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Purpura, petechiae, and bullae as first signs of juvenile granulomatosis with polyangiitis

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Abstract

We present a case of a 14-year-old girl who had a severe form of granulomatosis with polyangiitis (GPA) with extensive dermatological involvement, whose initial presentation was nonspecific leading to diagnostic confusion and initial consideration of infectious and other vasculitis causes. The patient presented with fever, congestion, malaise, and sinus pain. She was diagnosed with bacterial sinusitis and treated with antibiotics. Within weeks, she developed abdominal pain, hematuria, migratory arthritis, and palpable purpura and was diagnosed with Henoch-Schonlein purpura. She went on to develop hemoptysis and progression of the rash into erosive bullae. Investigations revealed that she was ANCA positive and had pauci-immune glomerulonephritis. Given her upper airway, pulmonary and renal involvement, and antineutrophil cytoplasmic antibodies positivity, a definitive diagnosis of a severe form of GPA was made. GPA is a chronic relapsing, life threatening vasculitis that predominantly affects small vessels. Conclusion: Our case demonstrates that GPA can present initially with nonspecific symptoms, including extensive dermatological involvement, leading to diagnostic confusion, and delays in treatment. In the case of a severe peripheral rash in the juvenile population and/or resistant upper airway symptoms, it is vital to consider a diagnosis of GPA to avoid serious organ or life threatening consequences.

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Abbreviations

ANCA:

Antineutrophil cytoplasmic antibody

EULAR:

European League against Rheumatism

GPA:

Granulomatosis with polyangiitis

HSP:

Henoch-Schonlein purpura

PRINTO:

Pediatric Rheumatology International Trials Organisation

PRES:

Pediatric Rheumatology European Society

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Acknowledgments

The authors wish to thank the patient and her family for allowing this paper to be published.

Financial Disclosure

The authors have no financial relationships relevant to this article to disclose.

Conflict of Interest

The authors have no conflicts of interest to disclose.

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Authors and Affiliations

  1. Northern Ontario School of Medicine, Sudbury, Canada

    Saara Rawn

  2. University of Calgary, Calgary, Canada

    Saara Rawn, Paivi Miettunen, Holly A. Brown & Heinrike Schmeling

  3. Alberta Children’s Hospital Research Institute, Calgary, Canada

    Paivi Miettunen & Heinrike Schmeling

  4. Calgary Laboratory Services, Calgary, Canada

    Holly A. Brown

  5. Section of Rheumatology, Alberta Children’s Hospital, University of Calgary, 2888 Shaganappi Trail NW, Calgary, AB, T3B6A8, Canada

    Heinrike Schmeling

Authors
  1. Saara Rawn
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  2. Paivi Miettunen
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  4. Heinrike Schmeling
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Corresponding author

Correspondence to Heinrike Schmeling.

Additional information

Communicated by Beat Steinmann

What is Known—What is New (Authors Summary)

We present here a severe case of pediatric granulomatosis with polyangiitis with extensive dermatological involvement. The initial presentation was nonspecific leading to diagnostic confusion and initial consideration of infectious and Henoch-Schonlein purpura. Therefore, diagnosis and treatment were delayed. With this case report, we would like to alert that if a child or adolescent presents with multiple nonspecific symptoms and has extensive skin involvement it is vital to consider a diagnosis of granulomatosis with polyangiitis early in order to initiate appropriate treatment and avoid serious organ or life threatening consequences.

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Rawn, S., Miettunen, P., Brown, H.A. et al. Purpura, petechiae, and bullae as first signs of juvenile granulomatosis with polyangiitis. Eur J Pediatr 173, 1685–1689 (2014). https://doi.org/10.1007/s00431-014-2298-2

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  • DOI: https://doi.org/10.1007/s00431-014-2298-2

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