INTRODUCTION: pemphigus vulgaris is a serious and infrequent disease in children. Its timely diagnosis and treatment allows modifying its prognosis. The objective is to describe its clinical characteristics, and the diagnostic and therapeutic approach of this uncommon autoimmune blistering disease in children. CLINICAL CASE: 2-year-old male patient, previously healthy. He initially presented generalized dermatosis with maximum lesion areas at the umbilical region, limbs and genitals; characterized by blisters, some denuded, and of easy bleeding, without mucosal involvement nor fever. Bullous impetigo was diagnosed and topical and systemic antibiotic treatment was started, showing no clinical improvement. He developed extension of the lesions with oral and anal mucosal involvement. The histologic and direct immunofluorescent study of lesions and perilesional skin confirm the diagnosis of pemphigus vulgaris. The patient started treatment with corticosteroids and immunomodulatory agents with good clinical response. CONCLUSIONS: Due to the similarity with other more prevalent infectious and inflammatory diseases, a high index of suspicion is required in order to avoid delays in the diagnosis and the start of treatment. In patients with blisters with an unexpected clinical evolu tion, it is necessary to conduct a joint evaluation with a dermatologist and to assess the opportunity of performing a biopsy of the lesion and perilesional skin for histological study and direct immunofluo rescence, which will allow diagnostic confirmation.
INTRODUCTION:pemphigus vulgaris is a serious and infrequent disease in children. Its timely diagnosis and treatment allows modifying its prognosis. The objective is to describe its clinical characteristics, and the diagnostic and therapeutic approach of this uncommon autoimmune blistering disease in children. CLINICAL CASE: 2-year-old male patient, previously healthy. He initially presented generalized dermatosis with maximum lesion areas at the umbilical region, limbs and genitals; characterized by blisters, some denuded, and of easy bleeding, without mucosal involvement nor fever. Bullous impetigo was diagnosed and topical and systemic antibiotic treatment was started, showing no clinical improvement. He developed extension of the lesions with oral and anal mucosal involvement. The histologic and direct immunofluorescent study of lesions and perilesional skin confirm the diagnosis of pemphigus vulgaris. The patient started treatment with corticosteroids and immunomodulatory agents with good clinical response. CONCLUSIONS: Due to the similarity with other more prevalent infectious and inflammatory diseases, a high index of suspicion is required in order to avoid delays in the diagnosis and the start of treatment. In patients with blisters with an unexpected clinical evolu tion, it is necessary to conduct a joint evaluation with a dermatologist and to assess the opportunity of performing a biopsy of the lesion and perilesional skin for histological study and direct immunofluo rescence, which will allow diagnostic confirmation.
Authors: Gastão Tenório Lins; Nathalia Lages Sarmento Barbosa; Eulina Maria Vieira de Abreu; Klinger Vagner Teixeira da Costa; Kelly Chrystine Barbosa Meneses; Rodrigo Neves Silva; Sonia Maria Soares Ferreira Journal: Autops Case Rep Date: 2021-04-30