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Bullous Pemphigoid in a Patient with Systemic Sclerosis (Scleroderma)

Posted Sep 11 2009 4:56pm

65-year-old Caucasian man with scleroderma presented for evaluation of 6 months of intense pruritus and recurrent cutaneous ulcerations. He began to develop ulcerated lesions on both the upper and lower extremities thought to be secondary to self-inflicted trauma from scratching areas of dry thickened scleroderma skin. Several times, these lesions became secondarily infected and required topical and systemic antibiotic therapy. It was noted that the ulcers seemed to respond to topical application of triamcinolone 0.1% cream.

Examination on presentation revealed diffuse cutaneous sclerosis with taut scaly skin over the dorsum of the hands, face, arms, trunk, and legs. Superimposed on areas of scleroderma skin were multiple papules, becoming confluent in some areas. The papules were erythematous with fine overlying scale and areas of lichenification. Well demarcated punched-out ulcerations were also present, particularly over the lower extremities ( Figure 1 ).

Mucous membranes and conjunctivae were normal. No bullae were present on examination, but he reported previously noting thin blisters on his legs.

H&E staining of the biopsy specimen from the right shoulder revealed acute spongiotic dermatitis with focal substitutive collagenosis, consistent with nonspecific eczematous change superimposed on scleroderma. Direct immunofluorescence performed on the biopsy specimen showed heavy linear deposition of IgG and C3 along the basement membrane zone ( Figure 2 ), a finding that is diagnostic for bullous pemphigoid.

He was given mycophenolate mofetil 1 g PO bid, and the prednisone was increased to 50 mg PO qam for 4 weeks before being reduced back to 40 mg PO qd. He responded to this therapy with improved pruritus, healed ulcerations, and resolution of papules. He currently is doing well with inactive pemphigoid skin disease, taking mycophenolate mofetil 1 g PO bid and prednisone 5 mg PO qod.

Although a case of a 73-year-old woman with generalized morphea who developed bullous pemphigoid after receiving whole-body UVA-1 phototherapy is reported1, the coexistent systemic sclerosis (scleroderma) with bullous pemphigoid is not described in the literature.

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