Pemphigus foliaceus, var. Herpetiformis and Sulphonamide

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2015
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SPRINGER INTERNATIONAL PUBLISHING
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Aoki, V.; Miyamoto, D.. Pemphigus foliaceus, var. Herpetiformis and Sulphonamide. In: . CLINICAL CASES IN AUTOIMMUNE BLISTERING DISEASES: SPRINGER INTERNATIONAL PUBLISHING, 2015. p.99-106.
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A 43-year-old woman presented a 2-year history of tender blisters and erythematous and crusted excoriations on the face, trunk and proximal extremities. During her first examination in 1995, erosions and yellow crusts on her face, thorax, upper torso and proximal limbs were present, without mucosal involvement. Laboratory profile confirmed the diagnosis of pemphigus foliaceus, as follows: histopathological analysis exhibited subcorneal abscess with mild spongiosis and scanty acantholytic cells; neutrophilic and linfohystiocitic perivascular infiltrate was present in the superficial dermis; direct immunofluorescence showed intercellular IgG deposits within the entire epidermis; indirect immunofluorescence revealed circulating IgG antibodies (titer=1:1,280) and positive ELISA index (99, recombinant desmoglein 1). Treatment with systemic corticosteroid (prednisone 1 mg/kg/day) was initiated. Clinical remission was achieved after 2 months, followed by gradual corticosteroid tapering. The patient presented reactivation when receiving prednisone 10 mg/day, with vesicles with a herpetiform arrangement on erythematous plaques on her legs, with intense pruritus (Fig. 13.1). © Springer International Publishing Switzerland 2015.
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