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Year : 2018  |  Volume : 19  |  Issue : 1  |  Page : 88-90

Twin-spot phenomenon: A rare association hypomelanosis of ito with nevoid hypertrichosis


Department of Dermatology, Calcutta National Medical College, Kolkata, West Bengal, India

Date of Web Publication28-Dec-2017

Correspondence Address:
Projna Biswas
Calcutta National Medical College, Kolkata, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_139_16

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How to cite this article:
Biswas P, Abhishek De, Pal D, Ansari A, Das S. Twin-spot phenomenon: A rare association hypomelanosis of ito with nevoid hypertrichosis. Indian J Paediatr Dermatol 2018;19:88-90

How to cite this URL:
Biswas P, Abhishek De, Pal D, Ansari A, Das S. Twin-spot phenomenon: A rare association hypomelanosis of ito with nevoid hypertrichosis. Indian J Paediatr Dermatol [serial online] 2018 [cited 2019 Jul 22];19:88-90. Available from: http://www.ijpd.in/text.asp?2018/19/1/88/206068

Sir,

Hypomelanosis of Ito (HI) is a relatively uncommon phenotype which is previously called as incontinentia pigmenti achromians where linear to whorled pattern of hypomelanotic macule follows Blaschko's line since birth without any history of verrucosity or vesicle. Neurological, musculoskeletal, dental, and ocular abnormalities are associated with HI in 70%–90%. The karyotype of HI shows chromosomal mosaicism. We are here to report a case of HI with generalized hypertrichosis with history of seizure which is a very rare association.

A 5-year-old girl was admitted to the pediatric department of our institute for the treatment of seizure and send to our department for evaluation of congenital skin lesions. She was the second daughter of a nonconsanguineous marriage; her elderly brother does not have any birthmark. There were no remarkable personal or familial antecedents. Pregnancy and labor had been uneventful. Her postnatal physical and mental development was normal, and her skin lesions remained unchanged.

On the examination, we found multiple streaks of linear hypopigmented macule following Bblaschko's line at trunk, upper limb, and lower limb [Figure 1],[Figure 2],[Figure 3]. She also had found a patch of well circumscribed dark terminal hypertrichosis on the upper back and lateral side of both upper limbs without any underlying pigmentary changes or vascular malformations [Figure 4]. Hypertrichosis in the back was presented in a whorled pattern roughly following Blaschko's line. She did not have any acneiform eruption or any sign of virilization. History revealed that these lines were also present since birth. No bony defect or dental abnormalities or eye involvement was detected. Patient's height, weight, and intelligence were normal according to her age.
Figure 1: Both lower limb with hypopigmented patch following Blaschko's lines standing position

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Figure 2: Both lower limb with hypopigmented patch following Blaschko's lines sitting position

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Figure 3: Trunk also having lesion of hypomelanosis of Ito

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Figure 4: Back shows nevoid hypertrichosis with terminal hair

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Hematological and biochemical profiles were normal. A skin biopsy from one patch of hypertrichosis showed many terminal hair follicles without any smooth muscle hyperplasia and without hyperpigmentation. A skin biopsy from a hypopigmented linear lesion showed a normal appearance in the hematoxylin and eosin staining.

HI is a neurocutaneous syndrome first described by Ito in 1952. Lesions are presented at birth along the Blaschko's line. The cutaneous lesions are characteristic and are usually noticed at birth. The hypopigmented lesions are distributed along the  Lines of Blaschko More Details and usually involve more than three body segments. Most patients have unilateral or bilateral lesions, but patchy lesions may also occur.[1] Most patients, in addition to cutaneous lesions, had multiple neurological, musculoskeletal, and other organ system involvements, on the other hand, few cases described as naevoid disorders at sites completely exclusive of each other and without any severe associated defects. In our case, the patient had a history of recurrent convulsions.

HI and naevoid hypertrichosis are both uncommon disorders and their coexistence is rarely described.

In 1991, Rogers [2] described a girl who had areas of hypertrichosis on the trunk and limbs, partly with a whorled pattern HI. In addition, this patient had epidermal nevus-like lesions, nail dystrophy, as well as neurological, gastrointestinal, ocular, pulmonary, and skeletal abnormalities. A girl described by Ballmer-Weber et al.[3] had genital hypertrichosis and in association with hypopigmented lesions following Blaschko's lines on her trunk and limbs. She also had facial dysmorphia, musculoskeletal anomalies, hernia, and mental disbalance. There was few other reports in our knowledge which describes some of the cases of same type of presentation.[4],[5],[6] Another report presented two cases of patchy nevoid hypertrichosis associated with achromic lesions following Blaschko's lines without any mental and skeletal abnormalities.[7]

Nevoid hypertrichosis is an uncommon type of localized congenital hypertrichosis consisting of a solitary circumscribed area of terminal hair growth without underlying abnormal skin pigmentation.[8],[9] Other naevoid conditions such as congenital melanocytic naevi, Becker's naevus, and smooth muscle hamartomas may also present, with localized excessive hair growth overlying the lesions, and can be differentiated clinically and histologically. Naevoid hypertrichosis usually presents as an isolated, solitary defect but the occurrence of partial lipoatrophy and bony abnormalities has been reported. The presence of multiple patches of naevoid hypertrichosis in a patient is relatively uncommon.[10] Nevoid hypertrichosis may occur in a mosaic pattern and may be associated with other cutaneous diseases that show a similar segmental pattern, as a possible twin-spot phenomenon. A coexistence of different skin conditions with a genetic origin in the same patient may be explained by the concept of twin spotting of mutant tissue that differ genetically from each other and from the surrounding normal skin.[11]

Our patient presented with this rare combination two nevoid condition HI and nevoid hypertrichosis which also is an example of twin-spot phenomenon.

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Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Ruiz-Maldonado R, Toussaint S, Tamayo L, Laterza A, del Castillo V. Hypomelanosis of Ito: Diagnostic criteria and report of 41 cases. Pediatr Dermatol 1992;9:1-10.  Back to cited text no. 1
    
2.
Rogers M. Naevoid hypertrichosis. Clin Exp Dermatol 1991;16:74.  Back to cited text no. 2
    
3.
Ballmer-Weber BK, Inaebnit D, Brand CU, Braathen LR. Sporadic hypomelanosis of Ito with focal hypertrichosis in a 16-month-old girl. Dermatology 1996;193:63-4.  Back to cited text no. 3
    
4.
Rupert LS, Bechtel M, Pellegrini A. Nevoid hypertrichosis: Multiple patches associated with premature graying of lesional hair. Pediatr Dermatol 1994;11:49-51.  Back to cited text no. 4
    
5.
Dudding TE, Rogers M, Roddick LG, Relic J, Edwards MJ. Nevoid hypertrichosis with multiple patches of hair that underwent almost complete spontaneous resolution. Am J Med Genet 1998;79:195-6.  Back to cited text no. 5
    
6.
Schauder S, Hanefeld F, Noske UM, Zoll B. Depigmented hypertrichosis following Blaschko's lines associated with cerebral and ocular malformations: A new neurocutaneous, autosomal lethal gene syndrome from the group of epidermal naevus syndromes? Br J Dermatol 2000;142:1204-7.  Back to cited text no. 6
    
7.
Lestringant GG, Topley J, Sztriha L, Frossard PM. Hypomelanosis of Ito may or may not involve hair growth. Dermatology 1997;195:71-2.  Back to cited text no. 7
    
8.
López-Barrantes O, Torrelo A, Mediero IG, Zambrano A, Happle R. Nevoid hypertrichosis and hypomelanosis. Eur J Dermatol 2002;12:583-5.  Back to cited text no. 8
    
9.
Vergani R, Betti R, Martino P, Crosti C. Giant nevoid hypertrichosis in an Iranian girl. Pediatr Dermatol 2002;19:64-6.  Back to cited text no. 9
    
10.
Sotiriadis D, Patsatsi A, Lazaridou E, Sotiriou E, Devliotou-Panagiotidou D. Multiple nevoid hypertrichosis as an isolated developmental defect. Pediatr Dermatol 2009;26:436-8.  Back to cited text no. 10
    
11.
Happle R. Loss of heterozygosity in human skin. J Am Acad Dermatol 1999;41(2 Pt 1):143-64.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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