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.

CLINICAL FEATURES

There are three forms of pemphigus.

Pemphigus foliaceous (PF) is caused by circulating antibodies to the 50-kd portion of the 160-kd desmoglomal protein desmoglein 1. PF patients develop superficial blisters which rupture quickly, leaving erosions surrounded by erythema. They heal with crusting and scaling. Lesions are most common on the scalp, face, neck and upper trunk. Once lesions appear they tend to be persistent. Mucous membrane lesions are minimal or absent. The patient may complain of pruritus or a stinging pain. An endemic form is seen in Brazil (Fogo selvagem). PF tends to be less severe than pemphigus vulgaris (PV).

Pemphigus Vulgaris (PV) is caused by circulating antibodies to desmoglein 3. In PV, oral lesions usually present for months before the development of skin lesions. The oral lesions are seen as painful erosions of the lips, tongue and buccal mucosa. The pain from the oral lesions may limit eating and drinking, leading to malnutrition. Other mucosal sites may be involved. After a few months, flaccid blisters and crusted lesions may appear anywhere on the body. In a variant of PV, pemphigus vegetans, verrucous, malodorous, lesions develop in the groin and axilla.

Paraneoplastic pemphigus (PNP) is usually associated in children with Castleman’s tumor, a thoracic B lymphocyte hyperplasia. Antibodies against desmoplakin 1, desmoplakin 2, envoplakin, periplakin and desmoglein 3 are found. PNP begins with severe, widespread oral disease. Target-like lesions on the skin and severe cuticle involvement may be seen. Oral lesions interfere with nutrition, and weight loss is seen. Severe pulmonary involvement and sloughing of airway mucosa (bronchiolitis obliterans) occurs.

TREATMENT

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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