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    Art:10.1007/s12098 013 1013 Z

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    Iwan Miswar
    This scientific letter reports the case of a 9-year-old girl who presented with Henoch-Schönlein purpura (HSP), an immune complex-mediated vasculitis commonly seen in children. While skin lesions in HSP are typically purpuric, this patient developed large hemorrhagic bullous lesions on her lower limbs and trunk. A skin biopsy confirmed leukocytoclastic vasculitis. She was treated with steroids and the bullous lesions subsided. However, she later developed nephritis with proteinuria and hematuria, indicating kidney involvement. Her renal biopsy showed IgA deposition consistent with HSP nephritis. Though rare, this case demonstrates that HSP can occasionally

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    Art:10.1007/s12098 013 1013 Z

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    Iwan Miswar
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    This scientific letter reports the case of a 9-year-old girl who presented with Henoch-Schönlein purpura (HSP), an immune complex-mediated vasculitis commonly seen in children. While skin lesions in HSP are typically purpuric, this patient developed large hemorrhagic bullous lesions on her lower limbs and trunk. A skin biopsy confirmed leukocytoclastic vasculitis. She was treated with steroids and the bullous lesions subsided. However, she later developed nephritis with proteinuria and hematuria, indicating kidney involvement. Her renal biopsy showed IgA deposition consistent with HSP nephritis. Though rare, this case demonstrates that HSP can occasionally

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    This scientific letter reports the case of a 9-year-old girl who presented with Henoch-Schönlein purpura (HSP), an immune complex-mediated vasculitis commonly seen in children. While skin lesions in HSP are typically purpuric, this patient developed large hemorrhagic bullous lesions on her lower limbs and trunk. A skin biopsy confirmed leukocytoclastic vasculitis. She was treated with steroids and the bullous lesions subsided. However, she later developed nephritis with proteinuria and hematuria, indicating kidney involvement. Her renal biopsy showed IgA deposition consistent with HSP nephritis. Though rare, this case demonstrates that HSP can occasionally

    Copyright:

    © All Rights Reserved
    Download as PDF, TXT or read online from Scribd
    Download as pdf or txt
    9 views2 pages

    Art:10.1007/s12098 013 1013 Z

    Uploaded by

    Iwan Miswar
    This scientific letter reports the case of a 9-year-old girl who presented with Henoch-Schönlein purpura (HSP), an immune complex-mediated vasculitis commonly seen in children. While skin lesions in HSP are typically purpuric, this patient developed large hemorrhagic bullous lesions on her lower limbs and trunk. A skin biopsy confirmed leukocytoclastic vasculitis. She was treated with steroids and the bullous lesions subsided. However, she later developed nephritis with proteinuria and hematuria, indicating kidney involvement. Her renal biopsy showed IgA deposition consistent with HSP nephritis. Though rare, this case demonstrates that HSP can occasionally

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    SCIENTIFIC LETTER TO THE EDITOR

    Hemorrhagic Bullous Lesions in a Girl with Henoch


    Schnlein 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 / Published online: 12 April 2013
    #Dr. K C Chaudhuri Foundation 2013
    To the Editor: Henoch Schnlein purpura (HSP) is com-
    mon 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 examina-
    tion, she had palpable purpura and hemorrhagic bullae rang-
    ing 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 vasculi-
    tis with IgA and C
    3
    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
    2
    /h) and microscopic hematuria. Re-
    nal function tests were normal. She received intravenous
    pulse methylprednisolone (30 mg/kg/d for 3 d). Renal biop-
    sy at this time was suggestive of focal segmental glomerular
    sclerosis with deposition of IgA (++++) and C
    3
    +in the
    mesangium. Electron microscopy showed subendothelial
    and paramesengial electron dense immune complex de-
    posits. She continued to have heavy proteinuria despite
    prednisolone therapy which necessitated addition of azathi-
    oprine. 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 hemor-
    rhagic 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 [35]. Polymor-
    phism 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 al-
    though 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
    S. Dogra
    Department of Dermatology, Postgraduate Institute of Medical
    Education and Research, Chandigarh, India
    B. Saikia
    :
    R. W. Minz
    Department of Immunopathology, Postgraduate Institute of
    Medical Education and Research, Chandigarh, India
    R. Nada
    Department of Histopathology, Postgraduate Institute of Medical
    Education and Research, Chandigarh, India
    Indian J Pediatr (February 2014) 81(2):210211
    DOI 10.1007/s12098-013-1013-z
    In conclusion, hemorrhagic bullous presentation of HSP
    is uncommon but well recognised diagnostic challenge for
    the physician.
    References
    1. Ozen S, Pistorio A, Iusan SM, et al. Paediatric Rheumatology Inter-
    national Trials Organisation (PRINTO). EULAR/PRINTO/PRES
    criteria for Henoch-Schnlein purpura, childhood polyarteritis nodosa,
    childhood Wegener granulomatosis and childhood Takayasu arteritis:
    Ankara 2008. Part II: final classification criteria. Ann Rheum Dis.
    2010;69:798806.
    2. Wananukul S, Pongprasit P, Korkji W. Henoch Schonlein
    purpura presenting as haemorrhagic vesicles and bullae: case
    report and literature review. Pediatr Dermatol. 1995;12:314
    7.
    3. Saulsbury FT. Haemorrhagic bullous lesions in HenochSchnlein
    purpura. Pediatr Dermatol. 1998;15:3579.
    4. Leung AK, Robson WL. Haemorrhagic bullous lesions in a child
    with Henoch Schonlein purpura. Pediatr Dermatol. 2006;23:139
    41.
    5. Kausar S, Yalamanchilli A. Management of hemorrhagic bullous
    lesion in Henoch-Schonlein Purpure; Is there a consensus? J
    Dermatol Treat. 2009;20:8890.
    Fig. 1 Hemorrhagic bullous lesions over lower limbs along with few
    palpable purpuric lesions
    Indian J Pediatr (February 2014) 81(2):210211 211

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