Characteristics of cutaneous manifestations in immunoglobulin a vasculitis and their relationships with system involvement and treatment needs

Abstract

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).

Conclusions: 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.

What is known: • Palpable purpura localized to the lower extremities is the expected cutaneous manifestation of immunoglobulin A vasculitis.

What is new: • 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.

Keywords: Gastrointestinal tract involvement; Henoch schonlein purpura; Immunoglobulin a vasculitis; Lesion type; Skin.