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Benign familial chronic pemphigus

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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This is an autosomally dominantly inherited Ca2+ ATPase disorder. Mutation in the ATP2C1 which encodes the human secretory pathway Ca2+/Mn2+ ATPase (hSPCA1) has been identified as the cause (1).

The typical presentation is with lesions similar to intertrigo that are refractory to conventional treatment.

There is often a family history of similar lesions.

The neck, groin, axillae, and back are generally affected. There might be intense itching or a burning feeling which may become worse by friction, heat, or sweating.

Secondary bacterial, fungal, and viral infections can be seen frequently (2).

Lesions do not usually appear until adult life (in their thirties or forties).

Signs and symptoms are likely to be worse during summer months compared to winter months (2).

Skin biopsy confirms the diagnosis (2).

Although there is no effective treatment method, topical corticosteroids and soothing compresses can be used to relieve symptoms. Antibiotics and antifungals can be used for the treatment of secondary infections (2).


  1. Szigeti R, Kellermayer R. Autosomal-Dominant Calcium ATPase Disorders. JID 2006;126:2370-2376.
  2. Xia Y, Vonhilsheimer G.E. Photo quiz - Pruritic Rash in the Intertriginous Areas. AFP 2006;74(6).

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