title: Protracted superficial Wegener granulomatosis in a child.
abstract: Wegener granulomatosis is a rare, chronic, multisystemic vasculitis affecting mainly the upper and lower respiratory tracts together with glomerulonephritis, but the disease may involve any other organ. Protracted superficial form is a rare variant of the disease. We report a case of protracted superficial Wegener granulomatosis in a 16-year-old boy in whom the disease started with recurrent digital ulcers at age 7 years. Later, he developed nodules and papules associated with upper airway involvement and ocular keratitis without lung or renal involvement. C-ANCA was positive. The patient was treated with oral prednisone. Similar cutaneous and mucosal lesions developed during two relapses of the disease without renal or respiratory involvement.
authors.affiliation: Department of Internal Medicine, La Rabta hospital, Tunis, Tunisia.
authors.forename: Imed
authors.initials: I
authors.lastname: Ben Ghorbel
authors.forename: Asma Sioud
authors.initials: AS
authors.lastname: Dhrif
authors.forename: Mohamed
authors.initials: M
authors.lastname: Miled
authors.forename: Mohamed Habib
authors.initials: MH
authors.lastname: Houman
mesh_terms: D000293:Adolescent; D005385:Fingers; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D008297:Male; D009297:Nasal Mucosa; D011241:Prednisone / Q000627:therapeutic use; D012883:Skin Ulcer / Q000209:etiology*; D014034:Toes
chemical_list: D005938:Glucocorticoids; D011241:Prednisone