Sporadic Pemphigus Foliaceus in a 3-Year-Old Vietnamese Girl: A Case Report and Literature Review

Abstract

Pemphigus foliaceus is an uncommon autoimmune intraepidermal blistering disease characterized by immunoglobulin (Ig) G autoantibodies that attack desmoglein-1 in the epidermis. There are two predominant forms of pemphigus foliaceus, sporadic and endemic. Sporadic pemphigus foliaceus is known to be more prevalent in middle-aged and elderly people and to be extremely rare in children. Less than 40 nonendemic pediatric pemphigus foliaceus cases have been documented in the literature. This report documents a case of sporadic pemphigus foliaceus in a 3-year-old Vietnamese girl who presented with generalized scaling and crusted erosions over the body.

Figure 1

Large flaky scales in the background of diffuse erythema on the face of a pemphigus foliaceus patient.

Figure 2

Diffuse scaly and crusted lesions on the trunk and extremities of a pemphigus foliaceus patient.

Figure 3

Skin biopsy of a pemphigus foliaceus patient with H&E staining shows a cleft within the subcorneal layer and superficial acantholysis. H&E, hematoxylin and eosin.

Figure 4

Positive DIF findings demonstrate C3 and IgG deposition in the upper epidermis of a pemphigus foliaceus patient. DIF, direct immunofluorescence; Ig, immunoglobulin.