Poster presentation, P101

Official XXIst International Pigment Cell Conference website - 21-24 Sept 2011, Bordeaux - France | updated: September 04 2011

Linear and Whorled Nevoid Hypermelanosis and Progressive Cribriform and Zosteriform Hyperpigmentation in Korea

Linear and whorled nevoid hypermelanosis (LWNH) is a rare skin condition characterized by swirls and whorls of hyperpigmented macules in a streaky configuration along the Lines of Blaschko, without preceding inflammation or atrophy. LWNH was first described as a distinctive entity in 1988 by Kalter. Histologically, it showed hyperpigmentation of basal layer without pigmentary incontinence or increase in dermal melanophage. Progressive cribriform and zosteriform hyperpigmentation (PCZH) was first described in 1978 by Rower and his colleagues as following clinical characteristics: a single area of uniformly-tan, cribriform, macular pigmentation in a zosteriform distribution, histologically a mild increase of melanin pigment in the basal layer and complete absence of nevus cells. LWNH has been used to encompass as wide-spectrum of clinical entities. PCZH is thought to be a variant of LWNH. Recently, we reviewed clinical and histological features of 3 cases of LWNH and 10 cases of PCZH from our hospital records for the past 5 years. Additionally, we further analyzed 12 cases of PCZH and LWNH from the Korean Dermatological Literature. We herein analyzed Korean cases of both diseases and reviewed the literature. In the PCZH, the age at onset ranged from birth to 64 years and in the LWNH, the age at onset ranged from birth to 14 years. Associated abnormalities including congenital hemi-hypertrophy, neuro-developmental delay and Becker’s nevus were reported.

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