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. 2012 May;57(3):215-8.
doi: 10.4103/0019-5154.96198.

Leukocytoclastic vasculitis: a window to systemic churg strauss syndrome

Sudhir V Medhekar  1 , Resham J Vasani, Ratnakar R Kamath
Affiliations

Leukocytoclastic vasculitis: a window to systemic churg strauss syndrome

Sudhir V Medhekar et al. Indian J Dermatol. 2012 May.

Abstract

A twenty year old male presented with purpuric lesions with chronic painful ulcers over the lower extremities and a recurrent pruritic rash on the trunk for 10 years. He was diagnosed as idiopathic leukocytoclastic vasculitis (LCV) after investigations failed to reveal a systemic association. He was treated with immunosuppressants at each visit with partial remission. In 2004, he was diagnosed with bronchial asthma and allergic rhinitis. In his recent admission, he showed necrotic ulcers on legs and extensive shiny, truncal micropapules. Examination revealed maxillary sinus tenderness and loss of sensation on the medial aspect of the left lower limb. Biopsy of ulcer and the micropapules showed the presence of extravascular eosinophils, while hematological investigations showed peripheral eosinophilia of 18%, raised serum Immunoglobulin E (IgE), Anti nuclear antibody (ANA) positivity and negative antineutrophil cytoplasmic antibody (ANCA). Radiography confirmed maxillary sinusitis, nerve conduction studies revealed mononeuritis of the anterior tibial nerve and pulmonary function tests (PFT) were normal. Clinical examination and investigations pointed towards the diagnosis of Churg-Strauss syndrome (CSS). This report highlights the development of full-blown CSS over a period of 12 years in a patient initially diagnosed as idiopathic LCV, emphasizing the need for regular follow-up of resistant and recurrent cases of LCV.

Keywords: Allergic granulomatosis; Churg-Strauss syndrome; asthma; eosinophilia; leukocytoclastic vasculitis.

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Conflict of interest statement

Conflict of Interest: Nil.

Figures

Figure 1
Figure 1
Stellate-shaped ulcerations with overlying hemorrhagic crusts healing with atrophic scars seen on the dorsa of feet and legs bilaterally
Figure 2
Figure 2
Micropapular rash involving the back
Figure 3
Figure 3
4× magnification-ulcerated epidermis with scale crust and perivascular mixed cell infiltrate involving the upper and mid dermis (H and E stain)
Figure 4
Figure 4
40× magnification showing thickening of the vessel wall with infiltration of lymphocytes, histiocytes and plasma cells in and around the vessel walls with the presence of extravascular eosinophils and extravasated RBCs (H and E stain)
Figure 5
Figure 5
40× magnification showing thickened vessel wall with infiltrate inside the walls with fibrin deposition (H and E stain)
Figure 6
Figure 6
Healing of the ulcerations on the dorsum of foot post treatment
See this image and copyright information in PMC

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