LAUSR

Dear Editor, Eosinophilic angiocentric fibrosis (EAF) is a rare, idiopathic fibroinflammatory disease usually involving upper airways, nasal cavity, paranasal sinuses, and rarely the orbit. The most common presentation is a nasal mass with prolonged obstructive symptoms. Mucosal ulceration and bone destruction may be observed. Diagnosis is made through histological examination and it may show small vessel eosinophilic-vasculitis with adjacent inflammatory infiltrate of lymphocytes and plasma-cells, or an obliterative concentric perivascular fibrosis in an “onion skin” pattern as in the later fibrotic stage. Several medical treatments have been proposed including immunosuppressant-drugs and systemic steroids, however, surgical approach still represents the only effective treatment. Chronic inflammation may lead to the development of an immunocompromised district, which is a skin area more vulnerable than the rest of the body for genetic or acquired reasons, prone to developing opportunistic infections, tumors, or dysimmune reactions. Herein we report the case of a 46-year-old man showing a destructive lesion of upper lip, nasal cavity, and cleft palate. His medical history was positive for chronic cocaine abuse and plaque psoriasis not controlled by topical treatment. At the first examination, the patient showed an exuding-destructive lesion involving upper-lip, the nasal-choanae and hard-palate, with local edema and speech impairment (Figure 1A). Patient underwent incisional biopsy of the lesion. Histopathological examination revealed angiocentric fibrosis with onion skin pattern and perivascular exudates of eosinophils accompanied by plasma cells and lymphocytes. There was absence of fibrinoid necrosis. These findings were diagnostic for EAF. Laboratory investigations revealed raised erythrocyte sedimentation rate and Creactive protein, however complete blood count was in the normal range. Autoimmune screening, including antinuclear antibody, antineutrophil cytoplasmic antibody (ANCA) and rheumatoid factor, was negative. Oral prednisolone 75 mg (0.75 mg/kg body weight) was started, without showing any clinical improvements after 1 monththerapy. Therefore, considering patient's medical history of plaque psoriasis, steroid induced worsening of the chronic hypertension, and methotrexate anti-inflammatory properties related to the effects on adenosine, the association with subcutaneous methotrexate and prednisone was started (methotrexate 12.5 mg/week + prednisone 50 mg/die 0.5 mg/kg body weight). After 3 weeks of treatment, a F IGURE 1 Upper lip and nasal lesions of eosinophilic angiocentric fibrosis at baseline (A), after 3 weeks of medical treatment (methotrexate 12.5 mg/week + predisolone 50 mg/die 0.5 mg/kg body weight) (B), and after surgery (C) Received: 2 March 2021 Revised: 3 August 2021 Accepted: 5 August 2021