LOCALIZED HYPERTRICHOSIS ASSOCIATED WITH PERIORBITAL NEUROFIBROMA - CLINICAL FINDINGS AND DIFFERENTIAL-DIAGNOSIS

Background: Congenital localized hypertrichosis in the periorbital reg ion is an uncommon finding. The authors report two patients with hyper trichosis and cutaneous hyperpigmentation overlying a periorbital neur ofibroma. Methods: In addition to a complete ophthalmic and systemic e xamination, the patients underwent computed tomography of the head and biopsy of the tumor, Results: Case 1 previously had received a diagno sis of neurofibromatosis type I. On examination, hyperpigmentation, hy pertrichosis, and swelling in the right supraorbital region were noted . A computed tomographic scan showed a tumor in the same region. The t umor was removed, and a plexiform neurofibroma was diagnosed. Case 2 w as admitted with hyperpigmentation, hypertrichosis, and swelling of th e left half of her face. Other signs of neurofibromatosis were absent. A computed tomographic scan showed a tumor, which was underlying the skin changes. Results of histologic examination of the biopsy specimen showed a plexiform neurofibroma. Conclusion: Neurofibroma-associated hypertrichosis should be considered in the differential diagnosis of c ongenital localized hypertrichosis.