We report on a 66-year old patient who developed slowly progressive, multiple, firm, white shiny papules periocularly and nasolabially as well as to a minor degree on the trunk and extremities over the previous 30 years. Histology repeatedly indicated perifollicular fibromas, trichodiscomas and one fibrofolliculoma. Similar lesions were reported in two sisters of the patient. Thorough examination discovered a large villous adenoma of the colon and multiple scars of chorioretinitis with reduction of the visual field of unknown cause. The diagnosis of a Hornstein-Birt-HoggDube-Syndrome was made. The diagnosis of a cutaneo-oculo-intestinal syndrome will be discussed.

Erythema migrans is a classical skin lesion of borreliosis. In children almost 90% develop an erythema migrans in a few days to a month following the bite of an infected tick. Solitary erythema migrans is characteristic for an early infection while multiple annular lesions are reported to be a sign for haematological spreading of the vector.

M. Hailey-Hailey (Pemphigus chronicus benignus familiaris) is a rare, autosomal-dominant inherited genodermatosis. For treatment topical application of steroids or surgical excision/dermabrasio have been used. We report here the successful treatment with calcipotriol.

A flood of new knowledge and discoveries in the basic science of keratins and keratinization has appeared in the past several years. This review summarizes this recent information with a focus on the epithelial keratin polypeptides, keratin intermediate filaments, keratohyaline granule proteins, cell envelope formation and cell envelope proteins, "soft" keratinization, true disorders of keratinization (i.e., epidermolysis bullosa simplex and epidermolytic hyperkeratosis), and disease and drug effects on keratinization.