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Year : 1995  |  Volume : 61  |  Issue : 3  |  Page : 166-167

Incontinentia pigmenti

Rakesh Bharti, Singh Manjit Bal 

Correspondence Address:
Rakesh Bharti


A case of incontinentia pigmenti, a rare genodermatosis, is being presented.

How to cite this article:
Bharti R, Bal SM. Incontinentia pigmenti.Indian J Dermatol Venereol Leprol 1995;61:166-167

How to cite this URL:
Bharti R, Bal SM. Incontinentia pigmenti. Indian J Dermatol Venereol Leprol [serial online] 1995 [cited 2020 Feb 29 ];61:166-167
Available from: http://www.ijdvl.com/text.asp?1995/61/3/166/4191

Full Text


Case reports of incontinentia pigmenti, a rare genodermatosis, are not many.[1][2][3][4][5] Inherited by a sex linked dominated gene and lethal to males, incontinentia pigmenti classically has manifestations of linear vesicular lesions, evolving into verrucous lesions within few weeks, followed by a peculier swirled pigmentation lasting for many years.[6]

 Case Report

A 7-month-old female child, second in order, born to non-consanguineous parents, with an uneventful normal vaginal delivery, was brought with the complaints of vesiculobullous eruptions on both lower limbs and right arm, almost since the day of birth and lasting for one to two months. These lesions then appeared on the trunk and subsequently all healed leaving warty lesions which flattened at places, except in inguinal regions. They left slaty brown pigment which was bizarre, linear and in spiral fashion. No vesicobullous lesions however, appeared in last two months.

Systemic examination including that of eyes, CNS and skeletal system, revealed nothing abnormal. Routine blood, urine and stool examinations did not show any abnormality except for 12 % eosinophils on differential leukocyte counting. VDRL of mother and the child was nonreactive. Histopathological examination of skin biopsy showed brown coloured melanin pigment in the cells of the basal layer at many places and also in the upper dermis inside the melanophages. In other areas of basal layer, melanin was diminished and the cells showed vacuolization. Mild mononuclear infiltrate and few eosinophils were present in the dermis. The epidermis showed acanthosis and hyperkeratosis. The biopsy findings were consistent with the clinical diagnosis of incontinentia pigmenti.

The other child of the couple, a male, elder to this female child, is fine and having no abnormality.


There is only a small number of cases of incontinentia pigmenti reported earlier in Indian literature. Our case report will only be enlarging the otherwise short list. Although eosinophilia and histopathological findings substantiate the clinical suspicion of incontinentia pigmenti, yet a normal healthy male sibling belies the lethality of incotinentia pigmenti gene; and we wish the elder brother of the patient, a long and healthy life.


1Pavithran K, Ramachandran P, Zochraih J. Incontinentia pigmenti. Ind J Dermatol Venereol Leprol 1984;50:274.
2Ramalakshmi M, Parthasarthi A. Incontinentia pigmenti. Ind J Dermatol Venereol Leprol 1987;53:244-5.
3Gharpuray MB, Joshi MB, Naik SV, et al. Incontinentia pigmenti stage II. Ind J Dermatol Venereol Leprol 1987;53:122-3.
4Handa F, Aggarwal RR, Sharma SC, et al. Incontinentia pigmenti. A case report with review of literature. Ind J Dermatol Venereol Leprol 1975;41:63-5.
5Jain VK, Kalla G Bumb RA. Incontinentia pigmenti. Ind J Dermatol Venereol Leprol 1992;58:39-40.
6Lever WF, Lever GS. Histopathology of skin. 7th edn. Philadelphia : J B Lippincott, 1990;93-5.


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