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 . include zosteriform hyperpigmentation , zosteriform lentiginous nevus , zebra - like hyperpigmentation , reticulate hyperpigmentation of iijima , and nevoid hyperpigmentation following blaschko lines . 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 . 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 . classical whorled , hyperpigmented macules along the lines of blaschko on the trunk with pinpoint areas of depigmentation hematological and routine biochemical tests revealed no abnormalities . histopathological evaluation of the depigmented lesion showed complete absence of melanocytes [ figure 2a ] . however , the pigmented macules revealed increased pigmentation of the basal cell layer with melanocytes present up to the mid - epidermis [ figure 2b ] . no focal areas of pigmentary incontinence were noticed in the dermis . based on clinicopathological correlation , a diagnosis of lwnh with punctate hypopigmentation was made . however , treatment for depigmented areas with narrow band ultraviolet b radiation and topical corticosteroids was advised . 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 ) linear and whorled nevoid hypermelanosis is a rare disorder of pigmentation characterized by hyperpigmented macules in a linear or whorled streaky configuration . 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 . 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 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 . there is sparing of the face , palms and soles , eyes , and mucous membranes . 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 . histopathology reveals diffuse moderate hyperpigmentation in the basal layer and prominence or vacuolization of melanocytes . genetic studies suggest somatic mosaicism as a cause for lwnh with mosaic trisomy of 7 , 14 , 18 , 20 ; x - chromosomal mosaicism has been reported . 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 . 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 . 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 . 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 .
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 .