Linear and Whorled Nevoid Hypermelanosis

Abstract

A case of hyperpigmentation distributed in streaks and whorls was recently delineated and separated from a confusing number of pigmentary disorders. A 13-year-old boy had hyperpigmented, reticulate spots on his trunk and neck in a linear and whorled distribution following the lines of Blaschko. Onset was at age 1 year. Histologically, the spots showed increased melanin in the basal layer of the epidermis, but no increase in melanocytes, incontinence of pigment, or melanophages were seen in the dermis. The karyotype was 46,XY and there was no evidence of chromosomal mosaicism or chimerism. This entity represents a disorder separated recently from other pigmentary alterations that follow Blaschko lines, such as Bloch-Sulzberger incontinentia pigmenti, hypomelanosis of Ito, early systematized epidermal nevus, and chimerism.

Linear and whorled nevoid hypermelanosis with hyper IgE syndrome

“…Skin changes are usually one-sided, and eruptions occupy the trunk [25]. In our patient, clinical, dermoscopic and histopathological examination showed characteristic features for LWNH, in line with other reported cases [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16]27]. Treatment of LWNH does not give satisfactory results.…”

“…Pigmentations are not preceded by any other skin changes. Stabilization of skin lesions usually occurs after 2-3 years [1][2][3][4]. Histopathologically it shows only epidermal melanosis [1,[5][6][7].…”

Disorders of pigmentation

“…350 Another study in which pigmentary anomalies along the lines of Blaschko were associated with abnormalities of the central nervous system included a few patients with incontinentia pigmenti and hypomelanosis of Ito; most cases could not be categorized further. 470 Included in the patterned hypermelanoses are cases reported as 'linear and whorled nevoid hypermelanosis', 463,465,[471][472][473][474][475][476][477][478][479] 'reticulate hyperpigmentation distributed in a zosteriform fashion', 480 'progressive cribriform and zosteriform hyperpigmentation', 481,482 'congenital curvilinear palpable hyperpigmentation', 483 'zebra-like hyperpigmentation', 484 'progressive zosteriform macular pigmented lesions', 485 'dyschromia in confetti' (following topical immunotherapy with diphenylcyclopropenone), 486 and 'infant with abnormal pigmentation'. 468 Dyspigmentation has also been associated with mosaic chromosome 5p tetrasomy 469 and with trisomy 20 mosaicism.…”

Genodermatosis with Reticulate, Patchy and Mottled Pigmentation of the Neck – A Clue to Rare Dermatologic Disorders

“…Most of these lentiginosis syndromes have complex associated systemic anomalies such as myxomas, cardiac and endocrine disorders, although isolated inherited generalized lentiginosis may occur [51, 55, 62, 63, 64]. Linear and whorled nevoid hypermelanosis may produce hyperpigmented, reticulate spots along the lines of Blaschko on the neck and the trunk [65]. This entity has been described under different terms such as zebra-like hyperpigmentation or zosteriform hyperpigmentation.…”