Corticoides tópicos como alternativa terapéutica en la dermatosis ampollosa por inmunoglobulina A lineal de la infancia. Caso clínico

Abstract

Presentación de casos clínicos RESUMENLa dermatosis por inmunoglobulina A lineal de la infancia es un trastorno autoinmunitario poco frecuente. Su etiología es desconocida, aunque se ha relacionado con fármacos, infecciones, enfermedades inmunológicas y procesos linfoproliferativos. Presentamos el caso de una niña de 6 años que consultaba por lesiones ampollosas periorales, sin otra sintomatología. Se pautó un tratamiento con mupirocina tópica primero y luego con metilprednisolona tópica, sin resolución del cuadro. Se realizó una biopsia cutánea, compatible con dermatosis por inmunoglobulina A lineal. No fue posible iniciar el tratamiento con dapsona por déficit parcial de glucosa-6-fosfato deshidrogenasa, por lo que se mantuvo el tratamiento tópico, con buena evolución de las lesiones. La dermatosis por inmunoglobulina A lineal es una enfermedad poco frecuente, cuyo diagnóstico diferencial incluye otras enfermedades ampollosas. La anatomía patológica es esencial para el diagnóstico. Si no es posible el tratamiento con dapsona, los corticoides tópicos pueden ser una alternativa, tanto en monoterapia como asociados a otros tratamientos. Palabras clave: dermatosis IgA lineal, corticoesteroides tópicos, dapsona, glucosa-6-fosfato deshidrogenasa. ABSTRACTLinear immunoglobulin A dermatosis of childhood is a rare autoimmune disorder. Its etiology remains unknown, although it has been linked to drugs, infections, immunological diseases and lymphoproliferative processes. We report the case of a 6 year old girl who consulted for perioral bullous lesions without other symptoms. Neither treatment with mupirocin nor methylprednisolone therapy achieved remission of cutaneous lesions. Skin biopsy showed a linear immunoglobulin A dermatosis. It was not possible to start treatment with dapsone because of a partial glucose-6-Corticoides tópicos como alternativa terapéutica en la dermatosis ampollosa por inmunoglobulina A lineal de la infancia. Caso clínico Topical corticosteroids as a therapeutic alternative in linear immunoglobulin A bullous dermatosis in childhood. Case report phosphate dehydrogenase deficiency, so topical treatment was maintained with good evolution of lesions. Linear immunoglobulin A dermatosis is a rare disease whose differential diagnosis includes other bullous diseases. Pathology is essential for diagnosis. When treatment with dapsone is not possible, topical corticosteroids may be an alternative, either alone or associated with other treatments.

Linear Immunoglobulin A Dermatosis: A Rare Case Illustrating Successful Treatment With Dapsone

“…Although usually well tolerated, it can be associated with serious adverse effects such as hemolysis, methemoglobinemia, agranulocytosis, hypersensitivity, and peripheral neuropathy. Therefore, before starting therapy, it is mandatory to exclude G6PD deficiency and perform an analytical study including a blood count with reticulocyte count and liver and kidney function tests [2,5,9]. Regular laboratory monitoring during treatment is also recommended.…”

“…Second-line agents include sulphonamides, which are usually better tolerated by patients. Colchicine can be used in patients with G6PD deficiency [9,17]. The duration of treatment for idiopathic LABD varies, usually remaining for a few weeks after complete lesion resolution.…”

“…Dapsone is the first-line treatment, with a rapid response [7,8]. Although it is usually well tolerated, it may be associated with severe adverse effects, requiring a comprehensive pretreatment investigative study and regular surveillance of key serum markers [2,5,9]. We describe the case of a four-year-old child diagnosed with LABD who underwent treatment with dapsone and systemic and topical corticosteroids.…”

Updates in the Diagnosis and Management of Linear IgA Disease: A Systematic Review

“…In a case of uncomplicated CBDC upon delivery with limited mucous membrane involvement, topical treatment with betamethasone valerate 0.05% at day 4 of life resulted in resolution at day 21 of life [21]. In a 6-year-old female with partial glucose-6-phosphate dehydrogenase deficiency, topical methylprednisolone 0.1% twice daily (vehicle not specified) and clobetasol shampoo resulted in resolution within a month [20]. In an adult case of vancomycin-induced LAD, vancomycin cessation and triamcinolone 0.1% ointment alone were enough to resolve bullae [3].…”

“…Monitoring for dapsone adverse reactions can be cumbersome as well. Numerous other treatments have been reported to be effective in the treatment of LAD, including topical corticosteroids, tetracyclines, dicloxacillin, oxacillin, erythromycin, sulfonamides, nicotinamide, rituximab, omalizumab, methotrexate, cyclosporine, etanercept, and intravenous immunoglobulin (IVIg) [1,[5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21]. This review provides updates on the diagnosis of LAD and emerging treatment modalities in order to assess their utility in the management of this disease.…”