PEMPHIGUS FOLIACEUS

By Gary M. White, MD

Pemphigus foliaceous


Pemphigus foliaceous (PF) is a superficial autoimmune blistering disorder (ABD) of which there are two main types: 1) Classic, 2) Endemic.

Endemic Pemphigus Foliaceus

An endemic type also known as fogo selvageum (FS) occurs primarily in Brazil and mainly affects children and young adults. Exposure to hematophagous black flies possibly is related to the cause of the disease. DIF shows intercellular IgG and/or C3.  A new focus of endemic PF has been identified in El Bagre, Colombia [JAAD 2003;49;599 and 609].  It has features similar to Senear-Usher syndrome.  

Clinical

Erosions, bulla and crusted plaques affect the face, chest and elsewhere. Some patients may have their disease exacerbated by UV exposure. Annular plaques with vesicles at the periphery occur in the pemphigus herpetiformis variant.

Some cases without bulla are initially misdiagnosed as eczema, or intertrigo (when occurring in the folds).

Rarely, the patient may show prominent pustular skin lesions [BJD 2001;145;132].

Treatment

Prednisone 20-60 mg/day is usually tried initially. Rituximab is an excellent second line agent [BJD 2015;172;1420]. A typical dosing schedule might be 1 gram on days 1 and 15. To avoid secondary infection, the authors recommended avoiding prolonged high-dose steroids and as well as avoiding a third immunosuppressive agent. Add infection prophylaxis (co-trimoxazole and valaciclovir) when more immunosuppression than prednisone and rituximab is needed.

Azathioprine may be added to prednisone. Hydroxychloroquine can be an effective steroid-sparing agent especially in patients whose disease is exacerbated by sunlight. Prednisone and methotrexate have been used in combination.

Plasma exchange successfully controlled one case of PF over a 10 year period [ADV 1998;78;295]. IVIG was very effective in one study of 11 patients [JAAD 2002;46;42].

Additional Pictures

Pemphigus foliaceous

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