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Biomedical subjects

P Freyschmidt-Paul

Publications and source records attributed to P Freyschmidt-Paul.

26 records · Page 2Linked to original sources
PubMedDate not supplied

Hermansky-Pudlak syndrome.

A 55-year-old man had oculocutaneous albinism and a history of frequent bruising following minimal trauma. The simultaneous occurrence of these features was first described by Hermansky and Pudlak in 1959. The Hermansky-Pudlak syndrome follows an autosomal recessive trait and is most frequently found in Puerto Rico and in the Swiss alps. It consists of the triad phenotype of hypopigmentation, prolonged bleeding time due to platelet storage pool deficiency and accumulation of ceroid pigment in lysosomal organelles. Other serious features are pulmonary fibrosis and granulomatous colitis. The disorder is caused by mutations in the HPS1 gene on chromosome 10q23. The HPS1 gene product is involved in the trafficking of melanosomes, platelet dense bodies, and lysosomes.

Diagnosis, Differential↗
PubMedDate not supplied

Treatment of alopecia areata in C3H/HeJ mice with the topical immunosuppressant FK506 (Tacrolimus).

Alopecia areata-like hair loss has been observed in C3H/HeJ mice and can be defined as a tissue-restricted T cell mediated disease of the hair follicle. Because FK506 has been described as suppressing T cell mediated autoimmune diseases, we addressed the question whether topical treatment of C3H/HeJ mice with FK506 has a beneficial effect on alopecia areata (AA). For this purpose six C3H/HeJ mice with AA were treated topically with 0.1% FK506 ointment, four mice received the vehicle only. Four of six FK506-treated mice showed complete hair regrowth, whereas 1/4 vehicle-treated mice regrew hair. Mice treated successfully with FK506 had reduced perifollicular infiltrates of CD4+ and CD8+ cells and a decreased expression of MHC class I and II and ICAM-1 on hair follicle epithelium, compared to control mice. We conclude that topical treatment with FK506 is able to induce hair regrowth in AA of C3H/HeJ mice, most likely by suppressing the T cell mediated immune response.

Administration, Cutaneous↗
PubMedDate not supplied

Treatment of alopecia areata in the DEBR model using Cyclosporin A lipid vesicles.

Alopecia areata (AA) is a chronic cutaneous disease with a suspected autoimmune origin. We evaluated the efficacy of 0.5% Cyclosporin A (CyA) in a topically applied liposomal formulation as a potential treatment for AA using the Dundee Experimental Bald Rat (DEBR) model. The vehicle consisted of liposomes (75% phosphatidylcholine, 5% lysophosphatidylcholine, 5% sterol, natural oils) of 10% wt. in ethanol with and without 2% wt. terpenes (d-limonene: citral: cineole, 10:45:45) as a penetration enhancer (PE). Fifteen DEBR were allocated to 3 groups of 5. Groups I, II and III received CyA vesicles with PE, CyA vesicles without PE, and CyA in ethanol respectively. All rats were treated twice a day for 6 weeks within a 4 cm2 area on one bald flank with CyA while the contralateral flank received an equivalent control formulation. Rats in group I exhibited visible hair regrowth on the drug treated site after one week of drug application. Group II rats had visible hair regrowth by the end of the second week. The hair growth was progressive and reached a maximum density at the site of application after six weeks in both groups. Histological examination revealed a reduced inflammatory infiltrate and improved hair follicle morphology within the drug treated area as compared to the contralateral vehicle treated skin. Group III rats showed neither visible signs of hair growth nor reduction of hair follicle inflammation. The results of this proof of concept preliminary study suggest that CyA vesicle formulations with and without PE have promising potential as a topical treatment for AA in humans.

Administration, Cutaneous↗