SCIENTIFIC LETTER TO THE EDITOR

Hemorrhagic Bullous Lesions in a Girl with HenochSchönlein Purpura

Sonia Mehra & Deepti Suri & Sunil Dogra & Anju Gupta &

Amit Rawat & Biman Saikia & Ranjana W. Minz &

Ritambhara Nada & Surjit Singh

Received: 6 February 2013 /Accepted: 13 March 2013# Dr. K C Chaudhuri Foundation 2013

To the Editor: Henoch Schönlein purpura (HSP) is com-mon childhood systemic vasculitic syndrome, mediatedby IgA1 immune complex deposition. Skin lesions areclassically purpuric but erythematous maculopapules,petechiae, urticarial wheals, and hemorrhagic edemaare also described. We report a girl with HSP whopresented with hemorrhagic bullous lesions and laterdeveloped glomerulonephritis.

A 9-y-old girl presented to us with abdominal pain,petechiae and palpable purpura mainly on lower limbs. Overthe next 2 d she developed vesiculobullous lesions. Some ofthese turned hemorrhagic. The lesions spread to the trunkand a few lesions also appeared on ear lobes. On examina-tion, she had palpable purpura and hemorrhagic bullae rang-ing from 3 mm to 5 cm in size (Fig. 1). Lesions weredistributed mainly on lower limbs and buttocks, with fewlesions on trunk and ear lobe. Based on clinical presentation,

a provisional diagnosis of HSP was made. Skin biopsy frombullous lesion was consistent with leukocytoclastic vasculi-tis with IgA and C3 deposits. She was given intravenousdexamethasone (3 mg/kg) in view of abdominal symptomsand continued on oral prednisolone. The bullous lesionsgradually subsided. However, 2 wk later she developedproteinuria (141 mg/m2/h) and microscopic hematuria. Re-nal function tests were normal. She received intravenouspulse methylprednisolone (30 mg/kg/d for 3 d). Renal biop-sy at this time was suggestive of focal segmental glomerularsclerosis with deposition of IgA (++++) and C3+in themesangium. Electron microscopy showed subendothelialand paramesengial electron dense immune complex de-posits. She continued to have heavy proteinuria despiteprednisolone therapy which necessitated addition of azathi-oprine. Prednisolone was tapered and stopped slowly. At 5 yof follow up, there has been no recurrence of skin lesionsand urinary abnormalities.

Diagnosis of HSP is usually clinical but recentlyclassification criteria has been proposed [1]. Rash inHSP can be polymorphic but vesiculobullous or hemor-rhagic bullous presentation of HSP in children is rare.Hemorrhagic bullae in children with HSP was firstdescribed by Wananukul et al. [2] and later variousother authors have reported the same [3–5]. Polymor-phism of skin lesions, variable time of presentation andatypical rashes can be a dermatologic challenge for thepediatrician facing children with HSP and mandates askin biopsy like in our case [3]. There is no consensuson the treatment for isolated skin manifestations al-though some authors have recommended use of steroidsfor the severe skin lesions [5]. Most important of all noprognostic 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 MedicalEducation and Research, Chandigarh 160012, Indiae-mail: surjitsinghpgi@hotmail.com

S. DograDepartment of Dermatology, Postgraduate Institute of MedicalEducation and Research, Chandigarh, India

B. Saikia :R. W. MinzDepartment of Immunopathology, Postgraduate Institute ofMedical Education and Research, Chandigarh, India

R. NadaDepartment of Histopathology, Postgraduate Institute of MedicalEducation and Research, Chandigarh, India

Indian J PediatrDOI 10.1007/s12098-013-1013-z

In conclusion, hemorrhagic bullous presentation of HSPis uncommon but well recognised diagnostic challenge forthe physician.

References

1. Ozen S, Pistorio A, Iusan SM, et al. Paediatric Rheumatology Inter-national Trials Organisation (PRINTO). EULAR/PRINTO/PREScriteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa,childhood Wegener granulomatosis and childhood Takayasu arteritis:Ankara 2008. Part II: final classification criteria. Ann Rheum Dis.2010;69:798–806.

2. Wananukul S, Pongprasit P, Korkji W. Henoch Schonleinpurpura presenting as haemorrhagic vesicles and bullae: casereport and literature review. Pediatr Dermatol. 1995;12:314–7.

3. Saulsbury FT. Haemorrhagic bullous lesions in Henoch–Schönleinpurpura. Pediatr Dermatol. 1998;15:357–9.

4. Leung AK, Robson WL. Haemorrhagic bullous lesions in a childwith Henoch Schonlein purpura. Pediatr Dermatol. 2006;23:139–41.

5. Kausar S, Yalamanchilli A. Management of hemorrhagic bullouslesion in Henoch-Schonlein Purpure; Is there a consensus? JDermatol Treat. 2009;20:88–90.

Fig. 1 Hemorrhagic bullous lesions over lower limbs along with fewpalpable purpuric lesions

Indian J Pediatr