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Year : 1997  |  Volume : 63  |  Issue : 6  |  Page : 368-369

Incontinentia pigmenti

Correspondence Address:
Pramod Agarwal

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Source of Support: None, Conflict of Interest: None

PMID: 20944381

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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

How to cite this URL:
Agarwal P. Incontinentia pigmenti. Indian J Dermatol Venereol Leprol [serial online] 1997 [cited 2020 Aug 13];63:368-9. Available from:

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. [1],[2]

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 Top

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 Top

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. [3] 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 Top

1.Wiklund DA, William L, Weston L, Incontinentia pigmenti : A four generation study. Arch Dermatol 1980; 116: 701-703.  Back to cited text no. 1    
2.Barnes C M. Incontinentia pigmenti : a report of a case with persistent activity into adult life. Cutts 1978; 22:621-624.  Back to cited text no. 2    
3.Carney R G. Incontinentia pigmenti : a world sta­tistical analysis. Arch Dermatol 1976; 112:535.  Back to cited text no. 3    


[Figure - 1], [Figure - 2]


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