Childhood pemphigus vulgaris is a challenging diagnosis

Abstract

Pemphigus Vulgaris (PV) is an uncommon autoimmune and blistering mucocutaneous disease. Childhood Pemphigus Vulgaris (CPV) is a pediatric variant of PV, which affects children below 12 years, being very rare among children under 10 years of age. CPV has similar clinical, histological, and immunological features as seen in PV in adults. The mucocutaneous clinical presentation is the most common in both age groups. Vesicles and erosions arising from the disease usually cause pain. A few CPV cases have been reported in the literature. This study reports a case of an 8-year-old male patient with oral lesions since the age of 3 years, and the diagnosis of pemphigus was achieved only 2 years after the appearance of the initial lesions. CPV remains a rare disease, making the diagnosis of this clinical case a challenge due to its age of onset and clinical features presented by the patient. Therefore, dentists and physicians should know how to differentiate CPV from other bullous autoimmune diseases more common in childhood.

Keywords: Child; Fluorescent Antibody Technique, Direct; Pemphigus.

Figure 1. Lesions at the first appointment. A – Ulcerative lesions in the ventral surface of the tongue; B – Lesions with an ulcerative surface in lower labial mucosa (biopsy site); C – Ulcers in glans and foreskin; D – Ulcer in the mucosa of the lower eyelid.

Figure 2. Photomicrographs of the biopsy specimen. A – Intraepithelial blister with a single layer of basal cells recovering the conjunctive tissue and lymphoplasmacytic inflammatory infiltrate with eosinophils (H&E, 5X); B – High magnification of image A (H&E, 20X); C – Ulcered area with grouped acantholytic cells (*) with degenerative alterations (H&E, 40X); D – View of the inferior area of epithelial blister and suprabasal slit showing tiny area with rare basal cells still attached to the conjunctive tissue (H&E, 40X); E – Positive direct immunofluorescence showing IgG3 in the intercellular space of cells of the remaining epithelium basal layer on the conjunctiva.

Figure 3. Lesions on the last follow-up. A – Ulcerative lesions in the ventral surface of the tongue; B – Ulcers in the transition between alveolar mucosa and lower labial mucosa; C – Remission of lesions in glans and foreskin; D – Absence of lesion in the lower eyelid.