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A 42-year-old white woman presented with a 25-year history of multiple painful cutaneous nodules on her back, arms, and legs. The first lesion appeared on one of her arms when she was a child. As she aged, the lesions became larger, more numerous, and more painful with cold or physical contact. Her medical history was significant for a resection of 2 benign mammary nodules at the age of 18 years, partial epilepsy at the age of 24 years, and both hysterectomy and resection of a left temporal astrocytoma at the age of 30 years. Two years later, the findings of a radiologic examination and a clinical course that was suggestive of an astrocytoma led to the diagnosis of a second cerebral lesion. A simple clinical and radiologic follow-up was decided on.
The patient’s medications included carbamazepine, gabapentin (subsequently changed to lamotrigine), and phenytoin in case of a crisis. Despite these treatments, her seizures became more and more frequent and the multiple cutaneous nodules remained painful. Her family history was notable in that similar cutaneous lesions had developed on her son and on every maternal relative and her maternal grandmother also had a cerebral tumor. Her 2 sisters and her mother had undergone hysterectomies as well. Physical examination revealed more than 50 pinkish papules, measuring up to 1 cm in diameter, mainly localized on the extensor surface of her limbs. There were similar lesions in a grouped arrangement on her back (Figure 1 and Figure 2). She also had right-sided hypoesthesia.
Figure 1.
Figure 2.
A skin biopsy specimen was obtained from a nodule on her arm (Figure 3).
Figure 3.
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