ABSTRACT
@#Linear IgA bullous dermatosis, also known as chronic bullous disease of childhood when present in the pediatric age group, is a rare blistering disease more predominantly seen in females less than five years old. This case describes a 2-year old girl who presented with scattered, tense vesicles and bullae on an erythematous base forming the classic “cluster of jewels” appearance. This clinical picture is often mistaken as bullous impetigo, commonly seen in children, delaying diagnosis and prompt treatment. Histopathologic examination showed subepidermal blistering with a predominantly neutrophilic inflammatory infiltrate. The direct immunofluorescence studies revealed a linear band of IgA deposition in the basement membrane zone consistent with the diagnosis of CBDC. The patient was started on colchicine and oral prednisone at 1 mg/kg/day and complete resolution was achieved within two weeks of therapy.
Subject(s)
ColchicineABSTRACT
BACKGROUND: Linear IgA bullous dermatosis (LABD) is an autoimmune, chronic bullous disease characterized by sub-epithelial bullae with linear IgA deposits along the basement membrane. LABD primarily affects young children and adults. There has been no study on LABD in Korea to date. OBJECTIVE: The purpose of this study was to evaluate the clinical features, laboratory examinations, treatments, and outcomes of Korean LABD patients. Patient characteristics including age at disease onset, gender, medical associations, medications, immunofluorescence findings, disease duration, treatment, and outcome were analyzed. METHODS: A retrospective analysis was conducted on 16 LABD patients diagnosed at Gangnam Severance Hospital between 1999 and 2014. RESULTS: A total of 16 LABD patients were included in the study, 5 children and 11 adults. The mean ages at disease onset in children and adults were 3.2 and 41 years, respectively. Eighty percent of children with LABD showed complete remission. In adults, partial remission was achieved in 36.4%, and complete remission in 54.5% of patients. Two patients were diagnosed with drug-induced LABD, and 2 with ulcerative colitis-associated LABD. CONCLUSION: Our report differed from previous reports in that all 5 children with LABD were male, and the incidence in adults was higher in females than males. Most patients responded well to dapsone and oral prednisolone. Since LABD is rare and can be misdiagnosed as impetigo or bullous pemphigoid, diagnosis by immunofluorescence microscopy is necessary for proper treatment to attain disease remission.
Subject(s)
Adult , Child , Female , Humans , Male , Basement Membrane , Dapsone , Diagnosis , Fluorescent Antibody Technique , Immunoglobulin A , Impetigo , Incidence , Korea , Linear IgA Bullous Dermatosis , Microscopy, Fluorescence , Pemphigoid, Bullous , Prednisolone , Retrospective Studies , UlcerABSTRACT
Chronic bullous disease of childhood (CBDC) is an autoimmune blistering disease that is characterized by Immunoglobulin A (IgA) deposits at the basement membrane zone. IgA autoantibodies (aAbs) from the serum of patients with CBDC react with antigens of 97 kDa (LABD97) and 120 kDa (LAD-1), and both of which are fragments of the extracellular domain of bullous pemphigoid 180 (BP180, type XVII collagen). The CBDC sera reacts with the immunodominant NC16a domain of BP180, which is the major region recognized by IgG aAbs in patients with bullous pemphigoid. A five-year-old boy presented with multiple pruritic tense blisters on the umbilical and inguinal areas for six weeks. The direct immunofluorescence of the perilesional area demonstrated linear deposits of IgA at the basement membrane zone. Using immunoblotting and an enzyme linked immunosorbent assay (ELISA), we identified the IgA aAbs reactive to antigens with a molecular weight of 120 kDa (LAD-1), which is a fragment of the extracellular domain of BP180.
Subject(s)
Humans , Autoantibodies , Basement Membrane , Blister , Enzyme-Linked Immunosorbent Assay , Fluorescent Antibody Technique, Direct , Immunoblotting , Immunoglobulin A , Immunoglobulin G , Linear IgA Bullous Dermatosis , Molecular Weight , Pemphigoid, Bullous , Transcutaneous Electric Nerve StimulationABSTRACT
La Dermatosis IgA lineal es una enfermedad vesiculoampollar subepidérmica autoinmune caracterizada por anticuerpos IgA en la unión dermoepidérmica. Es una enfermedad poco frecuente, siendo la mayoría de los casos idiopáticos, pero con reporte de casos por medicamentos, infecciones virales, enfermedades autoinmunes y tumores malignos. Se presentará un caso clínico de Dermatosis IgA lineal causada por Diclofenaco.
Linear IgA dermatosis is an autoimmune subepidermal vesiculobullous disease characterized by IgA antibodies at the dermo-epidermal junction. It is an uncommon disease, with most cases idiopathic, but with case reports caused by drug, viral infections, autoimmune diseases and malignant tumors. A clinical case of Linear IgA dermatosis caused by diclofenac is presented.