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[P236]A case of Marie-Unna hereditary hypotrichosis
( Hyun-bin Kwak ),( Soo-han Woo ),( Seok-kweon Yun ),( Han-uk Kim ),( Jin Park ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1
Marie Unna hereditary hypotrichosis (MUHH) is a rare autosomal dominant genodermatosis characterized by progressive non-scarring hair loss starting in early childhood and aggravating at puberty. Recent progress has been made in determining the genetic etiology of MUHH. Until now, two possible loci for MUHH have been mapped to chromosome 8p21 and 1p21.1.1q21.3. Herein, we report a 33-year-old male with characteristic clinical features suggestive of MUHH in previously affected Korean family. A 33-year-old Korean man presented with progressive alopecia of the scalp since childhood. On examination, hair loss patches were prominent on the vertex and occipital margins, with coarseness and kinking in texture. Eyebrows, eyelashes, and axillary and pubic hair were also scanty. There were no other ectodermal and systemic abnormalities. Laboratory findings, including hormone analyses, were within normal limits. Histopathologic examination revealed decreased and miniaturized hair follicles, with trichomalacia and some bizarre hair shaft morphology, pigment clumps in fibrous root sheath. Scanning electron microscopy revealed thickening, longitudinal grooving, twisting, and longitudinal and transverse breakage of the hair shaft. At least three members among three generations of the patient’s family, including her mother and sister displayed similar manifestations. Based on these clinicopathological findings, the patient was diagnosed with MUHH.
김영기(Young Gee Kim),이은소(Eun So Lee),이광훈(Kwang Hoon Lee) 대한피부과학회 1990 대한피부과학회지 Vol.28 No.1
We report a family suffering from Marie Unnas hypotrichosis (8 members affected in 4 generations). At birth they had normal hairs, subsequently loss of hair on the scalp, eyebrow and eyelash. At about 3rd years, coarse twisted scalp hair regrow until puberty when progressive loss from vertex and scalp margin was noticed. On physical examination and lsboratory studies, there was no associated abnormality in the patients. The scanning electron microscopic examination of the affected hair showed cuticular peeling and twisting or angulation of hair shaft. (Kor J Dermatol 28(1): 112 117, 1SSO)