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Biomedical subjects

R Happle

Publications and source records attributed to R Happle.

At least 109 records · Page 6Linked to original sources
PubMed1998

The essence of alternative medicine. A dermatologist's view from Germany.

In Germany, alternative medicine is presently very popular and is supported by the federal government. When deliberating on the essence of alternative medicine we should simultaneously reflect on the intellectual and moral basis of regular medicine. To provide an epistemological demarcation of the 2 fields, the following 12 theses are advanced: (1) alternative and regular medicine are speaking different languages; (2) alternative medicine is not unconventional medicine; (3) the paradigm of regular medicine is rational thinking; (4) the paradigm of alternative medicine is irrational thinking; (5) the present popularity of alternative medicine can be explained by romanticism; (6) some concepts of alternative medicine are falsifiable and others are not; (7) alternative medicine and evidence-based medicine are mutually exclusive; (8) the placebo effect is an important factor in regular medicine and the exclusive therapeutic principle of alternative medicine; (9) regular and alternative medicine have different aims: coming of age vs faithfulness; (10) alternative medicine is not always safe; (11) alternative medicine is not economic; and (12) alternative medicine will always exist. The fact that alternative methods are presently an integral part of medicine as taught at German universities, as well as of the physician's fee schedule, represents a collective aberration of mind that hopefully will last for only a short time.

Complementary Therapies↗
PubMed1998

Phacomatosis pigmentokeratotica: report of new cases and further delineation of the syndrome.

BACKGROUND: The epidermal nevus syndromes include different diseases that have the common feature of mosaicism. One of these has been recently identified and named phacomatosis pigmentokeratotica, in analogy to phacomatosis pigmentovascularis. It is characterized by an organoid nevus with sebaceous differentiation, a speckled-lentiginous nevus, and other associated anomalies. It has been hypothesized that this syndrome is caused by a particular genetic mechanism known as the twin-spot phenomenon. OBSERVATIONS: We describe 3 patients manifesting an association of organoid nevus showing sebaceous differentiation and speckled-lentiginous nevus with associated anomalies and update the neurologic findings of a previously described patient. Hemiatrophy seems to be a common finding in all cases; hyperpathia, dysesthesia, and hyperhidrosis, as well as other neurologic defects, may be present. CONCLUSIONS: The findings in these patients allowed us to better delineate this syndrome. Further studies are needed to elucidate the underlying genetic defect. At present, however, the hypothesis that best explains this phenotype is twin spotting. Clinical recognition of this syndrome can contribute to the classification of the epidermal nevus syndromes and give insight into unusual genetic mechanisms occurring in humans.

Adolescent↗
PubMed1998

[Neonatal lupus erythematosus and maternal HELLP syndrome: is there a pathogenetic link?].

A newborn boy developed annular erythematous lesions on his entire body. Histopathological examination showed typical features of lupus erythematosus. His mother was positive for anti-Ro/SSa and anti-La/SSb antibodies. Neonatal lupus erythematosus was diagnosed. During pregnancy the mother had suffered from HELLP syndrome. The reported case points out the necessity to differentiate HELLP syndrome from first manifestation of lupus erythematosus during pregnancy. A direct causal relationship between neonatal lupus erythematosus and HELLP syndrome of the mother seems to be unlikely.

Adult↗
PubMed1998

5 alpha-reductase activity in the human hair follicle concentrates in the dermal papilla.

Hair growth depends on a close interaction of different cell populations of the hair follicle. In certain regions of the body, androgens interfere with this highly regulated cooperation in a yet poorly understood manner. The response of hair follicles to androgens can be categorized as androgen-dependent, e.g. in the beard, androgen-sensitive, e.g. in the frontal scalp of affected individuals, or androgen-independent, e.g. in the occipital scalp. At the target cell level, the balance between 5 alpha-reductase, 17 beta-hydroxysteroid-dehydrogenase (17 beta-HSD) and 3 alpha-hydroxysteroid dehydrogenase (3 alpha-HSD) yields metabolites with different androgenic potential. We examined this target cell-specific androgen metabolism in microdissected intact sub-units of dermal papillae, connective tissue sheaths (CTS) and root sheaths. In dermal papillae, 5 alpha-reductase predominated with an accumulation of the strong androgen 5 alpha-dihydrotestosterone. The specific activity of 5 alpha-reductase in the papillae exceeded those in the other hair follicle compartments by a factor of at least 14 in the scalp (5.4, 0.4 and 0.1 pmol/h per mm3 in the papilla, CTS and root respectively and at least 80 in the beard (16.0, 0.2 and 0.4 pmol/h per mm3 in the papilla, CTS and root respectively). The root sheath keratinocytes expressed low 5 alpha-reductase levels, but high 17 beta-HSD levels, with androstenedione as the major metabolite. The CTS expressed both 5 alpha-reductase and 17 beta-HSD, resulting in androstenedione, 5 alpha-androsterone and 5 alpha-androstanedione. In the CTS and the root sheath, only minor amounts of 5 alpha-DHT were found. In beard papillae, the 5 alpha-reductase activity was three times that in the occipital scalp papillae. These results indicate that the androgen response of hair follicles depends on a differentiated intrafollicular androgen metabolism and that the dermal papilla might be a primary target in this process.

Androgens↗
PubMed1998

Desquamative gingivitis and balanitis--linear IgA disease or cicatricial pemphigoid?

A 38-year-old man presented with gingival inflammation together with erosions of the penis. Direct immunofluorescence demonstrated linear deposits of IgA at the basement membrane zone; indirect immunofluorescence and immunoblotting were negative. Linear IgA disease (LAD) was therefore suspected and treatment with dapsone initiated but this was changed to sulfamethoxy-pyridazine and systemic corticosteroids because of methaemoglobinaemia. During 1-year follow-up the lesions continued to wax and wane although they were never as extensive as before. Eighteen months after disease onset there was scarring of the penis together with suspected fibrosis of the inflamed gingival region. In addition the patient was HLA DQ7(3) positive, a haplotype thought to be increased in patients with cicatricial pemphigoid (CP); LAD with scarring or CP with solely linear IgA deposits are possible diagnoses of his condition.

Adult↗
PubMed1998

Conversion of psoriasis patients from the conventional formulation of cyclosporin A to a new microemulsion formulation: a randomized, open, multicentre assessment of safety and tolerability.

OBJECTIVE: To assess the safety, tolerability and efficacy of a new cyclosporin A (CyA) microemulsion formulation, Sandimmun Neoral (Neoral), in patients with severe psoriasis that was stable on CyA administered as Sandimmun (SIM). METHODS: In this 24-week, open, randomized, prospective, multicentre trial, 28 patients continued on the same dosage of SIM, while 30 converted to Neoral at 2.5 mg/kg/day or a dosage equivalent to their pre-conversion SIM dosage. During the study, dosages could be adjusted to maintain efficacy, because of adverse events or after disease stabilization. The maximum permitted dosage for either formulation was 5.0 mg/kg/day. Primary efficacy criteria were change in Psoriasis Area and Severity Index (PASI) from baseline and time to relapse. RESULTS: The dosage was increased to maintain efficacy in 22 patients (Neoral 13; SIM 9) and 20 dose reductions for safety were required (Neoral 14, SIM 6). In both groups, PASI scores remained stable throughout and relapses were primarily a result of dosage reduction after disease stabilization. No significant difference was found between groups in the proportion of patients remaining relapse-free. Adverse events were recorded in 20 patients receiving Neoral and 14 receiving SIM. Most drug-related events were of mild or moderate severity and reflected the known CyA side-effect profile. Dose titration guidelines ensured that mean blood pressure and serum creatinine concentrations remained stable in both groups. CONCLUSIONS: If the guidelines for CyA use are followed and the Neoral dosage does not exceed 5 mg/kg/day, conversion of stable patients with severe psoriasis from SIM to Neoral should present no clinically relevant safety or tolerability problems and efficacy of treatment is maintained.

Adolescent↗
PubMed1998

Nevus psiloliparus: a distinct fatty tissue nevus.

BACKGROUND: Encephalocraniocutaneous lipomatosis is usually associated with a peculiar type of fatty tissue nevus which represents a smoothly surfaced and hairless lesion involving the scalp. This disorder has so far not been recognized as a cutaneous entity. OBJECTIVE: The purpose of this article is to describe the characteristic features of this nevus and to give it a name. METHOD: From the study of two cases and from a review of the literature we delineate the clinical and histopathological criteria of this disorder for which we propose the term 'nevus psiloliparus'. This name is derived from the Greek words psilós = hairless and liparós = fatty, and describes the two most characteristic features of the disorder. RESULTS: A comprehensive comparison shows that nevus psiloliparus can be distinguished from other types of fatty tissue nevi by clinical criteria such as localization on the scalp, a flat smooth surface and absence of hair follicles, by the histopathological feature of isolated arrector pili muscles and by the presence of associated extracutaneous features in the form of encephalocraniocutaneous lipomatosis. In particular, nevus psiloliparus can be separated from the Hoffmann-Zurhelle nevus that has so far never been observed in cases of encephalocraniocutaneous lipomatosis. CONCLUSION: The presently available data suggest that nevus psiloliparus represents a distinct cutaneous entity. Future clinical and genetic research should show whether this concept holds true.

Adipose Tissue↗
PubMed1997

Becker nevus syndrome.

The new term Becker nevus syndrome is proposed for a phenotype characterized by the presence of a particular type of organoid epithelial nevus showing hyperpigmentation, increased hairiness and hamartomatous augmentation of smooth muscle fibers, and other developmental defects such as ipsilateral hypoplasia of breast and skeletal anomalies including scoliosis, spina bifida occulta, or ipsilateral hypoplasia of a limb. The present review includes 23 cases that can be categorized under this designation. The Becker nevus syndrome usually occurs sporadically. The associated anomalies tend to show a definite regional correspondence, suggesting a common origin from an early postzygotic mutation.

Adolescent↗
PubMed1997

IFN-gamma-induced HLA-DR but not ICAM-1 expression on cultured dermal papilla cells is downregulated by TNF-alpha.

The immune response present in untreated alopecia areata (AA) is characterized by overexpression of ICAM-1 and MHC molecules on dermal papilla cells of affected hair follicles and by a distinct cytokine pattern. After successful treatment with the potent contact allergen diphenylcyclopropenone (DCP), adhesion molecules are downregulated and a reversed pattern of cytokines is expressed. To determine which cytokines may be involved in this process we studied the expression and modulation of ICAM-1 and MHC class I and II molecules on cultured dermal papilla cells. Scalp biopsies were obtained from healthy donors and dermal papillae were isolated. The cells were treated with various cytokines and prostanoids. The surface molecules were labeled with FITC-conjugated antibodies, and the expression levels were quantified by FACScan analysis. Incubation with IFN-gamma led to a time-dependent upregulation of the surface molecules studied. IL-1 beta and TNF-alpha synergistically increased the expression of ICAM-1, but they failed to induce MHC molecules. However, both cytokines significantly reduced the IFN-gamma-induced HLA-DR expression. Pretreatment of cells with the cyclooxygenase inhibitor diclofenac, prostanoids, IL-10 or TGF-beta 1 did not alter the constitutive or IFN-gamma-elicited expression of surface molecules. A neutralizing anti-IL-1 beta-antibody did not affect any cytokine-induced changes. We conclude that with regard to surface molecules we can partly initiate in vitro the situation of AA in vivo. Moreover, our results suggest that TNF-alpha, which is markedly increased under DCP treatment, might be an effector of the therapeutic response in AA.

Adult↗
PubMed1997

Systematized inflammatory epidermal nevus with symmetrical involvement: an unusual case of CHILD syndrome?

The CHILD syndrome (congenital hemidysplasia with ichthyosiform nevus and limb defects) is usually characterized by lateralization of all associated anomalies. It has been assumed that the event of X-inactivation coincides and interferes with a clone of organizer cells controlling a large developmental field. A 16-year-old girl with bilateral manifestations of CHILD syndrome is described. The inflammatory skin lesions affected the body folds (ptychotropism) in a symmetrical distribution, although only the right side of the neck was involved. In addition, absence of several facial muscles, vertebral defects, and shortening of the leg on the right side were noted, and a ventricular septum defect was present. This unusual case may be explained by the assumption that X-inactivation did not coincide with the origin of inducer cell clones controlling large morphogenetic fields on either side of the body.

Abnormalities, Multiple↗
PubMed1997

Interleukin-1beta-induced inhibition of hair growth in vitro is mediated by cyclic AMP.

Interleukin (IL)-1 has been shown to be a potent inhibitor of hair growth in vitro. We hypothesized that this cytokine might be a decisive factor causing hair loss during the lymphocytic attack in alopecia areata. Neither the intracellular pathways involved in hair growth inhibition mediated by IL-1beta nor the signal transduction processes within hair follicles in general are known. We therefore investigated the intracellular signals involved in human hair growth in vitro. Hair follicles were isolated from scalp biopsies by microdissection, and hair growth was measured daily by image analysis. We assessed intracellular signal transducing elements using specific inhibitors or activators either alone or in combination with IL-1beta. The calcium ionophore A 23187 induced a rapid and complete arrest of hair growth, and phorbol-12-myristate-13-acetate (PMA), genistein, or IL-1beta decreased hair growth by approximately 60%-80%. IL-1beta-elicited hair growth arrest was not antagonized by calphostin C, a specific inhibitor of protein kinase C. In contrast, coincubation of IL-1beta with pertussis toxin or H 1004 neutralized the effect of IL-1beta, and dibutyryl-cAMP and cholera toxin, an activator of adenylate cyclase, inhibited hair growth. These data suggest that cAMP acts as a second messenger for IL-1beta-induced inhibition of hair growth. Moreover, our data indicate that in vitro hair growth is dependent on intracellular Ca2+ levels and activation of tyrosine kinase as well as protein kinase C. We were unable to detect a signal transducing element responsible for enhanced hair growth in vitro.

Calcimycin↗
PubMed1997

"Cutis tricolor": congenital hyper- and hypopigmented macules associated with a sporadic multisystem birth defect: an unusual example of twin spotting?

An uncommon coexistence of circumscribed hyperpigmentation and hypopigmentation, in close proximity to each other, is described in a 17 years old patient with various other cogenital defects, such as dysmorphic facial appearance, severe kyphoscoliosis, delayed motor development, epileptic seizures, and mental retardation. We suggest the combination of hyper- and hypopigmented cutaneous lesions is an example of allelic twin spotting. Because the skin of this patient showed three different degrees of pigmentation the term "cutis tricolor" is proposed.

Abnormalities, Multiple↗