National School of Medicine and Homeopathy, National Polytechnic Institute.
Pediatric Dermatology Service, High Specialty Medical Unit of the Dr. Gaudencio González Garza General Hospital, La Raza National Medical Center, Mexican Social Security Institute.
Bol Med Hosp Infant Mex. 2024;81(5):305-310. doi: 10.24875/BMHIM.24000043.
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.
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.
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.
线性 IgA 大疱性皮病（LABD）是一种罕见的自身免疫性疾病。尽管氨苯砜是初始治疗药物，但在耐药病例或无法使用氨苯砜时，会使用其他免疫调节剂。
一名 12 岁的墨西哥儿童，无相关病史，于 2023 年 5 月出现播散性皮肤病，影响全身各部位，包括黏膜，表现为红斑斑块，演变为浆液性和血清血性大疱和水疱，呈“串珠”样分布。皮肤活检怀疑并确诊为 LABD，直接免疫荧光显示表皮下水疱伴中性粒细胞浸润和真皮表皮交界处线性 IgA 沉积。泼尼松（2mg/kg/天）和环孢素（5mg/kg/天）治疗 2 周后病情改善，皮损消退。由于间歇性水疱，两种药物均需停药 3 个月。环孢素继续作为维持治疗，剂量为 4mg/kg/天，持续 8 个月。
本报告强调了环孢素作为 DAAL 的替代免疫调节剂的应用，环孢素是一种用于自身免疫性疾病的免疫抑制剂。包括本例在内的少数病例描述了完全缓解和控制皮肤病，同时在治疗开始时使用泼尼松。
