Clinical Spectrum of Henoch Schonlein Purpura in Adults: A Hospital Based Study

Abstract

Background and objectives: Henoch Schonlein purpura (HSP) is a small vessel vasculitic disorder common in children and has been extensively studied. Although it is known to also occur in adults there is relative paucity of data as regards to its clinical spectrum, complications and outcome, particularly in Indian context. Hence the study was undertaken with the objective to evaluate the various skin manifestations, systemic complications of HSP in adults and also compare it with data available in children in various published clinical studies.Study design, materials, methods : In this retrospective, observational, hospital-based cohort study conducted at Goa Medical College the premier teaching institute from Goa, clinical data of adult patients (>18 years age) fulfilling the diagnostic criteria as per European League Against Rheumatism (EULAR) 2010 criteria for HSP was obtained, over period of 6 years. All the clinical manifestations, complications, investigations, outcomes were recorded. Skin biopsy histopathology and immunofluorescent test findings were also obtained. The data was analysed and results were compared to the data available in pediatric studies to ascertain the similarities and differences.

Results: In our study cohort of 30 patients, we found a higher incidence of atypical and more extensive skin lesions particularly bullae (40%), necrotic ulcers (53.3%), urticarial wheals (53%) unlike in children as well as differences in distribution especially sparing of buttocks in adults. The incidence of gastrointestinal involvement was 80% which was higher than that reported by other studies in adults (35% to 70%). A significant 40% of patients had upper GI bleeding with endoscopy revealing small hemorrhages in gastric mucosa. Lower GIT bleed was seen in 8 patients. Renal involvement (microscopic hematuria, overt glomerulonephritis, nephrotic syndrome) was seen in 65% patients which was higher than that reported in children (43 %). Skin biopsy immunofluorescence was found to be positive in almost 66 % cases confirming IgA deposition which is the hallmark pathological finding.

Conclusions: HSP, though less common in adults than children, presents with atypical and more severe cutaneous manifestations like bullae, necrotic ulcers, urticarial wheals. Systemic involvement appears to be more frequent and causing more morbidity and mortality as compared to the data in children mentioned in standard literature and most of the patients required steroid therapy for treatment unlike in children where majority of these cases are self-limiting. Skin involvement does not necessarily mirror gastrointestinal involvement in terms of severity and temporal occurrence.