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A case of multiple aneurysmal fibrous histiocytomas

SIR, Aneurysmal fibrous histiocytoma (AFH) is a variant form of dermatofibroma, characterized by blood-filled spaces within the fibrohistiocytic tumour. Although cases of multiple dermatofibromas associated with a connective tissue disease (CTD) have been reported, to our knowledge no case of multiple AFHs has been described previously. We report a case of multiple AFHs and dermatofibromas in a Japanese woman with Sjögren syndrome. This case suggests that AFH may result from slow extravasation of blood into the tumour. A 38-year-old woman presented at our clinic with multiple cutaneous nodules and papules mainly on the extremities. The patient first noticed the lesions when she was 18 years of age, and some of the lesions had sometimes bled spontaneously for the last 3-year period. On physical examination, 18 small brown nodules and seven dark-red crusted nodules, which were asymptomatic and elastic hard, were located mainly on the lower legs as well as on the chest, arms and neck (Fig. 1). Histopathological examination of three specimens excised from the lesions on the neck and both arms (Fig. 1a-c) revealed similar findings. The tumour on the left arm was composed of abundant spindle-shaped fibroblasts, histiocytes and occasional multinucleated giant cells, and was characterized by the presence of many clefts or multishaped bloodfilled tissue spaces of various sizes surrounded by spindle cells rather than endothelial cells. The nuclei of these cells showed no evidence of atypia. CD34 immunostaining was negative. The epidermis above the tumour was atrophic, but slight acanthosis was observed at the periphery (Fig. 2). Based on these findings, the diagnosis of AFH was established. The lack of endothelial cells and especially the negative CD34 immunostaining argue against several other diagnoses including vascular tumours, e.g. Kaposi’s sarcoma, angiosarcoma and spindle cell haemangioma. As several cases of systemic lupus erythematosus (SLE) with multiple dermatofibromas have previously been reported, we investigated the patient for CTD. Laboratory studies revealed leucopenia (lowest leucocyte count 2Æ8 · 10 L), an antinuclear antibody titre of 1 : 2560 with a speckled pattern, an anti-SS-A antibody titre of 164Æ0 index (normal 0–7) and an