Der von Laurell und Eriksson 1963 (8) beschriebene a1-Antitrypsin-Defekt hat wie alle bisher bekannten Defektpathoproteinämien eine besondere klinische Bedeutung. Er wird wahrscheinlich rezessiv-autosomal vererbt (3, 6,9). 5070°/o der Defekt-

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Corneal lesions in ichthyosis, here combined with alopecia, are rarely described in the literature. The present observation relates to two sisters, whose grandparents were siblings. The mode of inheritance is autosomal recessive, as is usual in ichthyosis congenita but is also observed in ichthyosis vulgaris. The clinical picture and the histological findings correspond to ichthyosis vulgaris.

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