Wegener Granulomatosis in a Child: Cutaneous Findings as the Presenting Signs V. Brazzelli, M.D.,* C. Vassallo, M.D.,* F. Baldini, M.D.,* A. Ravelli, M.D.,† A. Martini, M.D.,† and G. Borroni, M.D.* *Department of Human and Hereditary Pathology, Institute of Dermatology, and †Department of Pediatrics, University of Pavia, Pavia, Italy Abstract: Wegener granulomatosis (WG) is a systemic disease that is particularly unusual in children. A limited form has been described with- out renal involvement. We report a 14-year-old girl in whom the disease started with acneiform nodular and papular lesions on the forehead. Later necrotic ulcers developed on her forehead, arms, and buttocks. The cu- taneous lesions were associated with upper and lower respiratory tract involvement, low-grade fever and arthralgias. Subsequently clinical and laboratory evaluations (increased ESR; leukocytosis and presence of se- rum IgG antibodies cANCA = 1:160), with chest roentgenograms revealing pulmonary densities and parenchymal infiltration, suggested the diagno- sis of WG. The histologic findings of a cutaneous biopsy specimen were ulceration of the epidermis with diffuse neutrophilic inflammatory infil- trate and a late-stage small vessel vasculitis in the dermis. Histopathol- ogy of the nasal mucosa was characterized by a granulomatous process with a dense lymphohistiocytic infiltrate with few giant cells, a finding that confirmed the diagnosis of WG. No renal involvement was present. One month of cyclophosphamide (125 mg/day) and prednisone (70 mg/day) therapy markedly improved the patient’s clinical condition. At present, 1 year later, she is free from any signs of the disease. According to the literature, the frequency of cutaneous lesions in WG ranges from 16% to 46%. They are the presenting sign only in 6% of patients. Cutaneous lesions are even more uncommon in children. In particular, an “acne- iform” presentation is a rare finding in WG. Wegener granulomatosis (WG) is a systemic vasculi- tis of small and medium-size vessels, affecting mostly the respiratory tract (sinuses, nose, pharynx, and lungs) and kidney, although any organ may be affected (1–3). A limited form without renal involvement has a more fa- vorable prognosis (4). WG may occur at any age, but the peak incidence is usually in middle-aged people (51.6 years for men and 47.2 for women with a male:female ratio of 1.25:1) (5–7). It is rarely observed in children (8). About 50 pediatric cases have been reported to date (5,6,8,9). We describe a young girl in whom WG presented as nodular and necrotic acneiform skin lesions on the forehead and later on the arms and buttocks. Address correspondence to Giovanni Borroni, M.D., Clinica Der- matologica, Universita ` di Pavia, Policlinico S. Matteo IRCCS, Pavia, Italy. Pediatric Dermatology Vol. 16 No. 4 277–280, 1999 277