title: [Pilonidal sinus in a patient with Buckley's syndrome. A case report].
abstract: Buckley's syndrome is a nosologic entity characterized by the presence from birth of extremely elevated levels of circulating immunoglobulin E and an important deficiency of polymorphonuclear chemotaxis. The consequences of such alterations are represented by the premature rising of recurrent cutaneous, pulmonary and articular infections, slowing down of growth and quite significant disturbances of the immune system. We report a case which presents all the characteristics of Buckley's syndrome: familial history for atopy; otorhinolaryngologic, gynecologic and pulmonary recurrent infections from birth (caused by Staphylococcus, Candida albicans but also Proteus, Enterococcus, Pyocyanea and Moraxella) some of which necessitate surgery therapy; immune disturbances. The patient had hospitalized in our Surgery Department for sinus pilonidalis abscess. At a first treatment of removal of the primary orifice and accurate curettage of the cavity (Marti's intervention) there followed another operation of larger exeresis for an abscess relapse of the sinus at a distance of 2 months. In the interval between the two treatments, an eczematous patch of 8 cm diameter appeared on the left buttock, a probable manifestation of the state of hypersensitivity of the patient. We believe, therefore, that in patients with Buckley's syndrome who must be subjected to surgical intervention it is necessary to resort to a radical treatment from the beginning, given the particular tendency to an infection relapse presented in these cases.
authors.affiliation: Service de Chirurgie Générale, Hôpital International, de l'Université de Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Schiffino
authors.forename: J
authors.initials: J
authors.lastname: Mouro
authors.forename: H
authors.initials: H
authors.lastname: Levard
authors.forename: F
authors.initials: F
authors.lastname: Dubois
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D007073:Immunoglobulin E; D010864:Pilonidal Sinus / Q000150:complications* / Q000601:surgery; D012008:Recurrence; D012086:Reoperation; D013577:Syndrome
chemical_list: D007073:Immunoglobulin E
vernacular_title: Sinus pilonidalis in paziente con sindrome di Buckley. Presentazione di un caso.