Lichen planus pemphigoides: report of two cases
Authors: Demirçay Zeynep , Baykal Can , Demirkesen Cuyan 
Published: March 2003

Case 1 A 73‐year‐old woman with a 5‐month history of widespread lichenoid eruption developed disseminated blisters. She had insulin‐dependent diabetes mellitus and renal tuberculosis treated with isoniazide (300 mg/day), rifampin (600 mg/day), ethambutol

(1.5 g/day) and pyrazinamide (2 g/day) following right nephrectomy. Antituberculous therapy which had been begun 2 months before the onset of lichenoid eruption was stopped by the patient following the appearence of blisters. Examination revealed lichenoid plaques on the face, neck, trunk and extremities and tense bullae on the neck and extremities (Fig. 1a). The bullae were either on lichenoid plaques or normal appearing skin. Some of the blisters were hemorrhagic. Two skin biopsies were performed. The first biopsy from a blister with a lichenoid base on the arm, showed typical changes of lichen planus (LP) with subepidermal cleft formation. The second biopsy from a blister on normal appearing skin on the trunk showed subepidermal bulla with perivascular inflammatory cell infiltrate of eosinophils and lymphocytes. Direct immunofluorescence (DIF) of perilesional skin showed linear deposition of IgG and C3 along basal membrane zone (BMZ). Direct immunofluorescence on salt‐split skin revealed epidermal binding of immunoreactants. Indirect immunofluorescence showed a circulating IgG autoantibody binding to the BMZ at a titre of 1/200. Immunoblot analysis of her serum recognized minor 180 Kd BP antigen. An initial trial of dapsone (100 mg/day) for 2 weeks was not effective. She was then treated with oral methylprednisolone (32 mg/day), which induced rapid improvement in her skin lesions (Fig. 1b). After 2 months of treatment, corticosteroid was stopped and the patient stayed in remission for 1 year till she died due to myocard infarction.

(a) Lichenoid papules and hemorrhagic blisters on the face and the neck. (b) After 2 months of oral methylprednisolone therapy

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Case 2 A 64‐year‐old man with a 2‐year history of pruritic papules on his hands developed a bullous eruption on his extremities. Physical examination revealed violaceous flat‐topped papules on the dorsum of the hands and blisters on the extremities (Fig. 2). Some of the blisters occured on an erythematous base while others occurred on normal appearing skin. Mucous membranes were not affected. A skin biopsy taken from a blister revealed subepidermal bulla containing fibrin, eosinophils and neutrophils and perivascular inflammatory infiltrate of lymphocytes and eosinophils in the dermis. Adjacent to the blister were changes typical of LP (Fig. 3). Direct immunofluorescence showed linear deposition of IgG and C3 along BMZ. Direct immunofluorescence on salt‐split skin showed epidermal binding of immunoreactants. The patient's skin lesions cleared rapidly after oral methylprednisolone (48 mg/day) and dapsone (100 mg/day) started. Methylprednisolone and dapson was discontinued after 12 and 16 weeks, respectively. There has been no recurrence during the follow‐up period of 1 year.

Crusted and eroded lesions on an erythematous base on the lower leg

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Subepidermal blister with preserved configuration of the papillary dermis at the floor. Adjacent epidermis displayed hyperkeratosis, hypergranulosis and a band like lichenoid infiltration (hematoxylin and eosin × 100)

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