Erythroderma, ichthyosiform

Ichthyosis vulgaris X-Linked ichthyosis Lamellar ichthyosis (nonbullous ichthyosiform erythroderma) Epidermolytic hyperkeratosis (bullous ichthyosis)... 

Characteristic clinical Congenital or neonatal ichthyosiform erythroderma + -... 

Happle R, Koch H, Lenz W. The CHILD syndrome. Congenital hemidysplasia with ichthyosiform erythroderma and limb defects. Eur J Pediatr 1980 134(1) 27. 

One case of centrilobular necrosis of the liver was reported by Thune and Moik (1980) in a 54-year-old woman with nonbullous congenital ichthyosiform erythroderma treated for 3 months with 50-75 mg/day etretinate. Besides... 

In comparison with psoriasis the ichthyoses have commanded relatively little attention from researchers as far as the population dynamics of the epidermis are concerned. Frost and Fisher and Wells found no serious disturbance of the rate of epidermal cell production in autosomal dominant ichthyosis. However, a high rate of epidermopoiesis was noted in non-bullous ichthyosiform erythroderma by Frost. ... 

Patients with autosomal dominant ichthyosis (ADI) fulfilled the usual clinical criteria for this disorder — (five men, four women, mean age = 40.5 years). The two patients with non-bullous ichthyosiform erythroderma are briefiy... 

Table 6.1 Clinical details of patients studied with non-buUous ichthyosiform erythroderma and lamellar ichthyosis...

F ichthyosiform erythroderma Fine prolific scaling and generalized erythema. Ectropion... 

M ichthyosiform erythroderma Generally similar to patient 3 but linear hyperkeratotic areas in antecubital and popliteal fossae and marked plantar hyperkeratosis... 

Structural abnormalities are demonstrable in some dominant types, in keratohyalin (defective in autosomal dominant ichthyosis vulgaris), and tono-filaments (impaired or disturbed in their arrangement in hystrix-like ichthyoses clump formation in bullous ichthyosiform erythroderma or epidermolytic hyperkeratosis shell formation associated with high numbers of binucleate cells in ichthyosis hystrix type Curth-Macklin impairment in ichthyosis hystrix gravior type Rheydt). Keratohyalin and tonofilaments are both structural proteins of keratinizing tissues. 

Hirone, T. (1969). Electron microscopic studies of ichthyosis and congenital ichthyosiform erythroderma. J. Electr. Microsc., 18,63... 

Ichthyosiform erythroderma with deafness Ichthyosiform erythroderma with unilateral limb defects Ichthyosis congenita with cataract Ichthyosis with mental retardation and hypogonadism (Rud s syndrome)... 

Tay s syndrome is an autosomal recessive disorder characterized by congenital ichthyosiform erythroderma, pili torti and trichorrhexis nodosa, mental and growth retardation. After some months the erythroderma subsides and the hyperkeratotic scaling is confined to the face, trunk and extensor aspects of the limbs. The palms and soles are thickened. The child shows a progeria-like appearance. 

Figure 15.1 Ichthyosiform erythroderma and immunodeficiency with short-limbed dwarfism in a 16-month-old girl...

Psoriasiform erythroderma is really a congenital psoriasis, but the gross and histologic features resemble a severe ichthyosiform erythroderma, marked by conspicuous loss of large, micaceous scales for several months. Some normal skin areas are often distinctive. Only the appearance of typical psoriatic patches or pustular psoriasis during childhood permits this diagnosis to be confirmed, as we have also personally observed in our patients. 

Zeligman, I. and Pomeranz, J. (1965). Variations of congenital ichthyosiform erythroderma. Arch. Dermatol, 91,120... 

Tay, C. H. (1971). Ichthyosiform erythroderma, hair shaft abnormalities and mental and growth retardation. Arch. Dermatol, 104,4... 

Ichthyosiform Erythroderma. 10 Years Treatment with Retinoic Acid... 

Following the publications of Stiittgen and Beer on the use of vitamin A acid in the treatment of ichthyosiform erythroderma, Thomson and Milne reported the treatment of a seven-year-old girl with ichthyosiform erythroderma (epidermolytic hyperkeratosis) (Figure 21.1) by topical and systemic retinoic acid. 

Successful treatment of congenital ichthyosiform erythroderma, pityriasis rubra pilaris, and Darier s disease have been documented with oral all-trans retinoic acid in daily doses varying from 10 to 60 mg for several weeks to monthsOrfanos et al. used oral 13-cis retinoic acid in the treatment of psoriasis with equivocal results More recently, better results were obtained in psoriasis using a trimethylphenyl derivative of retinoic acid (RO-10/9359, Hoffman-La Roche, Basle), up to 100 mg/day, either alone or in combination with topical dithranoP". Since this derivative appears to be more effective than 13-cis retinoic acid in the treatment of psoriasis, a study comparing these two synthetic retinoids in the treatment of other disorders of keratinization should be of value. 

Eriksen, L. and Cormane, R. H. (1975). Oral retinoic acid as therapy for congenital ichthyosiform erythroderma. Br. J. Dermatol., 92,343... 

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