Anti-laminin-c1 pemphigoid in an epitope spreading phenomenon, successfully treated with rituximab Dear Editor, Anti-laminin-c1 pemphigoid, also known as anti-p200 pemphigoid, is a rare subepidermal autoimmune blistering disorder with binding of autoantibodies (AAbs) to the dermal side on indirect immunofluorescence (IIF) microscopy. Other blistering disorder with binding of AAbs to the dermal side of salt-split skin includes epidermolysis bullosa acquisita (EBA), mucous membrane pemphigoid (MMP), cicatricial pemphigoid, and linear IgA bullous dermatosis (LABD). Dermal binders comprise about 15% of pemphigoid sera. 1 Anti-p200/laminin-c1 pemphigoid was recently shown to be the most frequent pemphigoid disease with dermal binding AAbs, comprising 78.7% of 141 patients, 2 and occurs more frequently in males and Asians. 3 We report a 71-year-old Chinese man who was diagnosed with LABD in 2003 at the age 55. Histology was consistent on two skin biopsies which showed a subepidermal blister with neutrophils and occasional eosinophils, with linear IgA deposition at the dermo-epidermal junction (DEJ) on direct immunofluorescence (DIF). He was initially treated with dapsone but was switched to colchicine and prednisolone after he developed erythema multiforme. He had required prednisolone of up to 40 mg/day before he was lost to follow-up. Sixteen years later , MBBS, FRCPA, FAMS
A biliary echography showed choledochal lithiasis. Yellow urticaria was diagnosed. The patient received intravenous antihistamines, with rapid improvement.Yellow urticaria is an unusual urticaria variant that appears in patients with hyperbilirubinaemia. It differs from the usual type because of the yellow colour of the hives. This colour seems to be due to the diffusion of bilirubin to the interstitial extravascular sector of the dermis due to the increased capillary permeability induced by urticaria. 1,2 The originality of our 2 cases show that the colour of the lesions should lead to suspect hyperbilirubinaemia. The origin of hyperbilirubinaemia can be hepatic, haemolytic or enzymatic defect. Most cases of yellow urticaria reported were associated with a liver failure with cholestasis. [1][2][3]
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