Pemphigus erythematosus

Pemphigus erythematosus

Pemphigus erythematosus (p. erythematosus, Senear-Usher syndrome) is a mild localized form of superficial pemphigus with the histological and immunofluorescent findings of p. foliaceus combined with features of lupus erythematosus. In general, the latter is subclinical, being suggested only by laboratory findings, but there are also rare reports of full-blown systemic disease being present. The condition shows a worldwide distribution and a slight female predominance. Exceptionally, it has been described in children although immunological confirmation of the diagnosis is available in only one case.

Clinically, it is commonly confined to the head, neck, and upper trunk, and typically resembles p. foliaceus. Lesions are erythematous, scaly, and crusted, with or without superficial vesicles, blisters or erosions. Facial involvement often shows a butterfly distribution reminiscent of lupus erythematosus or seborrheic dermatitis. Mucous membrane involvement is exceedingly rare.

There are reports of p. erythematosus developing after treatment with a number of drugs, notably D-penicillamine, and there are also instances attributed to therapy with propranolol, captopril, pyritinol, thiopronine, ceftazidime, and cefuroxime. P. erythematosus has also been described as a complication of heroin abuse.

P. erythematosus may rarely be associated with thymoma. Typically, the thymoma precedes the onset of cutaneous lesions, which often present following thymectomy. Most tumors have been benign but one malignant variant has been documented. P. erythematosus may also be a manifestation of paraneoplastic pemphigus.

Source: P. McKee, J. Calonje - McKee's Pathology of the Skin (Elsevier)

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