Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, 06800-Bilkent, Ankara, Turkey.
Eur J Pediatr. 2024 Nov 15;184(1):17. doi: 10.1007/s00431-024-05824-9.
The aim of this study was to evaluate the cutaneous manifestations of immunoglobulin A vasculitis (IgAV) in terms of skin lesion type, distribution and persistence and to investigate the relationship between cutaneous manifestations and system involvement and treatment needs. This retrospective observational study was conducted with 489 IgAV patients who were followed-up for at least 6 months between 2013 and 2024. Demographic characteristics, detailed cutaneous manifestations, clinical findings and treatments were retrieved from electronic medical records. IgAV patients were divided into subgroups according to the presence or absence of vesicles/bullae, necrosis/ulcer, rash spreading above the buttocks and persistence. The groups were analyzed statistically for demographic findings, systemic involvement and treatments. Of 489 patients, 36 (7.4%) had vesicles/bullae and 22 (4.5%) had necrosis/ulcers. 345 (70.6%) patients had cutaneous manifestations limited to the lower extremities and buttocks, 144 (29.4%) had cutaneous manifestations spreading from the lower extremities to the trunk, upper extremities or face. 36 (7.4%) patients had persistent rash for more than 1 month. Patients with necrosis/ulcer had more genital tract involvement (p = 0.04). Patients with rash spreading above the buttocks had more gastrointestinal tract and genital tract involvement (p = 0.014, p = 0.003). Patients with persistent rash had more renal involvement (p = 0.05). Patients with vesicles/bullae, necrosis/ulcer, rash spreading above the buttocks and persistent rash required more steroid treatment (p = 0.003, p = 0.001, p < 0.001, p = 0.03).
The characteristics of cutaneous manifestations in IgAV patients may be helpful in predicting the course of the disease. The skin lesion type, distribution and persistence of cutaneous manifestations in IgAV are associated with system involvement. In addition, the need for intensive therapy increases in the presence of vesicles/bullae, necrosis/ulcer, rash spreading over the buttocks and persistent rash in IgAV.
• Palpable purpura localized to the lower extremities is the expected cutaneous manifestation of immunoglobulin A vasculitis.
• The type, distribution and duration of rash in IgAV have effects on the immunoglobulin A vasculitis disease course. • Genital tract involvement is more common in patients with necrosis/ulcers. • Gastrointestinal tract involvement and genital tract involvement are more common in patients with rash spreading above the buttocks. • Renal involvement is more common in patients with persistent rash.
本研究旨在评估免疫球蛋白 A 血管炎（IgAV）的皮肤表现，包括皮肤损伤类型、分布和持续性，并探讨皮肤表现与系统受累和治疗需求之间的关系。
本回顾性观察性研究纳入了 2013 年至 2024 年间至少随访 6 个月的 489 例 IgAV 患者。从电子病历中检索人口统计学特征、详细的皮肤表现、临床发现和治疗情况。根据是否存在水疱/大疱、坏死/溃疡、皮疹蔓延至臀部以上和持续性，将 IgAV 患者分为亚组。对各组的人口统计学资料、系统受累和治疗情况进行统计学分析。
489 例患者中，36 例（7.4%）有水疱/大疱，22 例（4.5%）有坏死/溃疡。345 例（70.6%）患者的皮肤表现局限于下肢和臀部，144 例（29.4%）的皮肤表现从下肢蔓延至躯干、上肢或面部。36 例（7.4%）患者的皮疹持续 1 个月以上。有坏死/溃疡的患者生殖道受累更常见（p=0.04）。皮疹蔓延至臀部以上的患者胃肠道和生殖道受累更常见（p=0.014，p=0.003）。持续性皮疹患者肾脏受累更常见（p=0.05）。有水疱/大疱、坏死/溃疡、皮疹蔓延至臀部以上和持续性皮疹的患者需要更多的皮质类固醇治疗（p=0.003，p=0.001，p<0.001，p=0.03）。
IgAV 患者的皮肤表现特征有助于预测疾病进程。IgAV 的皮肤损伤类型、分布和持续性与系统受累有关。此外，在 IgAV 中存在水疱/大疱、坏死/溃疡、皮疹蔓延至臀部以上和持续性皮疹时，需要更强化的治疗。
