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Indian Journal of Dermatology
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E-IJD CORRESPONDENCE
Year : 2015  |  Volume : 60  |  Issue : 3  |  Page : 325
Linear and whorled nevoid hypermelanosis with tetralogy of fallot


1 Department of Medicine of Sensory and Motor Organs, Division of Dermatology, Faculty of Medicine, Tottori University, Tottori; Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
2 Department of Medicine of Sensory and Motor Organs, Division of Dermatology, Faculty of Medicine, Tottori University, Tottori, Japan
3 Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan

Date of Web Publication6-May-2015

Correspondence Address:
Takamichi Ito
Department of Medicine of Sensory and Motor Organs, Division of Dermatology, Faculty of Medicine, Tottori University, Tottori; Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, Fukuoka
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.156485

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How to cite this article:
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

How to cite this URL:
Ito T, Yoshida Y, Goto H, Furue M, Yamamoto O. Linear and whorled nevoid hypermelanosis with tetralogy of fallot. Indian J Dermatol [serial online] 2015 [cited 2021 Aug 3];60:325. Available from: https://www.e-ijd.org/text.asp?2015/60/3/325/156485


Sir,

Linear and whorled nevoid hypermelanosis (LWNH) is a rare disease with whorled hyperpigmentation without a preceding inflammatory process. [1] We present here an unusual case of LWNH with various associations.

A one-year-old Japanese boy presented with many pigmented macules that were symmetrically distributed on his trunk and extremities [Figure 1]. They had been noted just after birth without a distinct preceding inflammatory process or vesicular eruption. Dermoscopy demonstrated antler-like brown pigmentations [Figure 2]. The patient also had growth retardation (approximately 4 SD below the anticipated value in height and weight) and cryptorchidism. Of note, he had suffered from tetralogy of Fallot at birth and had undergone surgical treatments. There was no family history in his patients or relatives. Histopathologically, basal hyperpigmentation was increased without any other definite epidermal changes, atrophy, pigmentary incontinence or dermal inflammatory infiltrate [Figure 3]. These histological changes are non-specific and only suggest a diagnosis of LWNH. Based on clinical and histological findings, a diagnosis of LWNH was made.
Figure 1: Symmetrical linear and whorled brown macules following Blaschko's lines

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Figure 2: Dermoscopy. Antler-like brown pigmentations

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Figure 3: Basal hyperpigmentation without atrophy, pigmentary incontinence or inflammatory infiltration (Fontana-Masson staining, 200)

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LWNH is characterized by swirls and streaks of macular hyperpigmentation along the Blaschko's lines and usually appears within the first 2 years of life. [1] Different descriptive names including zebra-like hyperpigmentation and reticulate hyperpigmentation of Iijima has been used to describe the same condition. [1] LWNH is occasionally associated with systemic abnormalities such as developmental retardation, facial and body asymmetry, and cardiac defects, [1],[2] though tetralogy of Fallot as seen in our case is extremely rare. [3]

Distinct causes of LWNH are still unclear. One possibility is a developmental somatic mosaicism leading to the proliferation and migration of two mixed populations of melanocytes with different potentials for pigment production. [4] Indeed, several chromosomal abnormalities including mosaic trisomy 7, 14, 18 and 20 as well as X-chromosomal mosaicism have been reported in LWNH. [5]

Differential diagnosis includes incontinentia pigmenti, hypomelanosis of Ito, linear epidermal nevus and Goltz syndrome. Incontinentia pigmenti usually shows characteristic inflammatory vesicular and verrucous stages preceding the whorled pigmentation and exclusively affects females. Hypomelanosis of Ito is characterized by linear or whorled hypopigmentations that resemble those in LWNH but are inversely hypopigmented. In epidermal naevus, epidermal nevi become prominently elevated and verrucous with time, showing more definite papillomatosis and rete ridge elongation. In Goltz syndrome, the characteristic histopathologic finding is definite dermal hypoplasia. Our case can be clearly differentiated from these diseases.

It is of interest that dermoscopy showed antler-like brown hyperpigmentations in our case. Dermoscopy is a non-invasive diagnostic tool for various skin diseases. Although typical dermoscopic features of LWNH have not been clarified, dermoscopy might provide an additional clue for diagnosis.

 
   References Top

1.
Di Lernia V. Linear and whorled hypermelanosis. Pediatr Dermatol 2007;24:205-10.  Back to cited text no. 1
    
2.
Pinheiro A, Mathew MC, Thomas M, Jacob M, Srivastava VM, Cherian R, et al. The clinical profile of children in India with pigmentary anomalies along the lines of Blaschko and central nervous system manifestations. Pediatr Dermatol 2007;24:11-17.  Back to cited text no. 2
    
3.
Kuwahara RT, Henson T, TuncaY, Wilroy SW. Hyperpigmentation along the lines of Blaschko with associated chromosome 14 mosaicism. Pediatr Dermatol 2001;18:360-1.  Back to cited text no. 3
    
4.
Happle R. Mosaicism in human skin. Understanding the patterns and mechanisms. Arch Dermatol 1993;129:1460-70.  Back to cited text no. 4
    
5.
Hartmann A, Hofmann UB, Hoehn H, Broecker EB, Hamm H. Postnatal confirmation of prenatally diagnosed trisomy 20 mosaicism in a patient with linear and whorled nevoid hypermelanosis. Pediatr Dermatol 2004;21:636-41.  Back to cited text no. 5
    

What's new?
Linear and whorled nevoid hypermelanosis can be accompanied by tetralogy of Fallot.


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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