[Genetics of Hailey-Hailey familial chronic benign pemphigus].

Starting from 12 index patients with familial benign chronic pemphigus from 11 kindreds, systematic kindred examinations were carried out with 182 relatives. 11 pedigrees were established. In this process 6 patients out of 3 kindreds were identified additionally.

Proliferation and differentiation of cultured epidermal cells from patients with X-linked ichthyosis and ichthyosis vulgaris.

The growth, differentiation, and regeneration of epidermal cultures from patients with X-linked and autosomal dominant ichthyosis and normal individuals were compared. Cell proliferation was studied by combining the technique of fluorescence-activated cell sorting with [3H]thymidine labelling and autoradiography. As in normal epidermal cultures, a marked heterogeneity in the labelling intensity of S-phase cells was observed in the ichthyotic cultures with totally unlabelled as well as very strongly labelled cells.

[Keratoses with granular degeneration and their relations to each other. I. Epidermolytic hyperkeratosis (erythrodermia congenitalis ichthyosiformis bullosa)].

Report on the epidermolytic hyperkeratosis (congenital bullous ichthyosiform erythroderma) by means of a family suffering from this hyperkeratosis. Diagnostic advices are given. Classification and nomenclature are discussed criticly.

[Keratoses with granular degeneration and their relations to each other. II. Heterophenotypic expression of hyperkeratosis (erythrodermia congenitalis ichthyosiformis bullosa), naevus hystricoides and Vörner keratosis palmoplantaris cum degeneratione granulosa].

By means of a patient suffering from epidermolytic hyperkeratosis, epidermolytic hereditary palmoplantar keratoderma VORNER and epidermolytic hystric nevus the relations of this keratinization disorders are shown. The different clinical symptoms in several patients are probable caused by pleiotropy.

Gene diagnosis in X-linked ichthyosis.

Three families segregating for X-linked ichthyosis (XLI) were analysed using the full-length STS cDNA probe and an anonymous polymorphic DNA sequence closely linked to the STS gene. In patients from two of the families, submicroscopic chromosomal deletions could be detected using both the STS and the GMGX9 (DXS237 locus) probes. Patients in the third family showed the same hybridization pattern as healthy males following molecular hybridization with either of the probes.

Arylsulphatase C activity in leukocytes of patients and carriers of X-linked ichthyosis.

In Triton X-100 solubilized leukocytes of 17 patients and 8 obligate carriers of X-linked recessive ichthyosis (XLI) the activity of arylsulphatase C (ASC) was determined and expressed as the ratio to beta-galactosidase activity. The ASC/beta-gal ratio of XLI patients is markedly decreased (range 0.07-0.

[Clinico-histologic spectrum of lichen sclerosus et atrophicus].

Report on several clinical and histological variants of lichen sclerosus et atrophicus by means of two cases.

[Potential secondary effects in pollen-desensitization with mixed pollen-depot-extracts in regards to foreign body granuloma (author's transl)].

Desensibilitation as a causal therapy in Pollinosis gets increasing importance in the last decennium. The rate of successful results is reported with 50-80 per cent. In general the rate of secondary effects by this therapy is very low.

[Cutaneous foreign body granulomas caused by aluminium-hydroxid after desensitization with h Mischpollen-Depotallergen (author's transl)].

Report on a 16 years old female patient suffering from pollinosis and asthma. After desensitization with Mischpollen-Depotallergen foreign body granulomas developed in the injection areas. By fluorescence histochemical examination aluminiumhydroxid was found as cause.

[Dermatitis as an expression of progesterone hypersensitivity (autoimmune disease)].

By means of a report on a 39-years-old woman with cyclic eruption of dermatosis in praemenstruum, was caused by progesterone-sensitivity. The problem is discussed of this autoimmune-disease. It is supposed that this autoimmune-progesterone-mechanism may be of importance in gynecology.