By I. Anton-Lamprecht, M.-L. Arnold (auth.), Prof.Dr. Rudolf Happle, Prof.Dr. Edouard Grosshans (eds.)
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Additional info for Pediatric Dermatology: Advances in Diagnosis and Treatment
An important exception to this rule can be the Netherton syndrome when it presents as congenital ichthyosiform erythroderma. According to the literature this is a rare event and only seven such cases have been described. In our own experience with six Netherton syndrome patients, however, we observed only one case with ichthyosis linearis circumflexa and five cases with congenital ichthyosiform erythroderma. The exfoliative scaling found in these patients is somewhat different from erythrodermic lamellar ichthyosis.
In each case a family pedigree was constructed and we triedto examine close relatives (parents, brothers, and sisters). Traupe 32 Table 2. Clinical features of autosomal recessive lamellar ichthyoses: distinction of ELI from NELl Age (yrs) Sex Erythroderma Size of hyperkeratoses Light scaling + + + +++ + Dark scaling Flexural involvement Alopecia ichthyotica + + ++ +++ + Erythrodermic lamellar ichthyosis Case 1 10 F Case 2 54 F Case 3 18 F Case 4 20 41 F Case 5 +++ +++ + F +a +a Case 6 Y4 F ++ Case 7 8 M ++ Variable (+-+++) + + Variable ++ (+ - ++) Variable + (+ - ++) Variable ++ (+-++) + + +++ + ++ Nonerythrodermic lamellar ichthyosis Case 1 20 F Case 2 44 F Case 3 8 M +++ +++ ++ +++ +++ ++ +++ +++ ++ ++ +++ Graduation: -, absent; +, present; ++, marked; +++, very marked Erythroderma present only at birth and in the first years of life.
In their attempt more clearly to define the potential skeletal toxicity of oral isotretinoin, Ellis et al.  performed a prospective analysis of radiographic bone changes under treatment with this compound. 9mg/kg per day isotretinoin over a period of 9-12 months, no acceleration of epiphyseal maturation was detected; the bone age was consistent with the chronological age. Nevertheless, after 6-month-therapy multiple, small hyperostoses were found in five of these children and became more prominent after a further 6-month administration of the drug, being mostly located at the vertebral bodies.