Zur Klassifikation der Ichthyosis-Gruppe

Abstract

Subdivision of ichthyosis congenita into 3 types is no longer necessary because the concept of congenital ichthyosiform erythroderma (non bullous) accounts for all these types, including the possibility of associated symptoms. It is necessary to separate the "dry" types which are almost without exception recessive, and the bullous types of congenital erythroderma whose heredity is dominant. The most important of these phenocopies is peridermia (collodion baby) which at first can not be distinguished from congenital ichthyosiform erythroderma; after treatment by corticosteroids the fatal form of ichthyosis congenita is in fact seen only very rarely. The heredity of ichthyosis vulgaris (simplex, nitida, serpentina, hystrix, etc is simply or irregularly dominant but it can also be regularly recessive or sex linked (transmitted by the male). The types accompanied by severe symptoms are always recessive, even when severe skin changes (porcupine man) of a dominant character can appear. It is not certain that ichthyosis linearis circumflexa belongs to the group of ichthyosis vulgaris, while the so-called syndrome of Refsum should be regarded as a recessive variety of ichthyosis vulgaris sometimes accompanying severe disorders of the nervous system.