Linear and Whorled Nevoid Hypermelanosis and Axenfeld-Rieger Anomaly: A Novel Association

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.…”

“…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].…”