Linear IgA bullous dermatosis in a latin adolescent treated with cyclosporine and prednisone.

Autor: Ríos-Sánchez A; National School of Medicine and Homeopathy, National Polytechnic Institute., Godínez-Chaparro JA; Pediatric Dermatology Service, High Specialty Medical Unit of the Dr. Gaudencio González Garza General Hospital, La Raza National Medical Center, Mexican Social Security Institute., Quintal-Ramírez MJ; Department of Pathology, La Raza National Medical Center, Mexican Social Security Institute., Ramírez-Ricarte IR; Pediatric Dermatology Service, General Hospital of Mexico Dr. Eduardo Liceaga, Ministry of Health. Mexico City, Mexico.
Jazyk: angličtina
Zdroj: Boletin medico del Hospital Infantil de Mexico [Bol Med Hosp Infant Mex] 2024; Vol. 81 (5), pp. 305-310.
DOI: 10.24875/BMHIM.24000043
Abstrakt: Introduction: Linear IgA bullous dermatosis (LABD) is a rare autoimmune disease. Although dapsone is the initial treatment, other immunomodulators are used in resistant cases or when dapsone is unavailable.
Case Report: A 12-year-old Mexican child, with no relevant medical history, developed in May 2023 a disseminated dermatosis affecting all body segments, including mucous membranes, characterized by erythematous patches and plaques evolving into the formation of serous and serosanguinous blisters and vesicles, distributed in a "string of pearls" pattern. LABD was suspected and confirmed by skin biopsy, which showed a subepidermal blister with neutrophilic infiltration and linear Immunoglobulin A deposits at the dermo-epidermal junction by direct immunofluorescence. Treatment with prednisone (2 mg/kg/day) and cyclosporine (5 mg/kg/day) resulted in improvement and lesion remission within 2 weeks. Both drugs needed to be discontinued for 3 months due to intermittent blistering. Cyclosporine was continued as maintenance therapy at a dose of 4 mg/kg/day for 8 months.
Conclusions: The report highlights the use of cyclosporine as an alternative immunomodulator for DAAL, an immunosuppressive agent used in autoimmune disorders. Few cases, including this one, have described complete remission and control of the dermatosis with cyclosporine, accompanied by prednisone at the start of treatment.
(Copyright: © 2024 Permanyer.)
Databáze: MEDLINE