Linear and Whorled Nevoid Hypermelanosis: Report of a Case Associated with Cerebral Palsy

Abstract

A child had cerebral palsy and linear and whorled nevoid hypermelanosis on the right side of his body. He had spasticity and wasting in both lower limbs, with electroencephalographic changes and brain abnormalities on computerized tomographic scan. Chromosomal study of peripheral blood leukocytes found a normal male karyotype. The pathogenesis of this condition is still unclear.

Mosaic pigmentation disorders and associated syndromes

“…However, at least from the pictures and descriptions provided, I think that several of them probably featured a Blaschkolinear distribution, albeit less extensive and somehow incomplete when compared to full‐blown, systematized instances. Indeed, several syndromic cases reported in the past featured a limited segmental, macular or somehow mottled distribution, 4–6 thus matching even the less well‐defined cases by Cabrera et al. 1 …”

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.…”

“…Histopathologically it shows only epidermal melanosis [1,[5][6][7]. Linear and whorled nevoid hypermelanosis may be associated with congenital defects relating to the nervous, cardiovascular and skeletal systems and ocular anomalies [8][9][10][11][12]. The coexistence of hemiatrophy, Axenfeld-Rieger syndrome, inflammatory linear verrucous epidermal nevus (ILVEN), ichthyosis vulgaris and cerebrovascular malformations is also described [13][14][15][16].…”

“…Hong et al [20] described inversion of chromosome 9 corresponding with LWNH. Furthermore there are also described cases of the occurrence of LWNH inherited in subsequent generations [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21]. The differential diagnosis includes incontinentia pigmenti, epidermal nevi, hypomelanosis of Ito, Goltz syndrome and Moulin syndrome [22][23][24][25][26].…”

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.…”