BACKGROUND: Wells' syndrome is characterized by clinical features of cellulitis and a histological picture of eosinophilic infiltrate of the dermis with some "flame" figures. PATIENTS AND METHODS: The clinical and histological features of nine patients with Wells' syndrome seen from 1988 to 1998 were retrospectively reviewed. RESULTS: The clinical features of the nine patients (five men and four women) were urticaria (n=1), cellulitis (n=2), annular plaques (n=3), vesiculo-bullous lesions (n=2) and edema of the face with nodules of the conjunctiva (n=1). Histological examination of skin biopsies showed an eosinophilic infiltrate of the dermis associated with some "flame" figures in all cases. The infiltrate was located in the superficial or deep dermis in accordance with the different clinical features. One patient developed a non Hodgkin lymphoma and presented successively: a Wells' syndrome, a leucocytoklastic vasculitis and a Sweet's syndrome. Numerous treatment were used: topical corticosteroids, H1-antihistamines, dapsone and systemic corticosteroids. Two patients relapsed after treatment withdrawal. DISCUSSION: This study demonstrated a wide polymorphism of the clinical and histological features of Wells' syndrome. The clinical features seem to depend on the location of the dermal infiltrate, suggesting the existence of a spectrum of eosinophilic dermatoses, like in neutrophilic dermatoses. The successive occurrence of vasculitis, Wells' syndrome and Sweet'syndrome in a patient suggests an overlap between these diseases. Systemic corticosteroids are the most effective treatment, but may lead to a corticosteroid dependence.
BACKGROUND:Wells' syndrome is characterized by clinical features of cellulitis and a histological picture of eosinophilic infiltrate of the dermis with some "flame" figures. PATIENTS AND METHODS: The clinical and histological features of nine patients with Wells' syndrome seen from 1988 to 1998 were retrospectively reviewed. RESULTS: The clinical features of the nine patients (five men and four women) were urticaria (n=1), cellulitis (n=2), annular plaques (n=3), vesiculo-bullous lesions (n=2) and edema of the face with nodules of the conjunctiva (n=1). Histological examination of skin biopsies showed an eosinophilic infiltrate of the dermis associated with some "flame" figures in all cases. The infiltrate was located in the superficial or deep dermis in accordance with the different clinical features. One patient developed a non Hodgkin lymphoma and presented successively: a Wells' syndrome, a leucocytoklastic vasculitis and a Sweet's syndrome. Numerous treatment were used: topical corticosteroids, H1-antihistamines, dapsone and systemic corticosteroids. Two patients relapsed after treatment withdrawal. DISCUSSION: This study demonstrated a wide polymorphism of the clinical and histological features of Wells' syndrome. The clinical features seem to depend on the location of the dermal infiltrate, suggesting the existence of a spectrum of eosinophilic dermatoses, like in neutrophilic dermatoses. The successive occurrence of vasculitis, Wells' syndrome and Sweet'syndrome in a patient suggests an overlap between these diseases. Systemic corticosteroids are the most effective treatment, but may lead to a corticosteroid dependence.