Biomaterial Database

Treatment of Darier's disease with an oral aromatic retinoid (Ro 10-9359): a clinical and light and electron microscopic study. 1981

J Lauharanta, and K M Niemi, and A Lassus

An aromatic retinoid (Ro 10-9359) cleared 6 of 8 patients with Darier's disease in 4-6 weeks. After discontinuing the treatment, remission was maintained for up to 5 months. In 2 severe cases continuous treatment proved necessary in order to maintain the improved state. Acantholysis, various types of dyskeratosis and acanthosis with downward proliferation of rete ridges were seen by light microscopy before treatment. Electron microscopy revealed a decreased number of desmosomes and other cellular attachments and conspicuous microvillous changes of cell surface in separated basal cells. Keratinization in epidermal cells was premature and characterized by abnormal aggregations of keratinization products. Under the influence of retinoid there seemed to appear a new generation of basal cells better able to form intercellular attachments, after which the progress of keratinization in the entire epidermis shifted towards normal, and acanthosis decreased.

UI	MeSH Term	Description	Entries
D007644	Darier Disease	An autosomal dominantly inherited skin disorder characterized by warty malodorous papules that coalesce into plaques. It is caused by mutations in the ATP2A2 gene encoding SERCA2 protein, one of the SARCOPLASMIC RETICULUM CALCIUM-TRANSPORTING ATPASES. The condition is similar, clinically and histologically, to BENIGN FAMILIAL PEMPHIGUS, another autosomal dominant skin disorder. Both diseases have defective calcium pumps (CALCIUM-TRANSPORTING ATPASES) and unstable desmosomal adhesion junctions (DESMOSOMES) between KERATINOCYTES.	Acrokeratosis Verruciformis of Hopf,Darier-White Disease,Keratosis Follicularis,Acantholytic Dyskeratotic Epidermal Nevi,Acantholytic Dyskeratotic Epidermal Nevus,Acrokeratosis Verruciformis,Darier's Disease,Hopf Disease,Darier White Disease,Darier-White Diseases,Dariers Disease,Disease, Darier,Disease, Darier's,Disease, Darier-White,Disease, Hopf,Diseases, Darier-White,Diseases, Hopf,Hopf Acrokeratosis Verruciformis,Hopf Diseases,Verruciformis, Acrokeratosis
D008297	Male		Males
D008854	Microscopy, Electron	Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen.	Electron Microscopy
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D002986	Clinical Trials as Topic	Works about pre-planned studies of the safety, efficacy, or optimum dosage schedule (if appropriate) of one or more diagnostic, therapeutic, or prophylactic drugs, devices, or techniques selected according to predetermined criteria of eligibility and observed for predefined evidence of favorable and unfavorable effects. This concept includes clinical trials conducted both in the U.S. and in other countries.	Clinical Trial as Topic
D005050	Etretinate	An oral retinoid used in the treatment of keratotic genodermatosis, lichen planus, and psoriasis. Beneficial effects have also been claimed in the prophylaxis of epithelial neoplasia. The compound may be teratogenic.	B10-9359,Ethyl Etrinoate,Ro 10-9359,Ro-10-9359,Tigason,Tigazon,B10 9359,B109359,Etrinoate, Ethyl,Ro 10 9359,Ro 109359,Ro109359
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000284	Administration, Oral	The giving of drugs, chemicals, or other substances by mouth.	Drug Administration, Oral,Administration, Oral Drug,Oral Administration,Oral Drug Administration,Administrations, Oral,Administrations, Oral Drug,Drug Administrations, Oral,Oral Administrations,Oral Drug Administrations
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults