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Year : 2017  |  Volume : 8  |  Issue : 2  |  Page : 131-133  

Linear and whorled nevoid hypermelanosis with depigmentation

Department of Dermatology, Military Hospital, Kirkee, Pune, India

Date of Web Publication16-Mar-2017

Correspondence Address:
Preema Sinha
Department of Dermatology, Military Hospital, Kirkee, Pune
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2229-5178.202274

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Linear and whorled nevoid hypermelanosis (LWNH) is a rare disorder of pigmentation characterized by hyperpigmented macules in a linear or whorled streaky configuration. Lesions are distributed mainly on the trunk and extremities, sparing the palms, soles, and mucosae. Depigmentation occurring in the pre-existing whorls has not been reported till date. Herein, we report one such rare case of familial LWNH with depigmentation.

Keywords: Depigmentation, linear and whorled nevoid hypermelanosis, rare disorder

How to cite this article:
Sinha P, Chatterjee M, Singh KK, Sood A. Linear and whorled nevoid hypermelanosis with depigmentation. Indian Dermatol Online J 2017;8:131-3

How to cite this URL:
Sinha P, Chatterjee M, Singh KK, Sood A. Linear and whorled nevoid hypermelanosis with depigmentation. Indian Dermatol Online J [serial online] 2017 [cited 2022 Jan 23];8:131-3. Available from: https://www.idoj.in/text.asp?2017/8/2/131/202274

   Introduction Top

Linear and whorled nevoid hypermelanosis (LWNH) is a rare sporadic pigmentary anomaly, characterized by swirls and streaks of macular hyperpigmentation following the lines of Blaschko, without preceding inflammation, and is usually seen in the first 2 years of life.[1] The condition was first described in 1988 by Kalter et al.[1] Synonyms include zosteriform hyperpigmentation, zosteriform lentiginous nevus, zebra-like hyperpigmentation, reticulate hyperpigmentation of Iijima, and nevoid hyperpigmentation following Blaschko lines.[1],[2]

   Case Report Top

A 22-year-old male presented to the outpatient department with chief complaints of asymptomatic, dark-colored skin lesions over the body since 10 years of age. According to the patient, the lesions first appeared over both his arms and progressed within the next 2 years to involve the trunk and lower limbs. The lesions darkened with age and no new lesions appeared over the next 9 years; however, over the last 1 year he started noticing multiple white-colored, pinpoint lesions appearing over the hyperpigmented lesions without any preceding skin itching or redness. There was no history of warty lesions or blisters prior to the appearance of these lesions. There was no history suggestive of any recurrent lower respiratory infections or involvement of the cardiovascular and the central nervous systems. The patient described presence of similar dark-colored skin lesions involving the trunk, arms, and legs in his mother without a history of any light-colored patches. There was no history of consanguinity or recurrent fetal deaths in the family. There was no history of vitiligo or other autoimmune disorders in the family.

Systemic examination was unremarkable. Cutaneous examination revealed presence of multiple, whorled, hyperpigmented macules, arranged bilaterally and symmetrically, along the lines of Blaschko on the abdomen, chest, and back [Figure 1]. Similar lesions were arranged linearly on the extensor and flexor aspects of the arms and legs. Multiple pinpoint depigmented macules were seen along the areas of streaky macular pigmentation [Figure 1]. The texture of skin was normal over the streaks, the intervening skin, and over the depigmented areas. No clinical signs of Koebner phenomenon or any leukotrichia were visible.
Figure 1: Classical whorled, hyperpigmented macules along the lines of Blaschko on the trunk with pinpoint areas of depigmentation

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Hematological and routine biochemical tests revealed no abnormalities. Histopathological evaluation of the depigmented lesion showed complete absence of melanocytes [Figure 2]a. However, the pigmented macules revealed increased pigmentation of the basal cell layer with melanocytes present up to the mid-epidermis [Figure 2]b. No focal areas of pigmentary incontinence were noticed in the dermis. Based on clinicopathological correlation, a diagnosis of LWNH with punctate hypopigmentation was made. The individual was reassured of the benign nature of the disorder. However, treatment for depigmented areas with narrow band ultraviolet B radiation and topical corticosteroids was advised. A periodic follow-up was recommended at our dermatology unit. However, the patient was lost to follow up.
Figure 2: Picture collage showing histopathological examination (a) complete absence of melanocytes in the depigmented area (b) abundance of melanocytes in the hyperpigmented area (H and E, ×40)

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   Discussion Top

Linear and whorled nevoid hypermelanosis is a rare disorder of pigmentation characterized by hyperpigmented macules in a linear or whorled streaky configuration.[3] Onset is within a few weeks of age, with no preceding inflammation or palpable lesion. The usual age of the onset of hyperpigmentation is within the first few weeks of life, which continues to progress for a year or two before stabilization.[1],[4] Clinically, reticulate hyperpigmented macules coalescing to form streaks and whorled areas are seen over the trunk, extremities, and neck following the lines of Blaschko. The macules may become less prominent with age in some patients. Streaks and whorls follow the lines of Blaschko and do not cross the midline. The pigmented streaks display a V-shaped pattern over the spine, an S-shaped or whorled pattern over the anterior and lateral aspects of the trunk, and a linear arrangement over the extremities and genitalia.[5] There is sparing of the face, palms and soles, eyes, and mucous membranes.

Extracutaneous features include skeletal, neurological, and ocular abnormalities. Central nervous system diseases include microcephaly, arhinencephaly and epilepsy whereas cardiac defects include ventricular septal defect and tetralogy of Fallot. Developmental retardation, facial and body asymmetry, deafness, and brachydactyly have been mentioned in the literature in association with LWNH.[6],[7]

Histopathology reveals diffuse moderate hyperpigmentation in the basal layer and prominence or vacuolization of melanocytes. Pigmentary incontinence is usually, but not always absent. Genetic studies suggest somatic mosaicism as a cause for LWNH with mosaic trisomy of 7, 14, 18, 20; X-chromosomal mosaicism has been reported.[8]

The differential diagnosis includes incontinentia pigmenti, hypomelanosis of Ito, and epidermal nevus. Dermatoscopic feature mentioned include “net like” pattern of pigmentation in both linear and whorled parts by Naveen et al., and Ertam et al. described a “parallel” pattern which consisted of linear or circular arrangement of parallel whorled streaks along lines of Blaschko.[9],[10]

Explanation for appearance of the depigmented macules is not clear. Monogenic skin disorders are the ones commonly described with lines of Blaschko, however, polygenetic skin disorders such as psoriasis, lichen planus, segmental vitiligo, granuloma annulare, etc. can also present in similar patterns.[11] Literature search reveals an entity called as Blaschkolinear vitiligo, which has been described in association with segmental vitiligo, acrofacial vitiligo, and non-segmental vitiligo. Kovacevic et al. have reported a new entity called as “mixed vitiligo of Blaschko lines,” where they discussed presence of segmental and non-segmental vitiligo in Blaschkolinear pattern.[12]

Till date, no cases of LWNH with areas of depigmentation have been mentioned in the literature, and to the best of our knowledge, this is most probably the first case report of familial LWNH superimposed by pinpoint spots of depigmentation.

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There are no conflicts of interest.

   References Top

Kalter DC, Griffiths WA, Atherton DJ. Linear and whorled nevoid hypermelanosis. J Am Acad Dermatol 1988;19:1037-44.  Back to cited text no. 1
Metta AK, Ramachandra S, Sadath N, Manupati S. Linear and whorled nevoid hypermelanosis in three successive generations. Indian J Dermatol Venereol Leprol 2011;77:403.  Back to cited text no. 2
[PUBMED]  [Full text]  
Mendiratta V, Sharma RC, Arya L, Sardana K. Linear and whorled nevoid hypermelanosis. J Dermatol 2001;28:58-9.  Back to cited text no. 3
Brar BK, Mahajan BB, Puri N. Linear and whorled nevoid hypermelanosis. Indian J Dermatol Venereol Leprol 2009;74:512-3.  Back to cited text no. 4
Khunger N, Bansal S, Khandari R. Disorders of Hyperpigmentation. In: Sacchidanand S, Oberai C, Inamdar AC, editors. IADVL Textbook of Dermatology. 4th ed. Mumbai: Bhalani Publishing House; 2015. p. 1332-33.  Back to cited text no. 5
Alrobaee AA, Alsaif F. Linear and whorled hypermelanosis associated with developmental delay and generalized convulsions. Int J Dermatol 2004;43:145-7.  Back to cited text no. 6
Ito T, Yoshida Y, Goto H, Furue M, Yamamoto O. Linear and whorled nevoid hypermelanosis with tetralogy of fallot. Indian J Dermatol 2015;60:325.  Back to cited text no. 7
[PUBMED]  [Full text]  
Hartmann A, Hoffman UB, Hoehn H, Broecker EB, Hamm H. Postnatal confirmation of prenatally diagnosed trisomy 20 mosaicism in a patient with linear and whorled nevoid hypermelanosis. Paediatr Dermatol 2004;21:636-41.  Back to cited text no. 8
Naveen KN, Reshme P. Linear and whorled nevoid hypermelanosis with dermatoscopic features. Dermatol Online J 2014;20.  Back to cited text no. 9
Ertam I, Turk BG, Urkmez A, Kazandi A, Ozdemir F. Linear and whorled nevoid hypermelanosis: Dermatoscopic features.J Am Acad Dermatol 2009;60:328-31.  Back to cited text no. 10
Happle R. Superimposed segmental manifestation of polygenic skin disorders. J Am Acad Dermatol 2007;57:690-9.  Back to cited text no. 11
Kovacevic M, Stanimirovic A, Vucic M, Goren A, Situm M, Lukinovic SV, et al. Mixed vitiligo of Blaschko lines: A newly discovered presentation of vitiligo responsive to combination treatment. Dermatol Ther 2016;29:240-3.  Back to cited text no. 12


  [Figure 1], [Figure 2]

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