|Year : 1992 | Volume
| Issue : 1 | Page : 39-40
VK Jain, G Kalla, RA Bumb
V K Jain
Source of Support: None, Conflict of Interest: None
A 10-week old female child of Incontinentia pigmenti with linear and bizarre slaty brown hyperpigmented and verrucous lesions on both lower extremities is reported.
Keywords: Incontinentia pigmenti
|How to cite this article:|
Jain V K, Kalla G, Bumb R A. Incontinentia pigmenti. Indian J Dermatol Venereol Leprol 1992;58:39-40
| Introduction|| |
Incontinentia pigmenti (IP) is a rare genodermatosis. Only a few cases have been reported from India. ,,, The disease is inherited by a sex-linked dominant gene which is lethal to male. , Classically, the infant manifests linear vesicular lesions that evolve into verrucous lesions within a few weeks, to be followed by a peculiar swirled pigmentation that lasts for years. 
| Case Report|| |
A 10-week-old female child was brought to us for slaty brown pigmentation and a few warty lesions over both lower extremities and trunk.
The child was born asymptomatic at full term. On 4th day her parents noticed multiple vesiculo-bullous eruptions in linear groups over both feet. The recurrent crops continued for about a week and spread over whole of both lower limbs and buttocks. Few scattered lesions were also present on trunk. They healed in about a week to leave warty lesions, which flattened at most of the places in 6-8 weeks except at back of right big toe and right knee [Figure - 1] and left slaty brown pigmentation in characteristic bizarre, linear and spiral fashion [Figure - 2]. Systemic examination did not reveal any abnormality.
Haemogram showed eosinophilia of 10% and absolute eosinophil count was 780/cumm. VDRL test of the patient and her parents was non-reactive.
There was no history of consanguinity. Both parents were normal. Obstetric history of mother revealed that her first three consecutive conceptions resulted in preterm, stillborn and macerated males at the gestational age of 7 months, 5 months and 6 months respectively. Mother's blood group was `O' positive, fasting blood sugar was 83 mg% and serum creatinine was 1.3 mg%. Tests for cervical competence were normal and ultrasonography revealed no other anatomical malformation which could cause recurrent loss of pregnancies.
Since parents did not allow. a biopsy of skin, so histopathological examination couldn't be done.
| Comments|| |
IP has been reported quite infrequently. The characteristic clinical features with eosinophilia confirmed the diagnosis in our case. Consecutive loss of 3 male products of conception may be due to IP gene because the disease is lethal in male.
| References|| |
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|3.||Gharpuray MB,. Joshi MB, Naik SV, et al. Incontinentia,pigmenti stage II. IndJ Dermatol Venereol Leprol 1987; 53: 122-3. |
|4.||Handa F, Aggarwal RR, Sharma SC, et al. Incontinentia pigmenti - Case Report with review of literature. Ind J Dermatol Venereol 1975; 41 : 63 - 5. |
|5.||Wiklund DA, William L, Weston MD. Incontinentia pigmenti - A four generation study. Arch Dermatol 1980; .116 : 701 - 3. |
|6.||Gordon H, Gordon W. Incontinentia pigmentiClinical and genetic study of two familial cases. Dermatologica 1970; 140: 150 - 2. [PUBMED] |
|7.||Carney RG. lncontinentia pigmenti -.A reprot of five cases and review of literature. Arch Dermatol 1951; 64: 126-35. |
[Figure - 1], [Figure - 2]
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