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Indian Pediatr 2014;51: 679-680

Non-bullous Congenital Ichthyosiform Erythroderma


Indrashis Podder and Anupam Das

Department of Dermatology, Medical College and Hospital, Kolkata, India.
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A 4-day-old neonate was brought with complaints of fissuring and peeling of skin involving almost the whole body. The neonate had generalized desquamation not even sparing the palms, soles and face. The parents informed that the baby was born, enclosed in a constricting parchment-like membrane (collodion baby) that had gradually come-off. There was a past history of a child born with similar presentation in the previous pregnancy. There was no history of consanguinity.

Fig. 1 Generalized fissuring and peeling of skin.

On examination, the entire body surface showed extensive fissuring and peeling of skin (Fig.1). Diffuse erythema and scaling was also noted, scales being larger on the legs and finer at other places. No bullae, vesicles or quadrilateral scales could be found. There was no ectropion, eclabium or cicatricial alopecia. On histological examination, parakeratosis and perivascular neutrophilic infiltration was present. The neonate was advised symptomatic treatment with topical keratolytics and emollients.

Non-bullous congenital ichthyosiform erythroderma belongs to the family of autosomal-recessive ichthyosis. Lamellar ichthyosis (large, quadrilateral scales adherent at the centre) and Harlequin fetus are the other ichthyosiform disorders transmitted in recessive fashion. Apart from these varieties, ichthyosis may be autosomal dominant (Ichthyosis vulgaris; characterized by sparing of the flexures and presence of features suggestive of atopic dermatitis and keratosis pilaris) or X-linked ichthyosis (history of prolonged labor, only in males, presence of cryptorchidism, characteristic pre-auricular involvement).

 

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