Pemphigus

Published on 11/06/2015 by admin

Filed under Pediatrics

Last modified 22/04/2025

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Crusted lesions on the arm of a child with pemphigus foliaceous.

Completely eroded lips in adolescent with pemphigus vulgaris. His mouth was also severely eroded.

Trauma-induced erosions in an infant with pemphigus vulgaris.

Hemorrhagic crusts on lips and eroded conjunctiva in a boy with PNP and Castleman’s tumor.

There are three forms of pemphigus.

Localized PF may be treated with super-potent topical steroids. More widespread disease requires oral methylprednisolone 1 mg/kg/day. Long term remission is usually achieved.

PV treatment often requires methylprednisolone 1–2 mg/kg/day and mycophenalate mophetil 500–1000 mg twice a day. IVIG given in cycles is used to decrease the amount of the immunosuppressive drugs required. Rituximab may lead to long-term remission.

Treatment of PNP is similar to therapy of PV. Removal of the Castleman’s tumor may be beneficial. Mortality is high.

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Crusted lesions on the arm of a child with pemphigus foliaceous.

Completely eroded lips in adolescent with pemphigus vulgaris. His mouth was also severely eroded.

Trauma-induced erosions in an infant with pemphigus vulgaris.

Hemorrhagic crusts on lips and eroded conjunctiva in a boy with PNP and Castleman’s tumor.

There are three forms of pemphigus.

Pemphigus foliaceous (PF)

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