Tuberc Respir Dis.  2009 May;66(5):374-379. 10.4046/trd.2009.66.5.374.

A Case of Churg-Strauss Syndrome with Interstinal Perforation

• Park JH
• Jung YS
• Kim YK
• Lee YM
• Hwang JH
• Kim KU
• Kim DW
• Uh ST
• Kim JJ

• KMID: 1846401
• DOI: http://doi.org/10.4046/trd.2009.66.5.374

Abstract

Churg-Strauss syndrome (CSS) or allergic granulomatous angiitis is a rare syndrome that is characterized by hypereosinophilic systemic necrotizing vasculitis affecting small- to medium-sized arteries and veins. In general, it occurs in individuals with pre-existing allergic asthma. When CSS appears in patients, it has the following characteristics: eosinophilia of more than 10% in peripheral blood, paranasal sinusitis, pulmonary infiltrates, histological proof of vasculitis with extravascular eosinophils, and mononeuritis multiplex or polyneuropathy. Therapeutic trials dedicated to Churg-Strauss syndrome have been limited due to the rarity of this disorder and the difficulty in making a histological diagnosis. Proper treatment of patients with CSS is not widely known. In this case study, we report on our experience with an unusual patient case, characterized by purpura and a perforation of the small intestine after inadequate steroid therapy.

Keyword

Churg-Strauss syndrome; Asthma; Intestinal perforation; Eosinophilic infiltration

MeSH Terms

Arteries
Asthma
Churg-Strauss Syndrome
Eosinophilia
Eosinophils
Humans
Intestinal Perforation
Intestine, Small
Mononeuropathies
Polyneuropathies
Purpura
Sinusitis
Vasculitis
Veins

Figure

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  Figure 1 Chest radiography from 25 years old patient shows bilateral pleural effusion, patchy consolidation and increased interstitial marking of right lower lung field.

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  Figure 2 Computed tomography from the presented patient, there are multiple, variable-sized, nodules, or nodular airspace consolidation in both lungs (A). At lower area of lung shows mild bronchial dilatation, bronchial wall thickening and tiny centrilobular branching opacities (B).

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  Figure 3 Photography from purpura, which was stained by hematoxylin and eosin (H&E), shows epidermal hyperkeratosis and extravasated erythrocytes beneath of epidermis (arrow, A, ×40). Magnified pictures from same patient shows leukocytoclastic vasculitis with granulma formation at the perivenular area of dermal area (B, ×100), and marked neutrophils and eosinophils were infiltrated at dermal area (C, ×200).

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  Figure 4 Colonoscopic findings from the presented patient who had abdominal pain and diarrhea, shows multiple ulceration and mucosal edema. Findings of biopsy are eosinophilic infiltration and edema at the small and medium sized vessels.

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  Figure 5 Specimen from resected small intestine were combined with ulcerated area, perivascular infiltration of inflammatory cells and fibrin thrombus and fibronoid necrosis (A, H&E stain, ×40; B, H&E stain, ×100). Magnified finding of perivascular area showed marked infiltration of eosinophils and neutrophils which were compatible with eosinophilic leukocytoclastic vasculitis (C, H&E stain, ×200). Some other area showed granulation tissue with hemorrhage (not shown).

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