WELLS-SYNDROME ASSOCIATED WITH IDIOPATHIC HYPEREOSINOPHILIC SYNDROME

Citation
T. Bogenrieder et al., WELLS-SYNDROME ASSOCIATED WITH IDIOPATHIC HYPEREOSINOPHILIC SYNDROME, British journal of dermatology, 137(6), 1997, pp. 978-982
Citations number
29
Categorie Soggetti
Dermatology & Venereal Diseases
ISSN journal
00070963
Volume
137
Issue
6
Year of publication
1997
Pages
978 - 982
Database
ISI
SICI code
0007-0963(1997)137:6<978:WAWIHS>2.0.ZU;2-W
Abstract
Wells' syndrome, or eosinophilic cellulitis, is characterized by recur rent cutaneous swellings which resemble acute bacterial cellulitis, an d by distinctive histopathological changes. Skin lesions show dermal e osinophilic infiltration and the characteristic 'flame figures', which are composed of eosinophil major protein deposited on collagen bundle s. The idiopathic hypereosinophilic syndrome is a multisystem disease with a high mortality rate. It is characterized by peripheral blood eo sinophilia and eosinophilic infiltration of many organs, including the skin. The most common skin lesions are pruritic maculopapules and nod ules over the trunk and limbs, with urticaria and angio-oedema. In con trast to Wells' syndrome, the pathology of these skin lesions is non-s pecific with variable eosinophil infiltration. We report overlapping c linical and histopathological findings characteristic of both syndrome s in one patient. Our data favour the hypothesis that both syndromes r epresent an abnormal eosinophilic response to a variety of underlying diseases or causative agents and thus are different expressions of one disease entity linked to the immunobiology of eosinophils.