Churg-Strauss syndrome pathology

Author: Adjunct A/Prof Patrick Emanuel, Dermatopathologist, Clínica Ricardo Palma, Lima, Peru. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley. October 2018.


Introduction

Churg-Strauss syndrome is also known as eosinophilic granulomatosis with polyangiitis and allergic granulomatosis. It is an autoimmune disease which begins as with allergic symptoms (asthma/hay fever-like symptoms) and evolves to a vasculitis with associated tissue damage. Skin involvement occurs in about half of patients and presents as purpura, petechiae, cutaneous nodules, and papules.

Histology of Churg-Strauss syndrome

In Churg-Strauss syndrome, the histopathology is varied depending on the stage of the disease and the type of lesion biopsied. Usually, the presentation in the skin occurs in the later stages of the disease and the prominent features include tissue eosinophilia (often with eosinophilic deposition 'flame figures' on collagen fibres) and extravascular granulomas (figures 1–3). Later lesions characteristically show vasculitis, which involves small to medium-sized vessels in the dermis and subcutis.

Churg-Strauss syndrome pathology

Special studies for Churg-Strauss syndrome

None are generally needed.

Differential diagnosis for Churg-Strauss syndrome

Other forms of vasculitis do not generally show extravascular granulomas and the prominent tissue eosinophilia seen in Churg-Strauss syndrome. Correlation with clinical and serological findings is usually essential to rule out other forms of vasculitis.

Other diagnoses to be considered include:

  • Nuchal fibroma — these typically don’t have the characteristic abnormal elastic fibres seen in elastofibroma
  • Desmoid fibromatosis — this is more cellular and infiltrates skeletal muscle; the characteristic abnormal elastic fibres seen in elastofibroma are absent.

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Related information

 

References

  • Davis MD, Daoud MS, McEvoy MT, Su WP. Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation. J Am Acad Dermatol. 1997 Aug;37(2 Pt 1):199-203. PubMed PMID: 9270504.

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