Wells syndrome associated with Churg–Strauss syndrome

Abstract

Churg–Strauss syndrome (CSS) is a systemic vasculitis occurring in patients with a history of asthma. Wells' syndrome (WS) is a rare inflammatory dermatosis that clinically resembles cellulitis, and is histologically characterized by eosinophilic infiltration and flame figures. We report a case of WS associated with CSS. There have been three previous reports of WS associated with CSS; ours is the fourth. All cases had bullous lesions, and three cases were positive for antineutrophil cytoplasmic antibodies.

Idiopathic bullous eosinophilic cellulitis (Wells syndrome) responsive to topical tacrolimus and antihistamine combination

“…Clinical characteristics include short stature, senile appearance, cataract, early menopause, premature arteriosclerosis, skin changes (scleroderma-like features, premature canities, baldness, and skin ulceration), associated with an increased risk of malignancy. [1] We present a case of Werner's syndrome in view of its clinical rarity.…”

“…[1,4] Histopathological findings follow an evolutionary course through acute, subacute, and resolving stages. [2] Dermal edema and an eosinophilic infiltrate in the upper and deep dermis characterize the acute stage.…”

“…Wells' syndrome, or eosinophilic cellulitis, is a recurrent, hypersensitivity reaction to an arthropod bite, drug, infections, Churg-Strauss syndrome, or an overlap with hypereosinophilic syndrome. [1] Aberrant and inadequate eosinophil skin homing may be one of the key mechanisms of the pathogenesis of Wells syndrome. Pruritic, erythematous-edematous plaques that evolve rapidly over 2-3 days are its salient clinical features.…”

“…If an infection or other treatable precipitating factors can be identified, then there is often improvement with treatment of the underlying condition. [1] A 12-years-old girl with Wells syndrome H and E, ×100) had a complete recovery with cetirizine. [5] The present case too, responded well to cetirizine.…”

Systemic lupus erythematosus associated with Wells’ syndrome

“…Concurrence of WS and malignant diseases as colon cancer, trachea squamous carcinoma, nasopharyngeal carcinoma or angioimmunoblastic lymphadenopathy has been reported [7,8]. We reviewed all case reports of autoimmne diseases with WS, Wnding only four cases of Churg-Strauss syndrome (CSS) and one case of ulcerative colitis associated with WS [9,10]. However, the coexistence of SLE and WS was never mentioned in literature.…”