Treg-based therapies are also being developed in certain autoimmune disorders and may demonstrate favorable safety profiles. Several novel non-cell therapies such as bispecific antibodies, nanobodies, and RNAi drugs, may also offer future alternative competitive solutions with variable value propositions.
Widespread adoption of cell therapies will not only require strong efficacy and safety data, but also adapted pricing and access strategies. At oncology-based price points, CAR-Ts are unlikely to achieve broad market access in autoimmune disorders, with eligible patient populations that are potentially orders of magnitude greater than the number of currently addressable cancer patients. Developers have made strides towards reducing cell therapy COGS while improving manufacturing efficiency, but payors will inevitably restrict access until more sustainable pricing is achieved.
Despite these headwinds, industry leaders and investors remain confident that cell therapies are poised to address significant unmet need in patients suffering from autoimmune disorders. However, the extent of this impact on the treatment landscape remains to be seen, as the industry rapidly approaches an inflection point.
Adhd Medication Shortage Uk - trinexpharmacy.com
The UK is currently facing a Adhd Medication Shortage Uk, which has left many patients and their families grappling with uncertainty and frustration. ADHD, or Attention Deficit Hyperactivity Disorder, is a chronic condition that requires consistent medication to manage effectively. This shortage has highlighted the critical role these medications play in the daily lives of those affected by ADHD. Contact : +1 (747) 209 – 3649 E-mail : sales@trinexpharmacy.com
The Electrocardiogram - Physiologic Principles
These lecture slides, by Dr Sidra Arshad, offer a quick overview of the physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar lead (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
6. Describe the flow of current around the heart during the cardiac cycle
7. Discuss the placement and polarity of the leads of electrocardiograph
8. Describe the normal electrocardiograms recorded from the limb leads and explain the physiological basis of the different records that are obtained
9. Define mean electrical vector (axis) of the heart and give the normal range
10. Define the mean QRS vector
11. Describe the axes of leads (hexagonal reference system)
12. Comprehend the vectorial analysis of the normal ECG
13. Determine the mean electrical axis of the ventricular QRS and appreciate the mean axis deviation
14. Explain the concepts of current of injury, J point, and their significance
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. Chapter 3, Cardiology Explained, https://www.ncbi.nlm.nih.gov/books/NBK2214/
7. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
Hemodialysis: Chapter 4, Dialysate Circuit - Dr.Gawad
- Video recording of this lecture in English language: https://youtu.be/kqbnxVAZs-0
- Video recording of this lecture in Arabic language: https://youtu.be/SINlygW1Mpc
- Link to download the book free: https://nephrotube.blogspot.com/p/nephrotube-nephrology-books.html
- Link to NephroTube website: www.NephroTube.com
- Link to NephroTube social media accounts: https://nephrotube.blogspot.com/p/join-nephrotube-on-social-media.html
Identifying Major Symptoms of Slip Disc.
Our backs are like superheroes, holding us up and helping us move around. But sometimes, even superheroes can get hurt. That’s where slip discs come in.
Does Over-Masturbation Contribute to Chronic Prostatitis.pptx
In some case, your chronic prostatitis may be related to over-masturbation. Generally, natural medicine Diuretic and Anti-inflammatory Pill can help mee get a cure.
Efficacy of Avartana Sneha in Ayurveda
Avartana Sneha is a unique method of Preparation of Sneha Kalpana in Ayurveda, mainly it is indicated for the Vataja rogas.
Ketone bodies and metabolism-biochemistry
This slide consists of all the topics of ketone . This can be used for exam purpose for writing about Diabetic keto acidosis etc . Thank you
Histololgy of Female Reproductive System.pptx
Dive into an in-depth exploration of the histological structure of female reproductive system with this comprehensive lecture. Presented by Dr. Ayesha Irfan, Assistant Professor of Anatomy, this presentation covers the Gross anatomy and functional histology of the female reproductive organs. Ideal for students, educators, and anyone interested in medical science, this lecture provides clear explanations, detailed diagrams, and valuable insights into female reproductive system. Enhance your knowledge and understanding of this essential aspect of human biology.
Top Travel Vaccinations in Manchester
Travel vaccination in Manchester offers comprehensive immunization services for individuals planning international trips. Expert healthcare providers administer vaccines tailored to your destination, ensuring you stay protected against various diseases. Conveniently located clinics and flexible appointment options make it easy to get the necessary shots before your journey. Stay healthy and travel with confidence by getting vaccinated in Manchester. Visit us: www.nxhealthcare.co.uk
Recently uploaded (20)
CHEMOTHERAPY_RDP_CHAPTER 6_Anti Malarial Drugs.pdf
CHEMOTHERAPY_RDP_CHAPTER 6_Anti Malarial Drugs.pdf
Cell Therapy Expansion and Challenges in Autoimmune Disease
Cell Therapy Expansion and Challenges in Autoimmune Disease
Ear and its clinical correlations By Dr. Rabia Inam Gandapore.pptx
Ear and its clinical correlations By Dr. Rabia Inam Gandapore.pptx
Outbreak management including quarantine, isolation, contact.pptx
Outbreak management including quarantine, isolation, contact.pptx
Hemodialysis: Chapter 4, Dialysate Circuit - Dr.Gawad
Hemodialysis: Chapter 4, Dialysate Circuit - Dr.Gawad
Tests for analysis of different pharmaceutical.pptx
Tests for analysis of different pharmaceutical.pptx
Does Over-Masturbation Contribute to Chronic Prostatitis.pptx
Does Over-Masturbation Contribute to Chronic Prostatitis.pptx
Vohwinkel Syndrome vs. KID Syndrome
- 1. U C I D E R M A T O L O G Y G R A N D R O U N D S M A R C H 3 0 , 2 0 1 1 J E N N I F E R A R M S T R O N G Vohwinkel Syndrome vs. KID Syndrome
- 2. Connexin 26 Vohwinkel Syndrome and KID Syndrome are 2 clinically different syndromes both originating with missense mutation for Connexin 26 on Chrom 13.
- 3. What is a Connexin A protein which is the building blocks that forms channels in gap junctions Facilitates small molecules between cells Important intercellular connections
- 4. Connexin & Hearing Loss Connection Regulates K+ in Cochlea of inner ear.
- 5. Vohnwinkel Syndrome 2 Types Both group of palmoplanterkeratodermas 1 – Classical type with hearing loss Missense mutation of Connexin 26 2 – Ichthyosis- associated type Insertional mutation of the loricrin gene Develop thick ridged envelope
- 6. Vohwinkel Syndrome ……………………………………………………… Deafness, hearing impaired …..…Palmoplanter Keratoderma, honeycomb appearance ………………………… Starfish shaped hyperkeratotic plaques ……………………………….………… autoamputation (pseudoainhum)
- 7. Treatment - Vohwinkel Syndrome Rare Disease – Limited Options Topical - Usually inadequate - Keratolytics (i.esalicylates, urea) - Retinoids Systemic - Retinoids - Relapes after discontinue – almost all pts - Dose 60mg/Kg/PO Surgical - Surgical release of constriction bands to preserve digits
- 8. What is KID KID syndrome is a rare disorder characterized by keratitis (with progressive corneal opacification), ichthyosis, and deafness (neurosensory). Affects the epidermis but also Ectodermal tissue Predisposes patient to Squamous Cell Carcinoma on skin and tongue Also susceptible to bacterial, viral, and fungal infections
- 9. KID Acanthosis of the skin with a heavy-grained leather appearance.………..…………….. Hyperkeratotic Plaques………………….… ………………..…………VascularizingKeratitis …….……..…..Palmar Keratoderma Nail Dystrophy, ………………………………..fungal infections
- 10. KID Three months of age is typical age of cutaneous presentation Can start as general erythema Sometimes diffuse scaling Leathery skin
- 11. Ichthyosis Not a classic ichthyosis but more a erythrokeratoderma None scaling verrucous plaques, forehead and cheeks 79% alopecia sparse to totalalis
- 12. Key Findings Hearing loss Always by age 7 Variable degrees Subsequent speech delay Occular manifestations 95% Progressive Corneal vascularization Worsened by retinoids
- 13. Similarities Vohwinkels Syndrome KID Syndrome Connexin 26 Missense Mutation Connexin 26 Missense Mutation Hearing Loss Hearing LossPalmoplanter Keratoderma Palmoplanter Hyperkeratosis
- 14. Differences KID Syndrome ……………………. Alopecia ………………………………. OccularKeratitis ……………………………………………………...................….……. Small Teeth ………………………………..…………. Hyperkeratotic fixed plaques ……………………………………………………….…………. Leathering of skin …………………………………..…………. Increased squamous cell carcinoma …………………………………………………...………..….…. Nail dystrophy
- 15. Differences Vohwinkel Syndrome Intact hearing in ichthyosis association type……………….………………. Autoamputation (Pseudoainhum)…………….………………………. Star shaped plaques on dorsum of hands………………………….
- 16. Differences Vohwinkels Syndrome KID Syndrome - Retinoids improve - Retinoids worsen
- 17. References • Lucker GP, Van de Kerkhof PC, Steijlen PM. The hereditary palmoplantarkeratoses: an updated review and classification. Br J Dermatol. Jul 1994;131(1):1-14. • Camisa C, Rossana C. Variant of keratodermahereditariamutilans (Vohwinkel's syndrome). Treatment with orally administered isotretinoin. Arch Dermatol. Oct 1984;120(10):1323-8. • Solis RR, Diven DG, Trizna Z. Vohwinkel's syndrome in three generations. J Am AcadDermatol. Feb 2001;44(2 Suppl):376-8. • Kelsell DP, Dunlop J, Stevens HP, Lench NJ, Liang JN, Parry G, et al. Connexin 26 mutations in hereditary non- syndromicsensorineural deafness ?see comments?. Nature 1997; 387(6628): 80-3. • Richard G: Connexins: A connection with the skin. Exp Dermatol 9:77, 2000. • Jan AY et al: Genetic heterogeneity of KID syndrome: Identification of a Cx30 gene (GJB6) mutation in a patient with KID syndrome and congenital atrichia. J Invest Dermatol 122:1108. • Philip Fleckman, John J. DiGiovanna. Dermatology Part 2. Disorders Presenting in Skin and Mucous Membranes > Section 7. Disorders of Epidermal Differentiation and Keratinization>Chapter 47. The Ichthyoses. • Images from: Geneva Foundation for Medical Education and Research including images published in Journal of Investigative Dermatolog, The American Journal of Human Genetics, Pediatric DermatologyVolume 15, Issue 3, Article first published online: 16 MAR 2009.