A Case of a Surviving Male Infant with Incontinentia Pigmenti: CASE REPORT

12 Feb
2011

A 3-day-old boy was referred to our department for the evaluation of skin finding presented at birth. The lesion showed erythema, linear vesiculations, and focal crust on the trunk and leg, predominantly over the left side (Fig. 1A, B).

At the time of birth, several vesicles and ery­thematous papules developed over the left trunk and thigh along Blaschko’s lines. Blistered skin was replaced by a crusted lesion on the fifth day after birth (Fig. 1C). Then, hyperkeratotic papules and verrucous lesions occurred consecutively on the fifteenth day (Fig. 1D).

Fig. 1. (A, B) On the third day

Fig. 1. (A, B) On the third day, linear distribution of clustered vesicles on the left lower extremity and trunk along the Blaschko’s lines. (C) On the fifth day, blistered skin replaced by crusts and scaling. (D) On the fifteenth day, hyperkeratotic verrucous papules and plaques developed at the site of previous blistering.

The patient was the second child born on normal full term delivery to the patients. His parents and his elder brother were healthy, and had no other comparable dermatoses.

There was no remarkable abnormality except for the skin lesions on physical examination. Laboratory tests including complete blood count, blood che­mistry analysis, urinalysis, VDRL, bacterial culture, and TORCH serology (for toxoplasmosis, rubella, cytomegalovirus and herpes simplex) were in the normal range or negative. Also, radiologic findings in brain MRI, echocardiogram, and chest, abdomen, both extremities X-rays appeared normal. Chromo­some analysis from peripheral blood leukocytes showed a normal male karyotype (Fig. 2), non­etheless, due to the refusal of parental consent, molecular analysis was not executed. Viagra Soft Tabs

Fig. 2. Chromosome analysis

Fig. 2. Chromosome analysis from blood showed a normal male karyotype (46, XY).

A skin biopsy was taken from one of the blistered areas on the left thigh. Histopathologically, epidermal hyperkeratosis, spongiosis, dyskeratotic cells throughout the epidermis, basal liquefaction de­generation, and inflammatory infiltration composed predominantly of eosinophils were noted (Fig. 3).

Fig. 3. Histologic feature showing

Fig. 3. Histologic feature showing dyskeratotic cells throughout the spongiotic epidermis, basal liquefaction degeneration, and inflammatory infiltration composed predominantly of eosinophils (H&E, x100).

The patient was diagnosed as IP, and received antibiotic treatments to control secondary infection. After 2 months, the lesion disappeared with residual hyperpigmented streaks on the left thigh.
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