The document discusses various types of ichthyoses and ichthyosiform syndromes. It describes ichthyosis vulgaris, X-linked ichthyosis, lamellar ichthyosis, epidermolytic hyperkeratosis, harlequin fetus, bullous ichthyosiform erythroderma, ichthyosis linearis circumflexa, Netherton syndrome, Refsum syndrome, KID syndrome, and CHILD syndrome. It also discusses acquired ichthyosis due to vitamin deficiencies, infections, medications, and systemic diseases.
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.
They are a heterogenous group of inherited disorders of epidermal differentiation featuring excessive scaling, Ichthyosis vulgaris,
X-linked recessive ichthyosis,
Lamellar ichthyosis,
Non-bullous ichthyosiform erythroderma,
Bullous ichthyosiform erythroderma,
Ichthyosis bullosa of Siemens,
Harlequin ichthyosis
this ppt is about how to approach to a patient with non syndromic congenital ichthyosis..slide 32 is overall summary to approach to a patient with ichthyosis and last two slides are just about acquired ichthyosis..
by dr zuhaib alam mehsud,dermatology unit Hmc PESHAWAR
Skin involvement in rheumatic diseases/ DOI 10.13140/RG.2.2.10743.32169Enida Xhaferi
Skin disorders are observed in a variety of rheumatologic conditions and constitute the primary features in lupus erythematosus, dermatomyositis and systemic sclerosis. Skin involvement is also observed in systemic vasculitides, rheumatoid arthritis, Sjögren syndrome, psoriatic arthritis, systemic-onset juvenile rheumatoid arthritis, and relapsing polychondritis. It is important for the clinician to recognize and discern the most common cutaneal lesions and patterns encountered in patients with rheumatic diseases (like makule, papule, nodul, plaque, purpura, petechia, pustul, squam, erosion, erythema, onychodystrophy, onycholysis, urticaria, butterfly rash, Gottron papules and sign etc) because they provide clues regarding the systemic involvement of the pathology, diagnosis, therapeutic approach and prognosis. Skin biopsies are usually useful in determining the precise nature of the skin disorder. Below are presented briefly the major skin manifestations observed in lupus erythematosus, dermatomiositis, scleroderma and rheumatoid arthritis
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.
They are a heterogenous group of inherited disorders of epidermal differentiation featuring excessive scaling, Ichthyosis vulgaris,
X-linked recessive ichthyosis,
Lamellar ichthyosis,
Non-bullous ichthyosiform erythroderma,
Bullous ichthyosiform erythroderma,
Ichthyosis bullosa of Siemens,
Harlequin ichthyosis
this ppt is about how to approach to a patient with non syndromic congenital ichthyosis..slide 32 is overall summary to approach to a patient with ichthyosis and last two slides are just about acquired ichthyosis..
by dr zuhaib alam mehsud,dermatology unit Hmc PESHAWAR
Skin involvement in rheumatic diseases/ DOI 10.13140/RG.2.2.10743.32169Enida Xhaferi
Skin disorders are observed in a variety of rheumatologic conditions and constitute the primary features in lupus erythematosus, dermatomyositis and systemic sclerosis. Skin involvement is also observed in systemic vasculitides, rheumatoid arthritis, Sjögren syndrome, psoriatic arthritis, systemic-onset juvenile rheumatoid arthritis, and relapsing polychondritis. It is important for the clinician to recognize and discern the most common cutaneal lesions and patterns encountered in patients with rheumatic diseases (like makule, papule, nodul, plaque, purpura, petechia, pustul, squam, erosion, erythema, onychodystrophy, onycholysis, urticaria, butterfly rash, Gottron papules and sign etc) because they provide clues regarding the systemic involvement of the pathology, diagnosis, therapeutic approach and prognosis. Skin biopsies are usually useful in determining the precise nature of the skin disorder. Below are presented briefly the major skin manifestations observed in lupus erythematosus, dermatomiositis, scleroderma and rheumatoid arthritis
Hereditary disorder of keratinization characterized by expanding atrophic anular patch(es) surrounded by prominent keratotic ridge called the cornoid lamella
Молекулярная гетерогенность пузырчаток: парадигма буллезного эпидермолиза (Mo...Fund BELA / Фонд БЭЛА
Материалы с I Евразийской Конференции по редким заболеваниям и редким лекарствам и III Всероссийской Конференции по редким заболеваниям и редко применяемым медицинским технологиям
«Дорога жизни».
21-23 июня 2012 года в гостиничном комплексе «Измайлово»
Nutritional deficiencies and dentofacial growth /certified fixed orthodontic ...Indian dental academy
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about various genodermatoses and classified according to clinical presentation.
mentioned are introduction clinical features histology management of each disease.
Ichthyosis medical information |management | all aspects martinshaji
Ichthyosis is a condition that causes widespread and persistent thick, dry, "fish-scale" skin.
The skin of a person with ichthyosis is rough, dry and scaly and needs to be regularly moisturized
There are at least 20 different types of ichthyosis. Some types are inherited at birth and other types are acquired during adulthood. There's no cure for ichthyosis, but a daily skincare routine usually keeps the symptoms mild and manageable.
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14. Difference between I.V. and X-linked Ichthyosis (clinically) before 3 months of age in between 3-12 months of age Onset Coarser and darker Finer Scales Only male Both sexes Affected sex Palms and soles Flexures and face Spared area Absent Present Association with KP & AD Present Absent Eye involvement & hypogonadism Abdomen is more affected than back Affected area Moderate Mild Severity X-linked Ichthyosis Ichthyosis vulgaris Traits
33. Features of different types of Ichthyosis Becomes less severe with age Severe Moderate Mild Severity All over body, bullae and hyperkeratotic lesions over knee, elbows; keratoderma All over body, very severe, involves flexure, neck, face, scalp, scaly palms and sole All over body Only men All over body Distribution Abnormal distribution of keratinocytes Transglutaminase 1 Steroid sulphatase enzyme Flaggrin protein Defect AD AR X-linked AD Inheritance EK Lamellar Ichthyosis X-linked Ichthyosis IV Features
34. Features of different types of Ichthyosis (contd.) Systemic and oral retinois + antibiotics Retinoids- acitretin Emollients Emollients Treatment Erythroderma Scales are large and quadrangular, ectropion and eclabium Scales are black and brown, eye involvement, cryptorchidism Fine scales, improves in summer Other features Birth- bullae, erythroderma. Birth- collodion baby Before 3 months of age 3-12 months of age Onset EK Lamellar Ichthyosis X-linked Ichthyosis IV Features Tends to become less severe with age Causes serious disability Good Good Prognosis None None Palms and soles Flexures and face Spared areas