|LETTER TO EDITOR
|Year : 2018 | Volume
| Issue : 1 | Page : 88-90
Twin-spot phenomenon: A rare association hypomelanosis of ito with nevoid hypertrichosis
Projna Biswas, Abhishek De, Dayamay Pal, Asad Ansari, Sudip Das
Department of Dermatology, Calcutta National Medical College, Kolkata, West Bengal, India
|Date of Web Publication||28-Dec-2017|
Calcutta National Medical College, Kolkata, West Bengal
Source of Support: None, Conflict of Interest: None
|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 2020 Jul 9];19:88-90. Available from: http://www.ijpd.in/text.asp?2018/19/1/88/206068
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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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. 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  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. 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.,, Another report presented two cases of patchy nevoid hypertrichosis associated with achromic lesions following Blaschko's lines without any mental and skeletal abnormalities.
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., 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. 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.
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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[Figure 1], [Figure 2], [Figure 3], [Figure 4]