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CASE REPORTS

Non-bullous Ichthyosiform Erythroderma

Dr. Y. P. Fung

Date: Venue: Organizer:

9 May, 2001 Yaumatei Dermatology Clinic Social Hygiene Service, DH; Clinico-pathological Seminar

was noted with time as the collodion membrane shed, leaving generalized erythroderma and cracking and scaling of skin especially over the groin, sacral, and axillary creases (Figure 2). Resolution was near completion three months later with only mild residual ichthyosis at the sacral area.

CASE SUMMARY

Differential diagnosis

History

A full-term male neonate was delivered by Caesarian section due to meconium aspiration syndrome. After delivery, he was also noted to have a cling-film like membrane all over his skin. There was no history of consanguinity: his father was a Caucasian and his mother was a native Philipino. There was no family history of ichthyosis or skin disease.

Physical examination

The typical features of collodion baby were noted. There was a generalized glistening, taut reddish film stretched over the skin giving a "dipped in hot wax" appearance (Figure 1). There were mild ectropion and eclabion. Pinnae and nasal passages were normal. No constriction bands or blisters were noted. Improvement

The typical clinical features of collodion baby are generally easily distinguished from the severe harlequin ichthyosis (HI). But occasionally, mild form of HI has been recorded (Chrysalis babies). Restrictive dermopathy or stiff baby syndrome produces a generalized taut, thick, unyielding skin, which does not shed and may cause respiratory failure and early neonatal death. The possible outcomes of collodion baby include non-bullous congenital ichthyosiform erythroderma (NBIE), lamellar ichthyosis, rarer disorders (e.g. Netherton's syndrome, neutral lipid storage disease, trichothiodystrophy and anhidrotic ectodermal dysplasia) and normal skin (self-healing collodion baby).

Investigations Skin biopsy done at day 50 of life showed

Figure 1: Classical collodion baby with "dipped in hot wax appearance" (Day 8 of life)

Figure 2: Residual hyperkeratosis and large sheets of white scales remained after shedding of collodion membrane (Day 17 of life)

124 Hong Kong Dermatology & Venereology Bulletin

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