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R M Trüeb

Publications and source records attributed to R M Trüeb.

At least 19 recordsLinked to original sources
PubMed1999

Childhood bullous pemphigoid: report of a case with characterization of the targeted antigens.

The clinical and immunopathologic features of children with acquired subepidermal blistering disorders show considerable overlap, and their classification frequently requires characterization of the targeted antigens. A 8-month-old boy developed a generalized subepidermal blistering disorder with striking palmoplantar involvement. The patient's serum contained antibodies reacting against the epidermal side of 1 M sodium chloride separated normal human skin. Immunoblotting analysis demonstrated circulating IgG autoantibodies that reacted against a eukaryotic recombinant form of human bullous pemphigoid antigen 180 (BP180). In addition, the patient had circulating IgG autoantibodies that bound a protein of 120 kDa in skin basement membrane zone extracts, that might correspond to the linear IgA bullous disease (LABD) antigen. This study illustrates that a child with clinical and immunopathologic features considered characteristic of childhood bullous pemphigoid (BP) had circulating IgG antibodies that bound to an eukaryotic recombinant form of human BP180, and hence, fulfilled the diagnostic criteria of BP. Review of the literature disclosed only 10 cases of childhood BP, that were characterized on the basis of the targeted antigens. The concomitant presence of circulating IgG autoantibodies against BP180 and a 120 kDa protein may signify either coexistence of autoantibodies with distinct specificities or reflect antigenic cross-reactivity between BP180 and the 120/97 LABD antigen.

Autoantibodies↗
PubMed1999

[Androgenetic alopecia, hirsutism and hypertrichosis].

Having too much hair on the face or the body and not enough on the scalp respectively, is generally not a mirror of a life-threatening disease. However, the emotional impact of such cosmetic problems may be remarkable in the individual case. Currently rational treatment options are becoming increasingly available to correct such hair problems. This review highlights the new therapeutic achievements in the treatment of both androgenetic alopecia and hirsutism. Oral treatment of male patterned hair loss with finasteride in men is emphasized, and the use of antiandrogens in women is discussed. In addition, the mode of action and clinical results of topical minoxidil treatment find mention. The second part of the review deals with hirsutism and hypertrichosis. The diagnostic steps and investigations are briefly reviewed, and the advances in laser treatment of hirsutism and hypertrichosis are presented.

Adult↗
PubMed1998

[Temporary roentgen epilation after embolization of a cerebral arteriovenous malformation].

A patient with a large left-sided arteriovenous malformation underwent superselective angiography and therapeutic embolization. Sixteen days later he presented with an acute anagen-dystrophic hair loss localized to the occipital and right parietal regions corresponding to the irradiated scalp area. The diagnosis of an acute radiation injury to the hair follicle from prolonged fluoroscopic imaging during the interventional neuroradiologic procedure was made. This reversible side effect occurs typically after single short-term exposures of 300-400 cGy. Above single doses of 1200 cGy, the epilation is permanent. Patients have to be informed about the possibility of this reversible complication, which must be distinguished from alopecia areata, postoperative ischemic pressure alopecia and drug toxicity.

Adult↗
PubMed1998

[Lichen planopilaris simulating postmenopausal frontal fibrosing alopecia (Kossard)].

A 68-year old woman presented with a frontal fibrosing alopecia and lesions of the buccal mucous membranes typical for lichen planus. Postmenopausal frontal fibrosing alopecia (PFFA) has recently been described by Kossard as a distinct entity characterized by progressive recession of the frontotemporal and parietal hair margins leading to permanent alopecia in the form of a symmetrical band-like area of scanning in postmenopausal women. The histology (perifollicular lymphocytic infiltration and fibrosis, increase of apoptosis of hair follicle keratinocytes) is indistinguishable from that of lichen planopilaris. The localization and age- and sex-related characteristics of PFFA are not sufficient to delineate it as a discrete entity from lichen planopilaris. Our observation of oral lichen planus in a postmenopausal woman with frontal fibrosing alopecia points to the possibility that PFFA actually may represent a variant of lichen planopilaris with a predilection for the frontotemporal hairline. Other variants of lichen planopilaris include multifocal lichen planopilaris, disseminated lichen planopilaris (Lassueur-Graham-Little syndrome), lichenoid pseudopelade, and any combination of these ("mixed type"). An effective therapy of PFFA is not known. Also, treatment of lichen planopilaris forms in which fibrosis predominates over inflammation is similarly problematic, but the natural course of these diseases seems to be self-limited.

Aged↗
PubMed1998

[Mutation of the human hairless gene in atrichia universalis].

Recently a mutation of the human homologue of the murine hairless gene on chromosome 8p12 has been demonstrated in a Parkistani kindred with autosomal recessive atrichia universalis. Of the various forms of hereditary human hair loss, collectively and incorrectly termed alopecias, congenital universal alopecia, or rather atrichia, represents a rare disease which has nothing to do with the most frequent causes of hair loss, specifically androgenetic alopecia (AGA) and alopecia areata (AA). It may preferably be referred to as generalized atrichia, since alopecia universalis is also the term used for the most extreme form of disease progression in AA leading to complete loss of scalp and body hair. Interestingly the hairless mouse has formerly been proposed to be the animal model for atrichia with papular lesions (papular atrichia), which is likewise transmitted as an autosomal recessive trait. In contrast to these rare forms of hereditary atrichia with a Mendelian pattern of inheritance, both AGA and AA are considered to be polygenic, with hormonal and immunologic factors, respectively, modifying the phenotypic expression. Nevertheless, pinpointing a gene that encodes a transcription factor involved in hair growth may provide a more targeted approach to treat disorders of hair growth through transcriptional control of a variety of cytokines and growth factors implicated in the hair growth cycle.

Alopecia↗
PubMed1998

[Shampoos: composition and clinical applications].

Shampooing is the most common form of hair treatment. Shampoos have primarily been products aimed at cleansing the hair and scalp. The diversity of qualities demanded from a good shampoo by today's consumer go far beyond this general function. A cosmetic benefit is expected, and the shampoo formulation has to be tailored to all the possible variations associated with hair quality (dry, greasy, permed, bleached, dyed), age (baby shampoo), care habit (frequency of shampooing) and specific problems relating to the superficial condition of the scalp (dandruff, seborrhea). Selected ingredients of shampoos that have been popular with the consumer are currently under attack because of potential risks associated with their use (e.g. halogenated organic compounds, formaldehyde, musk fragrances, and crude coal tar). Our standard graduate training programs devote limited attention to the subject of shampoos, and much of the readily available information concerning shampoos is supplied by the industry. We should be increasingly aware that our patients look to us to supply independent information on what can be expected from a shampoo to enable them to make more informed choices at the consumer level.

Hair↗
PubMed1998

Novel homozygous and compound heterozygous COL17A1 mutations associated with junctional epidermolysis bullosa.

Junctional epidermolysis bullosa is a heritable, heterogeneous blistering skin disease with mechanically induced dermal-epidermal separation, mild skin atrophy, nail dystrophy, and alopecia. Four unrelated junctional epidermolysis bullosa families with different phenotypes were investigated here and four novel mutations associated with the disease were identified. Patients 1, 2, and 3 had generalized atrophic benign epidermolysis bullosa, with nonscarring blistering and varying degree of alopecia. Patient 4 had the localisata variant of junctional epidermolysis bullosa, with predominantly acral blistering and normal hair. All patients had mutations in the COL17A1 gene encoding collagen XVII, a hemidesmosomal transmembrane protein. Patients 1 and 2 carried homozygous deletions 520delAG and 2965delG, respectively. Patient 3 was compound heterozygous for a missense and a deletion mutation (G539E and 2666delTT), and patient 4 was heterozygous for a known mutation R1226X. The deletions led to premature termination codons and to drastically reduced collagen XVII mRNA and protein levels, consistent with the absence of the collagen in generalized atrophic benign epidermolysis bullosa skin. The missense mutation G539E allowed synthesis of immunoreactive collagen XVII in keratinocytes, but prevented its secretion, thus causing lack of the protein in the skin. The data suggest that different COL17A1 mutations and their combinations can result in a spectrum of biologic and clinical phenotypes of not only generalized atrophic benign epidermolysis bullosa, but also localized junctional epidermolysis bullosa.

Aged↗
PubMed1998

Histopathologic and ultrastructural study of lupus-like skin lesions in a patient with Bloom syndrome.

The histopathology of the lupus-like skin lesions associated with Bloom syndrome has been sporadically described. Skin biopsies from a 2-year-old boy with the classical features of Bloom syndrome, including lupus-like skin lesions, demonstrated marked interface changes with basal liquefaction degeneration, a moderate superficial mononuclear infiltrate, pigmentary incontinence, and capillary dilation in the papillary dermis. Immunophenotyping of the dermal infiltrate revealed predominance of T-cells. Basement membrane thickening on periodic acid-Schiff examination was not seen. Direct immunofluorescence failed to demonstrate deposits of immunoglobulin other than nonspecific IgM deposition along the basement membrane zone of lesional skin. Ultrastructurally, the most striking findings were disintegration of basal cell cytoplasm and tubuloreticular inclusions in vascular endothelia. Taken together, the histologic and ultrastructural features of lupus-like lesions associated with Bloom syndrome mimic those of cutaneous lupus erythematosus, with the exception of paucity of immune deposits at the dermoepidermal junction.

Basement Membrane↗
PubMed1998

Topical immunotherapy of severe alopecia areata with diphenylcyclopropenone: evaluation of 68 cases.

BACKGROUND: Currently, topical immunotherapy with diphenylcyclopropenone (DCP) represents the most accepted therapeutic modality in the treatment of severe alopecia areata (AA). OBJECTIVE: Efficacy, side effects and prognostic factors of DCP treatment in severe AA. METHODS: Retrospective study of 68 patients with severe AA (> 40% scalp hair loss), treated for at least 5 months at the Department of Dermatology, University Hospital of Zürich, between May 1989 and December 1996. RESULTS: The overall response rate was 70.6%, complete remission was obtained in 30.9% and partial remission in 39.7%. Among the investigated prognostic factors for the outcome of DCP therapy, only the extent of AA at the time of initiation of treatment was found to be of significance. CONCLUSION: DCP treatment of severe AA is an effective, albeit symptomatic therapy with frequent side effects and a relatively high relapse rate. The response rate depends on the type of AA.

Administration, Topical↗
PubMed1997

[Cicatricial alopecias: diagnosis and therapy].

The cicatricial alopecias often are both a diagnostic and therapeutic challenge to the practitioner. Where there is no obvious physical/chemical injury or acute infectious etiology, clinical differential diagnosis of scarring alopecia is often difficult. The loss of follicular orifices in an area of alopecia points to a permanent loss of hair. In all of these cases a scalp biopsy is indicated. Primary and secondary scarring alopecia are differentiated: While the former is due to preferential destruction of the follicle, the latter results from events outside the follicle, which eventually impinge upon and eradicate the follicle. These include infiltrative processes such as granulomatous inflammation or neoplastic disease. In the group of primary scarring alopecia, well-defined chronic-inflammatory diseases of the scalp amenable to specific therapies are differentiated microscopically on the basis of the pattern of inflammation and the type of inflammatory cell that predominates. Thus, accurate diagnosis based on histopathology is a prerequisite to any rational therapy. Management of the less well classified diseases is much more problematic. Where end-stage fibrosis is established, surgical treatment and/or prosthetic help are taken into consideration. With the expanding knowledge of the immunology and molecular biology of the hair follicle, there is hope for the feasibility of therapeutic interventions that interfere early in the course of the pathogenetic processes ultimately leading to the permanent loss of hair.

Alopecia↗
PubMed1997

[Idiopathic eosinophilia].

Peripheral and tissue eosinophilia are associated with a wide variety of inflammatory syndromes. These include both multisystem and limited diseases with vasculitis or non-vasculitic tissue damage and variable expression of end-stage-fibrosis. The idiopathic hypereosinophilic syndrome (IHS) represents a multisystem disorder defined by sustained eosinophilia of an undetectable cause with significant organ system dysfunction. Although not specified as such in the criteria for the diagnosis of IHS, there are idiopathic eosinophilic syndromes that are clinically distinct from IHS by virtue of the fact that the eosinophilic inflammation is limited to specific tissues (such as the skin) with an overall good prognosis. The pathogenic role of the eosinophilic granulocyte in these conditions is attested by evidence of eosinophil activation and degranulation at sites of tissue injury. The recruitment and localization of eosinophils to specific sites of tissue inflammation involves cytokines with haematopoietic growth factor activity, adhesion molecules expressed both by the vascular endothelium and eosinophils, and chemoattractants that stimulate eosinophil migration. Recently, overexpression of IL-5 in transgenic mice was shown to lead to both peripheral blood eosinophilia and tissue eosinophilia. With the advances in our understanding of cytokine-dependent regulatory mechanisms that control the peripheral eosinophil number as well as the recruitment and survivability of eosinophils at sites of inflammation, more targeted ways of manipulating the eosinophil reaction can be expected in the future.

Animals↗
PubMed1997

[Dapsone in granulomatous rosacea].

We report on two patients with granulomatous rosacea and another patient with granulomatous perioral dermatitis who responded well to dapsone. Dapsone has a pharmacological double function as both an antibiotic and an antiphlogistic drug. Before the introduction of isotretinoin, dapsone had its place in the treatment of severe acne. To date, its use in granulomatous rosacea has not been described. When hematologic parameters are monitored, dapsone is considered a safe and cost-effective drug, especially in countries where isotretinoin is not readily available. However, the definite value of dapsone in granulomatous rosacea should be established by a controlled study.

Adult↗
PubMed1997

[Tufted hair folliculitis].

A case of tufted hair folliculutis presenting as circumscribed, tender and inflamed areas in the occiput with residual tufted follicles in a 28-year old man is reported. Tufted hair folliculitis is a characteristic localized scarring bacterial folliculitis of the scalp due to Staphylococcus aureus. Histopathological studies reveal perifollicular inflammation around the upper portions of the follicles sparing the hair root level. Within areas of inflammation, several follicles converge toward a common follicular duct with a widely dilated opening. Currently, tufted hair folliculitis is considered a variant of folliculitis decalvans of Quinquaud. Staphylococcal infection is believed to be an initial causative factor, and underlying differences in follicular anatomy or host response may be important in determining which reaction pattern occurs in an affected individual. The development of atrophy with loss of adnexal structures (in folliculitis decalvans) or of hair tufts (in tufting folliculitis) may depend upon the depth and destructive potential of the inflammatory process. The therapeutic approach is problematic; prolonged treatment with oral antibiotics may stabilize the disease, but good and at times more definitive results (as in the presented case) have been reported after radical surgical excision of the involved areas.

Adult↗
PubMed1997

[Trichodynia].

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Alopecia↗