Intra-epidermal intercellular deposition of IgG on immunofluorescence is seen in :
Correct Answer: Pemphigus
Description: Ans. a. Pemphigus (Ref: Fitzpatrick 7/ep432-441; Rooks 8/ep40.27, 13.19, 10.12-10.28)Granular deposition of IgA at dermal papillae on immunofluorescence is seen in pemphigus.A 30-year-old male presents with flaccid bullae on an erythematous base and erosions over the oral mucous membrane. The blisters developed painful erosions rupture. The clinical features point towards a diagnosis of pemphigus vulgaris, which is characterized by a fishnet pattern of intraepidermal IgG deposition on immunofluorescence.DisorderImmunofluorescencePatternTarget antigenPemphigus vulgarisIntercellular, intraepidermalIgG, fish netQDesmoglein 3QPemphigus foliaceusIntercellular, intraepidermalIgG, fish netQDesmoglein 1QParaneoplastic pemphigusIntercellular and subepidermalIgG, fish netQPlakins (desmoplakin, envoplakin, periplakin, BP 230)Bullous pemphigoidBasement membrane zoneIgG, linearBP230> BP180Herpes gestationalisBasement membrane zoneIgG, linearBP230 > BP 180Dermatitis herpetiformisDermal papillaeIgA, granular(?) Epidermal tissue trnasgl utam inaseQLinear IgA diseaseBasement membrane zoneIgA, linearBP180PemphigusAn autoimmune disease characterized by acantholysis induced by deposition of lgG antibodies in the intercellular area of epidermisThe commonest variant is Pemphigus vulgaris, seen in adults mainlyMainly involving the upper part of the bodyCharacterized by cutaneous (thin-walled, delicate and flaccid bullae)Q and mucosal blistersMucosal lesions are present in all patients, oral mucosa most frequently involved, painful erosions extend peripherally with the shedding of the mucosa.Erosions are frequently secondarily infected (by bacteria in skin and Candida in mucosa), extensive lesions are associated with water and electrolyte imbalance.Associated with Nikolsky sign and Bulla spread sign or Asboe Hansen sign.Histology (Biopsy is best taken from the edge, from perilesional skin)Bulla is intra-epidermal, the split being suprabasalPresence of acantholytic cells (rounded keratinocytes present in the blister cavity and at the edge of the blister)A row of tombstone appearanceDirect Immunofluorescence (DIF):Done on the skin, shows intercellular deposits of IgG and C3 giving a fishnet appearanceTreatment:Systemic steroids and immunosuppressants.
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