congenital ichthyosiform erythroderma

(redirected from ichthyosiform erythroderma)


abnormal redness of the skin over widespread areas of the body.
congenital ichthyosiform erythroderma a generalized hereditary dermatitis with scaling, occurring in a bullous form (epidermolytic hyperkeratosis) and a nonbullous form (lamellar ichthyosis).
erythroderma desquamati´vum Leiner's disease.
psoriatic erythroderma a generalized psoriasis vulgaris, showing the chemical characteristics of exfoliative dermatitis.

con·gen·i·tal ich·thy·o·si·form e·ryth·ro·der·ma

a genodermatosis characterized by diffuse chronic erythema and scale formation which may be separated into bullous and nonbullous forms.

con·gen·i·tal ich·thy·o·si·form e·ryth·ro·der·ma

(kŏn-jen'i-tăl ik'thē-ō'si-fōrm ĕ-rith'rō-dĕr'mă)
A genodermatosis characterized by diffuse chronic erythema and scale formation, which occurs in bullous and nonbullous forms.
Synonym(s): ichthyosiform erythroderma.


Antonino, Italian physician, 1872-1950.
Carini syndrome - skin disease of the newborn characterized by fissures and shedding of membrane encasing the body to reveal red skin underneath. Synonym(s): alligator baby; congenital ichthyosiform erythroderma; lamellar desquamation of the newborn; lamellar ichthyosis


Earl Weldon, U.S. dermatologist, 1893–.
Comèl-Netherton syndrome - Synonym(s): Netherton syndrome
Netherton syndrome - brittle hair; atopic manifestations. Synonym(s): Comèl-Netherton syndrome; congenital ichthyosiform erythroderma
References in periodicals archive ?
Clinical features are congenital ichthyosiform erythroderma, atopic diathesis and specific bamboo hair appearance (3).
Several VX cases have been reported in association with underlying immune-mediated or other conditions, such as pemphigus vulgaris [8], lupus erythematosus [9], dystrophic epidermolysis bullosa [10], graft-versus-host disease (GVHD) [11], epithelial dysplasia and squamous cell carcinoma [1, 12, 13], or congenital hemidysplasia with ichthyosiform erythroderma and limb defects (CHILD) syndrome [14].
Congenital ichthyosiform erythroderma is often present at birth or shortly thereafter and manifests as generalized erythroderma and desquamation.
The 3 major types of autosomal recessive congenital ichthyosis are Harlequin ichthyosis, lamellar ichthyosis, and congenital ichthyosiform erythroderma. Harlequin ichthyosis is the least common and most severe form.
In 1902, Brocq5described ichthyosis hystrix as an atypical form of congenital bullous ichthyosiform erythroderma. It was later described by various names by other authors like nevus verrucous, systematized epidermal nevus and epidermolytic hyperkeratosis.Gianotti,6 in 1980, classified it as a localized form of congenital epidermolytic hyperkeratosis.
It may result from a pre-existing dermatoses or underlying lymphoma, drug eruptions, hereditary causes eg: ichthyosiform erythroderma. Hence it is mandatory to establish the cause in order to facilitate its management.
The condition has to be differentiated from non-bullous ichthyosiform erythroderma, X-linked ichthyosis, Chanarin-Dorfman syndrome and Trichothiodystrophy.2,4 In patients of non-bullous ichthyosiform erythroderma, the scales are thin, superficial, white or grey and semi adherent in nature, which were not seen in our patient.2
Observation of lipid vacuoles in neutrophils (Jordan's anomaly) in peripheral blood smears in patients with ichthyosiform erythroderma is diagnostic.
CHILD Sendromu (Congenital Hemidysplasia Ichthyosiform Erythroderma Limb Defects)
She was diagnosed as having congenital ichthyosiform erythroderma, a condition that affects just one in 300,000 children.
Hypnosis has been found useful to treat a number of skin disorders including acne excoriee, alopecia areata, atopic dermatitis, congenital ichthyosiform erythroderma, dyshidrotic dermatitis, erythromelalgia, furuncles, glossodynia, herpes simplex, hyperhidrosis, ichthyosis vulgaris, lichen planus, neurodermatitis, nummular dermatitis, postherpetic neuralgia, pruritus, psoriasis, rosacea, trichotillomania, urticaria, verruca vulgaris, and vitiligo.
Congenital ichthyosiform erythroderma treated by hypnosis.