A female infant was born to a 31-year-old gravida 2, para 1, abortion 1 woman at a gestational age of 31 weeks. The pregnancy was complicated by polyhydramnios and a cesarean section was perfo­rmed because of fetal distress. The infant weighed 1110я (<Ю percentile), the crown to heel measured 33 cm long (<10 percentile) and the head circum­ference measured 26.5 cm long (<10 percentile). Her Apgar scores were 1 at both 1 minute and 5 minutes. The arterial blood gas analysis showed pH 6.8, PC02 63 mmHg, P02 40.7 mmHg and S02 59.6%; suggesting respiratory acidosis. The chest X-ray revealed hypoaeration in both lungs. Conse­quently, an intubation of the infant was required.

The physical examination showed extensive areas of tight, rigid and shiny skin with superficial erosions, as well as visible cutaneous vessels (Fig. 1). The skin was fissured and crusted at the flexures. The extremities revealed flexion contractures of the elbows, hips, knees and ankles (Fig. 2). The infant had a characteristic face with hypertelorism, exoph- thalmia, the absence of eyebrows, hypoplastic and up-turned nose, and an open ‘O’ haped fixed mouth (Fig. 3). viagra 10 mg

fig1. shiny

Fig. 1. Shiny, taut skin with visible cutaneous vessels on the trunk and superficial erosions on the neck.

fig2. flexure

Fig. 2. Flexure contracture on the elbows, hips, knees, and ankles.

The histological examination showed flattened rete ridges and a generally-thinned dermis. The der­mis showed densely-packed collagen bundles arran­ged in parallel to the skin surface with hypoplastic appendageal structures (Fig. 4A). Elastic fibers were not detected in the dermis by the Verhoeff-van Gieson staining method (Fig. 4B).
The infant was diagnosed with restrictive dermo­pathy, and intubated, then ventilated at a neonatal intensive care unit. In spite of assisted ventilation, the infant died of respiratory failure 7 days after birth.
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fig3. the face

Fig. 3. The face shows an absence of evebrows. a small and cinched nose, micrognathia, a fixed ‘ У ;haped open mouth, and low set ears.

fig4. skin

Fig. 4. Skin biopsy showing a markedly-thinned dermis with paucity of the adnexal structures.

Category: Diseases / Tags: Congenital lethal skin disorder, Restrictive dermopathy

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