Clinicl aproch to blistering dissorder
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Clinicl aproch to blistering dissorder

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Infection ...

Infection
Herpes simplex
Herpes zoster
Varicella
Bullous impetigo
SSSS
Genetic
Epidermolysis bullosa
Hailey-Hailey disease
Incontinentia pigmenti

Immunobullous
Pemphigus group of diseases
Paraneoplastic pemphigus
Bullous pemphigoid
Mucous membrane pemphigoid
Linear IgA disease
Dermatitis herpetiformis
Epidermolysis bullosa acquisita
Mechanical
Friction blister

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    Clinicl aproch to blistering dissorder Clinicl aproch to blistering dissorder Presentation Transcript

    • SEMINAR PRESENTATION Clinical approach to a case of Blistering disorder MODERATOR: Dr. Amit Malhotra
    • Introduction • A blister is a fluid filled cavity formed within or beneath the epidermis. • Can be categorized as vesicles or bullae. • Vesicle- < 0.5 cm in diameter • Bulla- > 0.5 cm in diameter • Blisters are an obvious sign of disease that always draw attention of patient and physician.
    • Common causes of blistering • Infection – – – – – Herpes simplex Herpes zoster Varicella Bullous impetigo SSSS • Genetic – Epidermolysis bullosa – Hailey-Hailey disease – Incontinentia pigmenti
    • • Immunobullous – – – – – – – Pemphigus group of diseases Paraneoplastic pemphigus Bullous pemphigoid Mucous membrane pemphigoid Linear IgA disease Dermatitis herpetiformis Epidermolysis bullosa acquisita • Mechanical – Friction blister
    • • Dermatitis – Allergic contact dermatitis – Irritant dermatitis • Drugs – Bullous FDE – Erythema multiforme – SJS/ TEN • Metabolic – Diabetic bullae – Porphyria
    • • Disorders, in which blistering is the primary event are traditionally termed as blistering disorders or vesiculobullous disorders. • This group includes hereditary blistering disorders and immunobullous diseases.
    • Approach • History • Clinical examination
    • Age of onset Pemphigus vulgaris Middle age (40-60 yrs) Pemphigus foliaceus Middle age Paraneoplastic pemphigus Adult, children Bullous pemphigoid Elderly (60-75 yrs) Mucous membrane pemphigoid Old age (60-80 yrs) Pemphigoid gestationis Pregnant women Dermatitis herpetiformis Adult Linear IgA disease Before 5 & after 60 yrs EBA Adults & children Hailey- hailey disease Adults Epidermolysis bullosa At birth or during infancy
    • Initial site Pemphigus vulgaris Oral mucosa Pemphigus foliaceus Scalp, chest Bullous pemphigoid extremities Mucous membrane pemphigoid Oral or other mucosa Pemphigoid gestationis Periumblical, extremities Dermatitis herpetiformis Trunk, scalp Linear IgA disease Genital in children; no predilection in adults EBA Mucosa, extremities Hailey- hailey disease Friction sites
    • Mucosal involvement Pemphigus vulgaris Almost all Pemphigus foliaceus None Intercelluar IgA dermatosis Uncommon Paraneoplastic pemphigus Severe mucositis Bullous pemphigoid 10 – 40%, transient, mild Mucous membrane pemphigoid Almost all Pemphigoid gestationis Rare Dermatitis herpetiformis Rare Linear IgA disease 80% EBA 50% Hailey- hailey disease Uncommon
    • Distribution of lesions Pemphigus vulgaris Scalp, face, flexures, trunk Pemphigus foliaceus Seborrhoeic distribution Intercelluar IgA dermatosis Axillae, groins, face, scalp, proximal limbs Paraneoplastic pemphigus Upper body, palmoplantar Bullous pemphigoid Trunk, limbs, flexures Mucous membrane pemphigoid Infrequent; head, neck, upper trunk
    • Pemphigoid gestationis Abdomen, extremities Dermatitis herpetiformis Symmetrical over extensors of trunk including buttocks, elbows, knees. Linear IgA disease Perineum, face, trunk, limbs EBA Generalized, variable Epidermolysis bullosa Sites of trauma Hailey- Hailey disease Sides of neck, axillae, groins, perineum
    • Morphology of lesions Characteristics of bullae based on level of split
    • Lesions characteristics Pemphigus vulgaris Flaccid blisters, erosions, flexural vegetations Pemphigus vegetans Vesicles, pustules, erosions, vegetating Plaques Pemphigus foliaceus Scaly papules, crusted erosions, Erythroderma Intercelluar IgA dermatosis Flaccid pustules annular or circinate configuration Paraneoplastic pemphigus Polymorphous, bullae, erosions, ‘target lesions’ Bullous pemphigoid Urticated plaques, tense blisters, (milia) Mucous membrane pemphigoid Erosions, blisters, gingivitis, milia, Scarring
    • Pemphigoid gestationis Urticated plaques, tense blisters Dermatitis herpetiformis Papulovesicles Linear IgA disease Urticated plaques, annular lesions, tense blisters EBA Urticated plaques, tense blisters, milia ,Scarring Hailey- Hailey disease Flaccid vesicopustules, crusted erosions or expanding circinate plaques appear in areas exposed to friction
    • Pemphigus vulgaris A: flaccid blisters on normal skin. B: superficial blisters and erosions which take long to heal.
    • Pemphigus vegetans: heaped up vegetating plaques in flexures Pemphigus foliaceus: extensive areas of scaling and crusting and no blisters. removal of scale-crust reveals a minimally moist area.
    • Bullous pemphigoid: urticarial lesions. large hemorrhagic blisters some on normal skin
    • Pemphigoid gestationis Early pruritic erythematous stage Bullae arising on urticated erythematous skin on the thigh.
    • Chronic bullous disease of childhood: string of pearl appearance is typical Dermatitis herpetiformis: grouped vesicles develop either on normal or erythematous skin. Since the lesions are extremely itchy, they are rapidly excoriated
    • Differentiating features of Epidermolysis bullosa Type Time of inhe Clinical features present riatn ation ce MM/Nail Associated /Teeth features WeberCockyne EBS Childho AD od Localised to palms, Normal soles, waist or neck, no scarring, more in summers Hyperhydrosis good of palms, soles Koebner EBS At birth AD or infancy On occiput, back or legs in infancy, Hands or feet in childhood, no scarring Aggravated by warm weather, Hyperhydrosis of palms, soles Normal Progno sis May improv e after puberty
    • Epidermolysis bullosa Type Time of inhe Clinical features present ritan ation ce MM/Nail/Te Associated eth features Progno sis Severe in infancy, better with age DowlingAt birth AD meara EBS or infancy Herpitiform blisters on trunk, limbs, neck, No scarring MM, nails involved Milia, hyperpigme ntation seen EBS with muscular dystrophy Blisters on hands and feet then generalized Nail deformities, MM involvement , alopecia Muscle Poor weakness early or late onset, milia At birth AD or early infancy
    • Epidermolysis bullosa Type Time inhe Clinical features of ritan presen ce tation MM/Nail/ Teeth Associated features Progno sis Autosomal recessive lethal EBS At birth AR Generalised more on distal limbs Normal, oral mucosa mildly affected No scarring or milia seen Poor EBS with mottled pigmentati on At AD birth or infancy Reticulate pattern of macular pigmentation over trunk, limbs Nails involved, MM, teeth normal Punctate Improv keratoses on es with palms and age soles
    • Epidermolysis bullosa Type Time of inhe Clinical features present ritan ation ce MM/Nail Associated /Teeth features Prognosi s Poor, child may die in early infancy due to infection Herlitz JEB At birth AR or soon after birth Severe generalised blistering, skin fragility, difficult to handle child, erosions slow to heal Mm, nails teeth involved, larynx may be involved Sepsis or multiorgan failure may occur NonAt birth AR Herlitz JEB Generalised blistetrs, scalp involvement causes alopecia Involved Pigmentation Improve and nevi s with seen age
    • Epidermolysis bullosa Type Time inhe Clinical features of ritan prese ce ntatio n MM/Nail/ Associated Teeth features Progno sis JEB with pyloric atresia At birth AR Generalised skin and mucosal blisters Nail, teeth Non- bilious involved vomiting in newborn Very poor Progressive JEB 5-8 years AR Hands, feet, knees, Nail, teeth Finger elbows (sites of involved contractures, friction) deafness good
    • Epidermolysis bullosa Type Time of inhe Clinical features present ritan ation ce MM/Nail/T Associated eeth features Progno sis Dominant At birth AD dystrophic or early EB infancy Hands, feet, knees, Nail elbows (sites of dystrophy, friction) MM, teeth normal White Good papules on trunk, pasini variant Hallopeau - siemens EB At birth AR or early infancy Large, flaccid bullae at sites friction, healing slow, scarring Dystrophic nails, scarring alopecia, carious teeth Flexural contractures , esophagial strictures, inability to protude tongue Poordeath by 3-4 decade Non Hallopeau - siemens EB At birth AR Skin and mucosae are fragile Changes are localised Few complicatio ns good
    • Configuration • Grouping of blisters: dermatitis herpetiformis. • String of pearls sign- annular, polycyclic lesions often with blistering around the edge in CBDC
    • NIKOLSKIY SIGN • A positive Nikolskiy sign indicates intraepidermal cleavage and differentiates intraepidermal blisters from subepidermal blisters. • It is pathognomonic of pemphigus and staphylococcal scalded skin syndrome • The sign is best elicited by applying lateral pressure with the thumb or fingerpad on skin over a bony prominence. • This results in a shearing force that dislodges the upper layers of epidermis from the lower epidermis producing an erosion.
    • • Specifically, elicitation of the sign can help distinguish pemphigus vulgaris, which is strongly associated with the sign, from bullous pemphigoid, in which the sign is usually absent. • other diseases associated with a positive Nikolsky’s sign -toxic epidermal necrolysis, bullous impetigo, and epidermolysis bullosa
    • (a) Eliciting Nikolsky's sign on perilesional skin. Note the tangential pressure, (b) Eliciting Nikolsky's sign, peeling of skin revealing moist erosion
    • BULLA SPREAD SIGN • In the traditional "bulla spread" sign or Lutz sign, the margin of an intact bulla is first marked by a pen. • Slow, careful and unidirectional pressure applied by a finger to the bulla causes peripheral extension of the bulla beyond the marked margin. • The bulla thus extended has an irregular angulated border in pemphigus vulgaris, while a regular rounded border is observed in bullous pemphigoid or other subepidermal blistering disorders.
    • • This sign is positive in all varieties of pemphigus and many cases of subepidermal blisters, including bullous pemphigoid, DH , EBA, cicatricial pemphigoid, dystrophic epidermolysis bullosa, SJS, TEN. • Due to fragility of the roof of the blister it is usually negative in Hailey-Hailey disease and staphylococcal scalded skin syndrome.
    • Tzanck Smear • Is a quick bedside test. • A fresh blister is ruptured, the roof detached and the floor scraped using a scalpel blade. • If blister not present, then taken from erosion, after removing the crust. • The material so obtained is spread on a glass slide and stained with Giemsa stain.
    • Tzanck smear findings in bullous disorders
    • PEMPHIGUS VULGARIS • It reveals multiple acantholytic cells (Tzanck cells). • A typical Tzanck cell – – – – Large round keratinocyte Hypertrophic nucleus, Hazy or absent nucleoli, and Abundant basophilic cytoplasm. • The basophilic staining is deeper peripherally on the cell leading to a perinuclear halo.
    • Other differentials Differential History Examination Herpes simplex primary or recurrent outbreak of herpes simplex virus (HSV) vesicles associated with tenderness, burning, or tingling; HSV-1 is spread primarily through direct contact with infected saliva or other infected secretions, HSV-2 is spread primarily through sexual contact, symptoms typically start within 1 week after exposure grouped vesicles on an erythematous base, may evolve to pustules or erosions, lesions resolve within 2 to 6 weeks Herpes zoster (shingles) prior history of varicella infection, presents with prodrome of pain, itching, hyperesthesia followed by vesicular eruption painful, grouped vesicles on an erythematous base in a sensory dermatomal distribution, rarely crosses midline
    • Differential History Examination Varicella, initial viremia between days 4 acute(chickenpox) and 6; appearance of characteristic vesicular eruption on erythematous base, often referred to as "dewdrops on rose petals," low-grade fever, malaise, and headache successive crops of lesions appear over several days on trunk, face, and oral mucosa; typically lesions are in different stages of evolution from vesicles to crust and do not scar Impetigo bullae are ≥2 cm in diameter and initially clear, subsequently becoming turbid; buccal mucosa may be involved, classic facial yellowish to golden crusting, streptococcal form tends to have thicker and darker crusts typically occurs in children, very contagious, risk factors include increased humidity, poor hygiene, malnutrition and overcrowding, concomitant skin disease
    • Differential History Examination Staphylococcal typically child or adult with scalded skin renal insufficiency syndrome prodromal fever, tender skin evolve to generalized erythema with flexural accentuation and then flaccid bullae formation; Nikolsky sign present, desquamation follows starting in flexural areas; in contrast to toxic epidermal necrolysis, does not affect oral mucosa and may be a helpful clue to diagnosis Congenital syphilis primarily acrally located vesicles and bullae, may be hemorrhagic 40% of infected newborns have skin findings, neonate develops lesions within first 2 weeks of life through transplacental transmission, mother with history of secondary or tertiary syphilis
    • Differential History Examination Eczematous dermatitis (contact, nummular, and pompholytic) personal or family history of atopy, recent exposure to chemicals, personal hygiene products, fabrics, or plant allergens (e.g., poison ivy, poison oak) predominantly localized distribution of vesicles and papules with surrounding erythematous base, later lesions may be covered by scale or crusting Friction blister recent activity involving affected area (new shoes, gloves, or products) tense bullae in area of pressure or friction Miliaria exposure to hot or humid climates, pruritic or asymptomatic febrile illness in bedridden patient, papules or vesicles layered clothing preventing dissipation of heat or moisture
    • Differential History Examination Coma bullae coma from trauma, illness, or an overdose of a narcotic drug erythema with vesicles or bullae at sites subjected to pressure (hands, wrists, scapulae, sacrum, knees, heels) Bullous arthropod recent arthropod exposure in a bite reaction sensitized patient, typically present as grouped pruritic or asymptomatic blisters in patients who are otherwise well grouped pruritic or asymptomatic blisters, distribution and location of the lesions usually localized to a specific area of body (depending on causative arthropod)
    • Differential History Examination Nutritional deficiencies (zinc, biotin, niacin, essential fatty acids) inherited or acquired deficiency, breastfed newborns, history of parenteral nutrition, characteristic cutaneous finding is a photosensitive eruption (preferentially involving the face, neck, upper chest, dorsal hands, and extensor forearms), which worsens in spring and summer dermatitis is bullous or pustular, periorificial and acral locations, associated erythematous eroded, crusted patches; with repeated sun exposure, the involved areas become thickened, scaly, and hyperpigmented Diabetic bullae (bullosis diabeticorum) longstanding history of diabetes, spontaneously healing blisters within 4 to 5 weeks of onset painless noninflammatory blisters typically on acral locations, including amputation sites
    • Differential History Porphyria cutanea photosensitivity, fragility of suntarda exposed skin that results in blistering and erosions of the dorsal hands, forearms, ears, feet, and face; ingestion of alcohol, estrogens, and polychlorinated cyclic hydrocarbons exacerbates condition Pseudoporphyria cutanea tarda Examination tense blisters on sunexposed skin, heal with scarring, dyspigmentation, and milia; hypertrichosis, sclerodermatous thickenings, and scarring alopecia hemodialysis, drug exposures bullae on sun-exposed body (NSAIDs, furosemide, nalidixic acid, areas (face, ears, dorsal tetracycline), skin fragility, hands, forearms) photosensitivity, absence of hypertrichosis, and skin sclerosis
    • Differential History Examination Incontinentia pigmenti X-linked dominant, female infant 4 to 6 weeks old with vesicles in a patterned distribution (Blaschko lines), cutaneous features evolve through 4 stages from infancy to adolescence noninflammatory vesicles in a patterned distribution (Blaschko lines), abnormalities of teeth, eyes, hair Bullous ichthyosiform erythroderma (epidermolytic hyperkeratosis) presents at birth, or shortly after, with erythema, blistering, or peeling; may be confused with staphylococcal scalded skin syndrome or epidermolysis bullosa widespread erythema, blistering and peeling infant with or without palmarplantar involvement
    • Differential History Examination Mastocytosis acquired solitary or widespread cutaneous eruption, lesion periodically urticates and blisters then returns to original form 5 mm to 15 mm papules, yellow-brown to yellow-red in color; edema, urtication, and vesicle and bullae formation, urticaria surrounding erythematous flare when rubbed (Darier sign) Bullous lupus erythematosus occurs in patients with a diagnosis of systemic lupus, sun-exposed skin is preferentially involved lesions are not pruritic or symmetric, do not have a predilection for extensor surfaces of arms, elbows, knees, or scalp; vesicles and bullae typically photodistributed or widespread, asymptomatic
    • Differential History Examination Erythema multiforme ingestion of new medications in the days or weeks before onset, implicated medications include antibiotics (trimethoprimsulfamethoxazole), anticonvulsants (lamotrigine), NSAIDs, and allopurinol characterized by atypical targetoid lesions, macules, vesicles, bullae on palms and soles; may be generalized Stevens-Johnson syndrome more fulminant form of erythema multiforme with systemic and mucosal involvement of <10% of body surface area, severe mucocutaneous reaction with prodrome of fever, malaise, chills, 1 day to 2 weeks before onset palms, soles, and extensor surfaces with macules, may evolve to papules, vesicles, bullae, urticarial plaques, or confluent erythema; center of lesions purpuric, vesicular, or necrotic imparting targetoid appearance, secondary infection follows
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