A Case of Linear and Whorled Nevoid Hypermelanosis Associated with Congenital Hemihypertrophy / 대한피부과학회지

Linear and whorled nevoid hypermelanosis(LWNH) is a congenital disorder characterized by hyperpigmented macules in a linear and whorled pattern along Blaschko's lines, and develops a few weeks after birth. Skin manifestations closely resemble hypomelanosis of Ito with inverse pigmentation. There is no preceding inflammatory event and no pigmentary incontinence histologically. It may be associated with various congenital abnormalities. We report a case of linear and whorled nevoid hypermelanosis associated with congenital hemihypertrophy of left extremities in a 10-year old boy.

A Case of Linear and Whorled Nevoid Hypermelanosis with Intracranial Lipoma / 대한소아신경학회지

Linear and whorled nevoid hypermelanosis(LWNH) is characterized by macules in streaks and whorls along Blaschko's lines, that are not preceded by inflammation. LWNH can present with various findings such as neurologic, musculoskeletal, ophthalmologic, facial and cardiac abnormalities. Intracranial lipoma is very rare condition, with an incidence of less than 1% of all intracranial tumors. We recently experienced a case of LWNH with intracranial lipoma of corpus callosum. We report this case with a brief review of the related literature.

A Case of Linear and Whorled Nevoid Hypermelanosis / 대한피부과학회지

Linear and whorled nevoid hypermelanosis(LWNH) is a congenital disorder which was first described by Kalter in 1988 and is characterized by asymmetrically distributed linear and whorled hyperpigmentation following Blaschko's lines. It usually appears at birth or during the first weeks of life and is rarely associated with various congenital defects. Its pathogenesis is not well known, but developmental somatic mosaicism appears the most likely cause. It is probably not a rare disease, but not well recognized or is confused with other entities. Herein, we report a typical case of LWNH in 9 month-old boy.

Linear and whorled nevoid hypermelanosis with delayed psychomotor development

We report a case of a 25-month-old girl presented to us for the evaluation of a severe delayed psychomotor development who also has pigmentary abnormalities. Linear and whorled hyperpigmentations following Blaschko's lines were noticed on her entire body except on her face, palms, soles, eyes and mucous membranes, which closely resembled those found in hypomelanosis of Ito, but inversely pigmented. Histologic examination revealed basal layer hyperpigmentation without incontinence of pigment or dermal melanophages. Chromosomal analysis of cultured peripheral leukocytes and fibroblasts from the hyperpigmented and the hypopigmented skin revealed normal female karyotype with no evidence of mosaicism or chimerism. This entity represents a kind of neurocutaneous syndrome-referred to by some authors as linear and whorled nevoid hypermelanosis.