|Year : 1997 | Volume
| Issue : 6 | Page : 368-369
Source of Support: None, Conflict of Interest: None
A case of incontinentia pigmenti, a rare genodermatosis is being reported from Kingdom of Saudi Arabia.
Keywords: Incontinentia pigmenti
|How to cite this article:|
Agarwal P. Incontinentia pigmenti. Indian J Dermatol Venereol Leprol 1997;63:368-9
Incontinentia pigmenti (Bloch Sulzberger syndrome) is an uncommon genodermatosis that primarily affects female infants, inherited by X-linked dominant gene and is lethal in males. Classically, it manifests with linear vesicular lesions evolving into verrucous lesions within a few weeks and followed by peculiar swirled pigmentation which lasts for many years. ,
Although cases of incontinentia pigmenti have been reported from many countries, to the author's best knowledge, this is the first case being reported form Kingdom of Saudi Arabia.
| Case Report|| |
A one-month-old female baby was presented with vesicular eruptions involving all four extremities and bizarre slaty brown pigmentation over the trunk [Figure - 1]. The baby was fourth in order, born to non consanguineous parents with normal full term delivery. In two months span, the vesicular eruptions healed leaving warty lesions. At the end of second month a few new vesicular lesions appeared over scalp which healed in another six weeks time without any atrophy. Systemic examination including ocular, CNS and skeletal system revealed nothing abnormal. Eosinophils in blood count were normal at one month age which became 12% during third month. Histologically, skin biopsy from thigh at one month age, revealed intraepidermal vesicles with eosinophils inside and infiltrate consisting of mononuclear cells and eosinophils in the dermis [Figure - 2].
| Discussion|| |
Incontinentia pigmenti is a rare and unusual genodermatosis with distinctive clinical and histopathologic feature. The statistical analysis report of Carney (1976) confirmed the salient features of incontinentia pigmenti as: X-linked dominant inheritance, marked female predominance, brown to grey pigmentation in certain configurations, three stages in many patients (vesiculobullous with eosinophilia, verrucous and pigmented) and various congenital anomalies.  The patient presented had vesicular and verrucous stages of incontinentia pigmenti over extremities and pigmentary changes over trunk. There was no other congenital abnormality. Eosinophilia and histopathological evidence of intraepidermal vesicles filled with eosinophils further supported the diagnosis in our patient.
| References|| |
|1.||Wiklund DA, William L, Weston L, Incontinentia pigmenti : A four generation study. Arch Dermatol 1980; 116: 701-703. |
|2.||Barnes C M. Incontinentia pigmenti : a report of a case with persistent activity into adult life. Cutts 1978; 22:621-624. |
|3.||Carney R G. Incontinentia pigmenti : a world statistical analysis. Arch Dermatol 1976; 112:535. |
[Figure - 1], [Figure - 2]