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Pathology of  Allergic Granulomatosis 

(Churg-Strauss Syndrome)

Dr Sampurna Roy MD      

 

Dermatopathology Quiz Case 104

Diagnosis: Allergic Granulomatosis (Churg-Strauss Syndrome)

 

                                                                                                                      

 

 

Allergic granulomatosis -(Churg-Strauss syndrome) is a small-vessel vasculitis characterized by severe asthma, lung/tissues infiltrates, extravascular necrotizing granulomas, and eosinophilia.

Cutaneous involvement is common but may not be highly suggestive.

Site: Usually located on the extremities and on the scalp.

Cutaneous presentation: The lesions present as  pupura, erythematous or urticarial lesions and nodules.

Ulceration may occur in some cases secondary to arteritis and thrombosis of vessels.

Microscopic features:

3 major microscopic features are : 

       

(A) Necrotizing vasculitis. 

(B) Tissue infiltration by eosinophils.  

(C) Extravascular granulomas.

Vasculitis may be present in the small arteries and veins.

In rare cases larger arteries may be involved. (Differential diagnosis: Polyarteritis nodosa ).

Older lesions show scarring. 

The tissue is usually infiltrated by the eosinophils.  Destruction of the eosinophils lead to release of granules and eosinophilia of the collagen.

In some cases extravascular granulomas are present.

These granulomas may show fibrinoid or partly basophilic central necrosis.

Eosinophils, neutrophils and debris may be present scattered within the granuloma.

A high index of suspicion on skin lesions and the proper lesion selection for histological examination may be very important for early diagnosis of Churg-Strauss syndrome.

Clinical-pathological correlation is essential, as both clinical and histological features are not pathognomonic.

 

Further reading:

Churg-Strauss syndrome.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome) with microscopic eosinophilic thromboembolism and cardiac involvement: report of two cases.

Churg-Strauss syndrome: a case report.

Churg-Strauss syndrome presenting as scar reactivation: histopathologic features and an illustration of 'locus minoris resistentiae'.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort.

Churg-Strauss syndrome presenting with cutaneous vasculitis.

Churg-Strauss syndrome].

Genital papules revealing a Churg-Strauss syndrome.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

 

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