Childhood pemphigus vulgaris: Report of two cases with emphasis on diagnostic approach

Oral Medicine

Childhood pemphigus vulgaris (CPV) is a rare entity with clinical features and management primarily similar to those of adults; diagnosis may be difficult due to clinical similarity to other disorders such as herpetic gingivostomatitis which is more prevalent in this age group.

We report two cases of CPV with emphasis on diagnostic approach. While treating such patients, it is imperative to diagnose CPV in the early stages to prevent serious morbidity and to adjust drug dosage to minimize side effects.

Introduction : Pemphigus vulgaris (PV) is an autoimmune blistering disorder of mucous membranes and skin and commonly affects adults between ages of 40 and 60 years.

Childhood PV (CPV) is a rare entity (0.1–0.5 cases/100,000 inhabitants/year) and comprises only a small minority of the vesiculobullous disorders seen in childhood.

The age of the onset varies from 13 to 19 years with similar clinical, histological, and immunological features as seen in adult PV (APV). Autoantibodies are primarily directed against desmoglein-3 (Dsg3) with or without the concomitant involvement of Dsg1.

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When mucocutaneous lesions are present, PV patients can also present with antibodies against Dsg1 which is indicative of a poorer prognosis of the disease. These autoantibodies lead to the destruction of cell–cell cohesion and blister formation.

The sequence of diagnostic procedures in a suspected case of vesiculobullous disorder after a detailed history and comprehensive clinical examination may include Tzanck test (TT) as the first step, the findings of which can be further substantiated by routine histopathology and immunofluorescence.


° contempclindent.org
° Varun Surya / Priya Kumar / Sunita Gupta / Aadithya B Urs

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