Linear and Whorled Nevoid Hypermelanosis: Unique Clinical Presentations and Their Possible Association With Chromosomal Abnormality inv(9)

Hong, Soon-Tae; Ahn, Sang Hyeon; Lee, Won‐Soo

doi:10.1001/archderm.144.3.415

Cited by 17 publications

(6 citation statements)

References 5 publications

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“…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.…”

Section: Patterned Hypermelanosismentioning

confidence: 99%

Disorders of pigmentation

Weedon

2010

Weedon's Skin Pathology

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Section: Patterned Hypermelanosismentioning

confidence: 99%

Disorders of pigmentation

Weedon

2010

Weedon's Skin Pathology

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“…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].…”

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confidence: 99%

Linear and whorled nevoid hypermelanosis with hyper IgE syndrome

Sieniawska¹,

Lesiak²,

Pietruszewska³

et al. 2015

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Introduction. Linear and whorled nevoid hypermelanosis (LWNH) is a disorder of pigmentation characterized by macular hyperpigmentation following the Blaschko lines. Only 50 cases of LWNH have been described so far. Linear and whorled nevoid hypermelanosis may be associated with congenital defects. Underlying chromosomal mosaicism has been demonstrated in only a few published cases. Diagnosis is based on characteristic clinical and histopathological findings. Objective. Presentation of the first Polish case of LWNH. Case report. We present a 29-year old Caucasian woman with streaks of reticulate hyperpigmented macules arranged in a whorled pattern over the trunk and extremities. Histopathology showed hyperpigmentation of the epidermal basal layer and prominent melanocytes, without pigment incontinence. Based on clinical and histopathological findings the diagnosis of LWNH was established. Additionally hyper IgE syndrome has been diagnosed in the patient. Conclusions. In LWNH the therapeutic challenge is the diagnosis and treatment of comorbidities; otherwise it may cause a serious threat to the patient's health. streszczenie Wprowadzenie. Podłużna i wirowata znamieniopodobna hipermelanoza (ang. linear and whorled nevoid hypermelanosis with hyper IgE syndrome -LWNH) jest zaburzeniem pigmentacji skóry charakteryzującym się występowaniem przebarwień ułożonych wzdłuż linii Blaschko. Do tej pory opisano ok. 50 przypadków tej choroby. Schorzenie może współistnieć z licznymi wadami wrodzonymi. W kilku opisanych przypadkach stwierdzono występowanie mozaikowatości chromosomalnej. Rozpoznanie ustala się na podstawie charakterystycznego przebiegu klinicznego i badania histopatologicznego. Cel pracy. Przedstawienie pierwszego w Polsce przypadku LWNH. Opis przypadku. Prezentujemy przypadek 29-letniej kobiety z ogniskami hiperpigmentacji o układzie linijnym i wirowym zlokalizowanymi na skórze tułowia i kończyn. W badaniu histopatologicznym wycinka skóry stwierdzono hiperpigmentację w podstawnej warstwie naskórka oraz okołonaczyniowe skupienia komórek limfoidalnych z obecnością melanofagów. Na podstawie badania histopatologicznego i obrazu kliLinear and whorled nevoid hypermelanosis with hyper ige syndrome Podłużna i wirowata znamieniopodobna hipermelanoza z współwystępowaniem zespołu hiper-ige

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“…No effective treatment is available for LWNH though chemical peels 3 , depigmenting agents 16 and lasers have been tried without much success.Cosmetic camouflage does offer some promise.…”

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confidence: 99%

Linear and Whorled Nevoid Hypermelanosis

Hazarika¹

2013

jemds

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Linear and whorled nevoid hypermelanosis is a rare sporadic skin condition characterized by swirling streaks of hyperpigmention following the lines of Blaschko. The hyperpigmentation often occurs alone, but additional symptoms have been described in individual cases. An 18 year old girl presented with insidious onset, asymptomatic, generalized, hyperpigmented streaky lines since late childhood without any other systemic abnormality. Histopathology showed increase in basal cells containing melanin without any dermal change, consistent with linear and whorled nevoid hypermelanosis. KEY WORDS: Linear, whorled, nevoid hypermelanosis, lines of blaschko. INTRODUCTION: Linear and whorled nevoid hypermelanosis (LWNH) was first described by Kalter et al 1 in 1988 as a sporadic pigmentary anomaly characterized by swirls and streaks of macular(flat) hyperpigmentation following the lines of Blaschko.Usually occurring as isolated disorder of pigmentation, associated chromosomal abnormalities with central nervous, cardiovascular, musculoskeletal and ocular abnormalities have been reported. CASE REPORT: An 18 year old female complained of hyperpigmented (dark coloured) streaky skin lesions on face, trunk and extremities, occurring insidiously, since late childhood. Lesions were first noticed on the face and subsequently elsewhere. Lesions were asymptomatic but cosmetically disfiguring. Retrospective history from mother, failed to elicit presence of fluid filled or elevated skin lesions preceding the pigmentation. She was born of full term, normal vaginal delivery, to nonconsanguineous parents, with normal growth and development.Family history was not suggestive. General and systemic examination including detailed musculoskeletal, neurological, ocular and cardiovascular assessment was normal. Cutaneous examination revealed macular hyperpigmented lesions in streaks and whorls following the lines of Blaschko. Lesions were distributed as bilaterally symmetrical linear streaks on upper and lower extremities while on the chest and trunk, lesions were patchy whorls which did not cross the midline. Facial lesions were seen over the forehead and pre-auricular areas.No skin surface changes like roughness or scaling was noted over the lesion or elsewhere. Palms, soles, mucosa, hair, nails, teeth were normal. Differential diagnoses considered were epidermal naevus, incontinentia pigmenti and linear and whorled naevoid hypermelanosis. Hemogram and metabolic profile presented no abnormalities. X ray skull and ECG were normal. CT scan brain, echocardiography and chromosomal studies could not be done due to financial constraints. Skin biopsy revealed-mild papillomatosis and diffuse increase in basal cells containing melanin without any dermal change, which was consistent with linear and whorled naevoid hypermelanosis. The benign nature of the disease was explained to the patient. She was prescribed glycolic acid 6% crème for the facial lesions along with sunscreen.

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Linear and Whorled Nevoid Hypermelanosis: Unique Clinical Presentations and Their Possible Association With Chromosomal Abnormality inv(9)

Cited by 17 publications

References 5 publications

Disorders of pigmentation

Disorders of pigmentation

Linear and whorled nevoid hypermelanosis with hyper IgE syndrome

Linear and Whorled Nevoid Hypermelanosis

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