Wegener granulomatosis in a child: Cutaneous findings as the presenting signs

Wegener granulomatosis (WG) is a systemic disease that is particularly unus ual in children. A limited form has been described without renal involvemen t. We report a 14-year-old girl in whom the disease started with acneiform nodular and papular lesions on the forehead. Later necrotic ulcers develope d on her forehead, arms, and buttocks. The cutaneous lesions were associate d with upper and lower respiratory tract involvement, low-grade fever and a rthralgias. Subsequently clinical and laboratory evaluations (increased ESR ; leukocytosis and presence of serum IgG antibodies cANCA = 1:160), with ch est roentgenograms revealing pulmonary densities and parenchymal infiltrati on, suggested the diagnosis of WG. The histologic findings of a cutaneous b iopsy specimen were ulceration of the epidermis with diffuse neutrophilic i nflammatory infiltrate and a late-stage small vessel vasculitis in the derm is. Histopathology 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 presen t. One month of cyclophosphamide (125 mg/day) and prednisone (70 mg/day) th erapy markedly improved the patient's clinical condition. At present, 1 yea r later, she is free from any signs of the disease. According to the litera ture, 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 "acneiform" presentation is a rare finding in WG.