Wells' Syndrome Associated with Churg-Strauss Syndrome

Churg-Strauss syndrome (CSS) is a multisystem granulomatous vasculitis that is characterized by peripheral eosinophilia and the infiltration of eosinophils into systemic organs. The skin lesions of CSS consist mainly of palpable purpura and nodules. Wells' syndrome (WS) is a rare inflammatory dermatosis that is associated with recurrent granulomatous dermatitis and eosinophilia. Since these two diseases are rare, any overlap between them is very unusual. Herein, we report a patient with CSS, who initially presented a skin eruption of erythematous urticarial-plaques, vesicles, and blisters. Upon biopsy, the histology of these plaques indicated eosinophilic infiltration and "flame figures" within the dermis, which was consistent with a diagnosis of WS. Although the association between WS and CSS that was observed in our patient may be purely coincidental, it could also suggest a common pathogenetic background of these two distinct diseases, as both share several many common features.

A Case of Wells' Syndrome Treated with Systemic PUVA / 대한피부과학회지

Wells' syndrome, or eosinophilic cellulitis, is characterized by recurrent cutaneous swellings which resemble acute bacterial cellulitis, and by distinctive histopathological changes. Skin lesions show dermal eosinophilic infiltration and the characteristic 'flame figures', but not pathognomonic, which are composed of eosinophil major protein deposited on collagen bundles. A 51-year-old woman developed a chronic, pruritic, erythematous to dark-brown colored, annular, infiltrated plaques with papules, vesicles and some crusts on the chest, abdomen, back and both forearms with the clinical and histological features of Wells' syndrome. Skin lesions had recurred frequently with systemic corticosteroids therapy, so we tried systemic PUVA and treated her successfully without recurrence until now.

A Case of Wells' Syndrome Treated with Systemic PUVA / 대한피부과학회지

Wells' syndrome, or eosinophilic cellulitis, is characterized by recurrent cutaneous swellings which resemble acute bacterial cellulitis, and by distinctive histopathological changes. Skin lesions show dermal eosinophilic infiltration and the characteristic 'flame figures', but not pathognomonic, which are composed of eosinophil major protein deposited on collagen bundles. A 51-year-old woman developed a chronic, pruritic, erythematous to dark-brown colored, annular, infiltrated plaques with papules, vesicles and some crusts on the chest, abdomen, back and both forearms with the clinical and histological features of Wells' syndrome. Skin lesions had recurred frequently with systemic corticosteroids therapy, so we tried systemic PUVA and treated her successfully without recurrence until now.

Eosinophilic Cellulitis (Wells' Syndrome)

Eosinophilic cellulitis is a rare dermatosis first described by Wells, and characterized by recurrent episodes of sudden outbreaks of erythematous cutaneous swellings often painful or pruritic. Microscopically, the lesion shows diffuse tissue eosinophilia and fibrinoid flame figures, evolution of associated focal necrobiosis, and formation of focal microgranulomas associated with eosinophils. The cause is still unknown but the triggers which have been thought to precipitate the disease include insect bites, parasitic infections such as toxocara, onchocerciasis, nasopharyngeal carcinoma, rheumatoid arthritis and spider bites. However many cases are idiopathic. The author experienced a case of eosinophilic cellulitis of a 52-year-old woman with multiple cutaneous tender plaques of cellulitis for approximately 10 years with history of repeated remission and recurrent episodes. Biopsy was taken from ulcerated edematous nodule of inguinal region under the clinical impression of deep fungal infection, pyoderma gangrenosum and polyarteritis nodosa. Cultures for fungal and common organisms were negative. Histologically, the entire dermis was infiltrated by numerous eosinophils and scattered histiocytes. There were scattered flame figures showing necrobiotic foci in the collagen with accumulation of eosinophils, granulated free cosinophilic granules and histiocytes.

Eosinophilic Cellulitis (Wells' Syndrome)

Eosinophilic cellulitis is a rare dermatosis first described by Wells, and characterized by recurrent episodes of sudden outbreaks of erythematous cutaneous swellings often painful or pruritic. Microscopically, the lesion shows diffuse tissue eosinophilia and fibrinoid flame figures, evolution of associated focal necrobiosis, and formation of focal microgranulomas associated with eosinophils. The cause is still unknown but the triggers which have been thought to precipitate the disease include insect bites, parasitic infections such as toxocara, onchocerciasis, nasopharyngeal carcinoma, rheumatoid arthritis and spider bites. However many cases are idiopathic. The author experienced a case of eosinophilic cellulitis of a 52-year-old woman with multiple cutaneous tender plaques of cellulitis for approximately 10 years with history of repeated remission and recurrent episodes. Biopsy was taken from ulcerated edematous nodule of inguinal region under the clinical impression of deep fungal infection, pyoderma gangrenosum and polyarteritis nodosa. Cultures for fungal and common organisms were negative. Histologically, the entire dermis was infiltrated by numerous eosinophils and scattered histiocytes. There were scattered flame figures showing necrobiotic foci in the collagen with accumulation of eosinophils, granulated free cosinophilic granules and histiocytes.