Hemorrhagic Bullous Lesions in a Girl with Henoch Schönlein Purpura

Abstract

To the Editor: Henoch Schonlein purpura (HSP) is common childhood systemic vasculitic syndrome, mediated by IgA1 immune complex deposition. Skin lesions are classically purpuric but erythematous maculopapules, petechiae, urticarial wheals, and hemorrhagic edema are also described. We report a girl with HSP who presented with hemorrhagic bullous lesions and later developed glomerulonephritis. A 9-y-old girl presented to us with abdominal pain, petechiae and palpable purpura mainly on lower limbs. Over the next 2 d she developed vesiculobullous lesions. Some of these turned hemorrhagic. The lesions spread to the trunk and a few lesions also appeared on ear lobes. On examination, she had palpable purpura and hemorrhagic bullae ranging from 3 mm to 5 cm in size (Fig. 1). Lesions were distributed mainly on lower limbs and buttocks, with few lesions on trunk and ear lobe. Based on clinical presentation, a provisional diagnosis of HSP was made. Skin biopsy from bullous lesion was consistent with leukocytoclastic vasculitis with IgA and C3 deposits. She was given intravenous dexamethasone (3 mg/kg) in view of abdominal symptoms and continued on oral prednisolone. The bullous lesions gradually subsided. However, 2 wk later she developed proteinuria (141 mg/m/h) and microscopic hematuria. Renal function tests were normal. She received intravenous pulse methylprednisolone (30 mg/kg/d for 3 d). Renal biopsy at this time was suggestive of focal segmental glomerular sclerosis with deposition of IgA (++++) and C3+in the mesangium. Electron microscopy showed subendothelial and paramesengial electron dense immune complex deposits. She continued to have heavy proteinuria despite prednisolone therapy which necessitated addition of azathioprine. Prednisolone was tapered and stopped slowly. At 5 y of follow up, there has been no recurrence of skin lesions and urinary abnormalities. Diagnosis of HSP is usually clinical but recently classification criteria has been proposed [1]. Rash in HSP can be polymorphic but vesiculobullous or hemorrhagic bullous presentation of HSP in children is rare. Hemorrhagic bullae in children with HSP was first described by Wananukul et al. [2] and later various other authors have reported the same [3–5]. Polymorphism of skin lesions, variable time of presentation and atypical rashes can be a dermatologic challenge for the pediatrician facing children with HSP and mandates a skin biopsy like in our case [3]. There is no consensus on the treatment for isolated skin manifestations although some authors have recommended use of steroids for the severe skin lesions [5]. Most important of all no prognostic significance has been attached to these lesions [5]. S. Mehra :D. Suri :A. Gupta :A. Rawat : S. Singh (*) Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India e-mail: surjitsinghpgi@hotmail.com