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Biomedical subjects

R Happle

Publications and source records attributed to R Happle.

At least 397 records · Page 22Linked to original sources
PubMedDate not supplied

Influence of estrogens on the androgen metabolism in different subunits of human hair follicles.

The molecular pathways involved in estrogen-mediated induction of hair growth in androgenetic alopecia are unknown. Some authors found that estradiol (E) inhibited 5alpha-reductase (5alpha-R) activity and therefore we addressed the question whether 17alpha- or 17beta-E are able to modulate the activity of 5alpha-R, 3beta-hydroxysteroid dehydrogenase (3beta-HSD) or 17beta-hydroxysteroid dehydrogenase (17beta-HSD) in isolated compartments of human hair follicles. For this purpose, scalp biopsies from volunteers were taken and from each biopsy root sheaths, connective tissue sheaths and dermal papillae (DP) were dissected and incubated in the presence of 3H-testosterone (T) and, in addition, either 17alpha-E, 17beta-E, progesterone or finasteride for up to 48 hrs. Thereafter high-performance liquid chromatography analysis of culture supernatants was performed to detect T-metabolites. At the tested concentrations, finasteride was found to be a major inhibitor of dihydrotestosterone (DHT) formation. Even 1 nM finasteride inhibited DHT synthesis in DP by 86% and 1 nM progesterone by 75%. Estrogens were less able to inhibit the synthesis of DHT in DP (e.g. 100 nM 17alpha-E: 20%; 100 nM 17beta-E: 60%). Whether E directly inhibits 5alpha-R in DP's or whether the effect of estrogens might be explained by an increased conversion of T to the weaker androgens such as androstendione (via 17beta-HSD), androstenediol (via 3beta-HSD) or 17beta-E (via aromatase), thereby diminishing the amount of T available for the conversion to DHT, remains to be shown.

3-Hydroxysteroid Dehydrogenases↗
PubMedDate not supplied

Acrodermatitis acidemica secondary to malnutrition in glutaric aciduria type I.

We encountered a patient with glutaric aciduria type I (GA-I) associated with skin lesions resembling acrodermatitis enteropathica (AE). This child was being fed with a low-protein diet when the skin disorder developed. A deficiency in plasma levels of essential amino acids, particularly isoleucine, and zinc was confirmed. Supplementation of a high-caloric, protein-rich diet together with zinc, selenium and vitamins led to a prompt improvement of the skin lesions. We assume that in our patient the skin lesions were the result of malnutrition, rather than being primarily associated with the underlying metabolic disease. To our knowledge, no other report is so far available concerning GA-I complicated by skin eruptions.

Acrodermatitis↗
PubMedDate not supplied

Trichoteiromania.

We describe a 61-year-old patient who compulsively rubbed her hair and her scalp because of a psychiatric disorder. Permanent rubbing resulted in fracturing of the hair shafts, leaving 2 cm long hairs and areas with stubs of 1mm length, giving the impression of bald spots. The distal ends of affected hair shafts were split, giving the impression of white tips. Light microscopy of the hair shafts showed split, brush-like ends of otherwise normal hair. We suggest the name trichoteiromania, which means "compulsive rubbing of hair", as a new term to describe hair loss in such cases.

Alopecia↗
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

Evaluation of skin susceptibility to irritancy by routine patch testing with sodium lauryl sulfate.

Irritant patch testing with sodium lauryl sulfate (SLS) will become more and more a routine test determining skin susceptibility in men. Recently, it has been shown that for practical reasons, irritant SLS patch testing can take place on the back simultaneously with a routine allergic patch test to other contact allergens. However, SLS patch testing has mostly been performed on the forearm in studying experimental skin irritation so far. The aim of this study was to determine whether there is a relationship in skin response to aqueous SLS (0.125%; 0.25%; 0.5% and 1.0%) between the forearm and the back assessed by visual scoring and measurement of transepidermal water loss (TEWL). We found a pronounced reaction of the forearm compared to the back. TEWL values as well as visual scores correlated well with SLS concentration. There was also a high correlation in visual scoring between the forearm and the back. Based on test sensitivity and specificity we suggest a 48 hrs patch test for routine screening with 0.5% SLS on the forearm evaluated by TEWL measurement or visual scoring 24 hrs after patch removal. A mild erythema (scored as < or =1) is considered to be normal. If for practical reasons, the SLS patch is placed on the back simultaneously with the allergic patch test, 0.5% SLS may be sufficient, too. TEWL measurement so far provides a reliable method and will certainly be necessary for experimental studies on irritant skin reactions, particularly when different SLS concentrations are used. After a 48 hrs patch test with SLS 0.5% TEWL measurement should be performed at 72 hrs. A value of < or =31.6 g/m(2)hr seems to follow the normal distribution.

Back↗
PubMedDate not supplied

[Congenital homolateral epidermal hyperplasia and hypoplastic hemidysplasia (splitting of the Solomon's syndrome) (author's transl)].

The observation of a 16-year-old girl born with an ectromelia and an ipsilateral inflammatory verrucous epidermal nevus led us to a synthetic study of 17 similar cases already published since 1927: all these cases concern female patients and are characterized by a unilateral hypoplastic dysplasia, most often of limbs, and inflammatory epidermal hyperplastic lesions described as ichthyosiform, psoriasiform or verrucous, usually distributed on the same side on the skin overlying the dysplastic body areas. The skin lesions may be partly regressive after birth and their histological features are suggestive of inflammatory linear verrucous epidermal nevus (I. L. V. E. N.). These associations may be representative of a special form of Solomon's syndrome whose heterogeneity has be recently emphasized. We propose to subdivide it in 3 forms: the epidermal nevus syndrome (Solomon's syndrome)--the organoid nevus syndrome (Schimmelpenning's syndrome)--the I. L. V. E. N. syndrome, probably X-linked dominant inherited (lethal for hemizygous males), associated with ipsilateral hypoplastic body lesions and, however less frequently than in the epidermal nevus syndrome, with ocular and nervous abnormalities. The distribution of cutaneous lesions has some similarities with the pattern of skin symptoms of X-linked dominant traits such as chondrodysplasia punctata, focal dermal hypoplasia or incontinentia pigmenti. The most typical feature of this syndrome is the strong inflammatory aspect of the epidermal nevus erroneously described in previous cases as unilateral psoriasis or ichthyosiform erythroderma.

Adolescent↗
PubMedDate not supplied

Elements of the interleukin-1 signaling system show hair cycle-dependent gene expression in murine skin.

Stringently controlled changes in the local, cytokine-mediated signaling milieu of hair follicles (HF) have been implicated as major elements of hair cycle control and several lines of clinical and experimental evidence point towards interleukin-1 (IL-1) as an important inducer of hair loss. To address the question as to whether the steady state mRNA levels of the gene expression of the IL-1 family parallel distinct phases of the murine hair cycle, we have exploited the high degree of synchrony during depilation-induced HF cycling in mice, and have determined the mRNA levels of IL-1alpha, IL-1beta, IL-1-receptor antagonist, IL-1 receptor (R)-I and IL-1-R-II by semiquantitative RT-PCR. Our results indicate that the induced murine hair cycle is associated with profound fluctuations in the steady state mRNA levels of members of the IL-1 signaling system. Most interestingly, IL-1alpha and IL-1beta transcript levels increased dramatically with the onset of spontaneous catagen (around day 18) and peaked during telogen (day 25). These fluctuations in the IL-1alpha and IL-1beta transcript levels were paralleled by substantial expression changes of the corresponding signal transducing type I IL-1 receptor. Therefore, our findings are consistent with the concept that IL-1alpha, IL-1beta, IL-1-RI and IL-1-RII are involved in the control of catagen development.

Animals↗
PubMedDate not supplied

Vulvar amyloidosis mimicking giant condylomata acuminata in a patient with multiple myeloma.

We report a case of unusual cutaneous amyloidosis involving the vulva in a patient with multiple myeloma. Genital examination revealed a dense agglomeration of verrucous papules and pedunculated condyloma-like tumors. The correct diagnosis was established by immunohistochemical examinations that visualized large amounts of lambda light chains, whereas no reaction was detected for kappa light chains or human papilloma virus. In this way, the differential diagnosis of condylomata acuminata could be ruled out. Condyloma-like lesions have been described in patients suffering from multiple myeloma, but the present case is unusual because of the extensive involvement. Vulvar amyloidosis should be added to the list of possible presentations of myeloma-associated systemic amyloidoses.

Adult↗