CHILDHOOD PEMPHIGUS VULGARIS WITH UNUSUAL AND AGGRESSIVE CLINICAL PRESENTATION

Abstract

Childhood pemphigus vulgaris (CPV) is an uncommon disease involving the skin and mucous membranes, and few cases of CPV have been reported. This study reports a case of a 5-year-old male patient with oral lesions since he had 3 years old. In the clinical examination, he presented buccal opening limitation, hoarseness, healed lesions in the skin, and painless ulcers in oral, genital, and ocular mucosa. The oral lesions involved lips, labial mucosa, tongue, palate, oral commissures, lower alveolar mucosa, buccal mucosa, and gum. Posteriorly, videolaryngoscopy showed lesions in the pharynx and larynx. An incisional biopsy and direct immunofluorescence from a sample of the lower lip mucosa was performed, which confirmed the diagnosis of CPV. The patient was treated with systemic medication, which improved his clinical conditional. However, the lesions remain in labial mucosa and ventral surface of the tongue. The patient has a clinical follow-up of 2 years. Childhood pemphigus vulgaris (CPV) is an uncommon disease involving the skin and mucous membranes, and few cases of CPV have been reported. This study reports a case of a 5-year-old male patient with oral lesions since he had 3 years old. In the clinical examination, he presented buccal opening limitation, hoarseness, healed lesions in the skin, and painless ulcers in oral, genital, and ocular mucosa. The oral lesions involved lips, labial mucosa, tongue, palate, oral commissures, lower alveolar mucosa, buccal mucosa, and gum. Posteriorly, videolaryngoscopy showed lesions in the pharynx and larynx. An incisional biopsy and direct immunofluorescence from a sample of the lower lip mucosa was performed, which confirmed the diagnosis of CPV. The patient was treated with systemic medication, which improved his clinical conditional. However, the lesions remain in labial mucosa and ventral surface of the tongue. The patient has a clinical follow-up of 2 years.