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R M Trüeb

Publications and source records attributed to R M Trüeb.

At least 37 records · Page 2Linked to original sources
PubMed2001

[Reticular pigmented anomaly of the flexures. Dowling-Degos disease of the intertrigo type in association with acne inversa].

Reticulated pigmented anomaly of the flexures (Dowling-Degos disease) is a rare autosomal-dominant genodermatosis with variable penetrance that is characterized by reticulated hyperpigmentation of the flexures. Acne inversa has been previously described in the literature under several synonyms, such as acne triad, acne tetrad and hidradenitis suppurativa. Acne inversa is an inflammation affecting the pilosebaceous units of the flexures. We report a case of Dowling-Degos disease in association with acne inversa. The association of these diseases has been described several times and it appears not to be coincidental. Pathogenetic mechanisms leading to this coincidence are discussed.

Acne Vulgaris↗
PubMed2001

The value of hair cosmetics and pharmaceuticals.

As early as can be traced, written documents testify endeavors shown by humanity to please by means of the hair. Hair care, color and style play an important role in people's physical appearance and self-perception. Dermatologists should be knowledgeable about the procedures people follow to look their best and should have the competence to provide patients with information on the benefits and hazards of hair cosmetics and pharmaceuticals. Shampooing is the most common form of cosmetic hair treatment. The diversity of qualities expected from a shampoo by today's consumer surpass the primary function of cleansing. Current shampoo formulations are adapted to the variations associated with hair quality, hair care habit and specific problems related to the superficial condition of the scalp. Hand in hand, test methods are developed to evaluate the efficacy of hair care products so that consumers are offered products that perform as claimed. Through the development of cosmetics with pharmaceutically active compounds, products are evolving that are becoming more similar to topical therapeutic agents (cosmeceuticals). The efficacy of cosmeceuticals that claim to act as hair growth stimulants should be measured by the standards set by the drugs minoxidil and, more recently, oral finasteride. Finally, health hazards associated with the use of hair care products, especially rinse-off products, have been overemphasized by the media and need careful correction by opinion leaders.

Dermatitis, Allergic Contact↗
PubMed2001

Acquired hair fragility in pili anulati: causal relationship with androgenetic alopecia.

Pili anulati are defined by characteristic alternating light and dark banding in the hair shaft, due to air-filled spaces between the macrofibrillar units of the hair cortex, and are regarded as a congenital hair shaft disorder without increased hair fragility. Two cases of pili anulati are presented, in which fragility of hair developed in a causal relationship with the onset of androgenetic alopecia. Accordingly, trichorrhexis-nodosa-like hair fracturing was exclusively limited to the androgenetic region. In general, secondary trichorrhexis nodosa is an unspecific finding related to excess stress of hair in relation to its fragility. With onset of hair thinning due to androgenetic alopecia, progressive reduction of hair shaft diameter may cause increased fragility in pili anulati. In this case, hair shaft fracturing occurs within the area of androgenetic alopecia and colocalizes with the air-filled cavities of pili anulati.

Adult↗
PubMed2001

Acral necrosis of the fingers as initial manifestation of cutaneous polyarteritis nodosa--a case report.

Cutaneous polyarteritis nodosa (CPN) is a well-known entity showing subcutaneous tender nodules, livedo reticularis, and ulcerations as predominant features; arthralgia, myalgia, peripheral neuropathy, and general symptoms such as fever and malaise may also be present. Although the localization of the disease is not specific, the characteristic primary lesions are painful subcutaneous nodules on the lower extremities. Here we report the case of a 33-year-old man with blue-colored distal phalanges and necrosis of several fingertips on both hands as the initial manifestation of CPN.

Adult↗
PubMed2000

[Autoimmune dermatoses: new therapeutic strategies].

In the recent past, advances in both basic and clinical research have considerably contributed to the understanding of the cellular and molecular events that lead to autoimmune diseases. Nevertheless, current treatment protocols are essentially confined to systemic corticosteroids and cytotoxic agents. These are effective in controlling inflammation, however the occurrence of relapses after the treatment is stopped and the risks of toxicity and long-term immunosuppression necessitate the search for more selective strategies of immunointervention. Major efforts are directed towards therapies with increased cellular selectivity and, ideally, autoantigen specificity. Cyclosporine A, monoclonal antibodies (anti-CD4) and immunotoxins (IL-2 fusion toxins) currently constitute the main clinical orientation. At the experimental level, strategies are directed towards antigen-specific manipulation of costimulatory pathways (anti-gp39, CTLA4Ig), peptide therapy (MHC antagonism, TCR vaccination), and tolerance induction to the autoantigen (oral tolerization). Alternatively, intervention in the cytokine cascade of the inflammatory response by means of cytokine-induced immunomodulation (IL-4, IL-10) or cytokine antagonism (anti-TNF alpha) are strategies with a therapeutic potential. However, it must be borne in mind, that in animal models of antigen-induced autoimmune disease, it is simple to intervene in the development of autoimmunity, since the induction events are designed by the investigator. In contrast, in the majority of patients with autoimmune disease, we do not know whether a specific autoantigen initiated the disease, nor do we know the time point at which the antigenic challenge occurred. As clinicians we are faced with late-term manifestations of the immunologic events that lead to clinical disease. Finally, in limited forms of cutaneous autoimmune disease, it is important to recognize distinct subsets with a favorable prognosis, for which less aggressive therapeutic modalities may be used.

Autoimmune Diseases↗
PubMed2000

Fibrosing alopecia in a pattern distribution: patterned lichen planopilaris or androgenetic alopecia with a lichenoid tissue reaction pattern?

BACKGROUND: Androgenetic alopecia is characterized by a defined area of progressive nonscarring alopecia. The clinical and histological findings in 15 women and 4 men with progressive scarring alopecia in a pattern distribution were studied. The results were evaluated and compared with clinicopathologic entities that feature scarring of the central scalp area, specifically, lichen planopilaris, pseudopelade, and follicular degeneration syndrome. OBSERVATIONS: Patients developed progressive fibrosing alopecia of the central scalp, without the multifocal areas of involvement typical of lichen planopilaris and pseudopelade. Perifollicular erythema, follicular keratosis, and loss of follicular orifices were limited to a patterned area of involvement. Biopsy specimens of early lesions demonstrated hair follicle miniaturization and a lichenoid inflammatory infiltrate targeting the upper follicle region. Advanced lesions showed perifollicular lamellar fibrosis and completely fibrosed follicular tracts indistinguishable from end-stage lichen planopilaris, pseudopelade, or follicular degeneration syndrome. CONCLUSIONS: Some patients with androgenetic alopecia might have additional clinical and histological features of inflammation and fibrosis limited to the area of androgenetic hair loss. In these patients, the histological findings of early lesions are identical to those seen in lichen planopilaris. The lichenoid tissue reaction leading to follicular destruction in these patients might be pathogenetically related to the events underlying androgenetic alopecia.

Adult↗
PubMed2000

[Interrelations between physician, hairdresser and mass media in management of hair loss].

Hair problems are attacked at the levels of information supply, trichological competence and evidence based medical treatment. The alopecias represent a major health care challenge, and the psychological significance of hair loss has only recently found appreciation in the medical community. They may pose a vexing problem for non-specialized physicians, who tend to trivialize such complaints, although the patient's perception of abnormality determines whether or not advice is sought. Perception of a hair problem is subject to sociocultural standards; films and advertisements help determine the threshold for "normality" and what is "healthy" and "beautiful", and the media provide an abundance of undiscriminating information with respect to hair care. Patients with hair problems frequently consult the hairdresser prior to the medical specialist. Thus additional areas of competence should be incorporated into the hairdresser's training qualifications. Besides manual, creative and psychological aptitudes, "trichological competence" relating to the recognition of most important hair disorders, recognition of personal limitations and cooperation with the medical specialist are required. To optimize the interaction between the physician, hairdressers and the media, more effective communication and public activities from the medical profession are desirable, with success depending on full identification of the set goals, qualification and adherence to the standards determined by the medical speciality.

Alopecia↗
PubMed2000

[Idiopathic chronic telegon effluvium in the woman].

In approximately 30% of cases of chronic diffuse loss of scalp hair with a duration of at least 6 months, no underlying abnormality can be found. Typically this occurs in women, starting abruptly without a recognizable initiating factor, and involving the entire scalp area with increased shedding of telogen hair. With the exception of bitemporal recession, hair thinning is usually discrete, and contrasts to the great emotional overtones in this situation. This may initially lead to the differential diagnosis of psychogenic pseudo effluvium. Due to synchronization of the hair cycle, the amount of shed hair is greater than that in androgenetic alopecia, while miniaturized hairs are not a feature of the disorder. Overlap with androgenetic alopecia and/or psychogeneic pseudo effluvium is not uncommon. Scalp dysesthesia or a sensation of "pain in the hair" (trichodynia) is an accompanying symptom in a significant proportion of cases, and correlates better with emotional upset than with actual hair loss. Current therapeutic recommendations are pragmatic, and based on both experimental observations of the sheep wool industry and clinical experience. They include the use of L-cystine-containing oral preparations and of corticosteroids. Further investigation into the molecular controls of the hair cycle are required to find a more specific form of therapy, for which the expense and risk-benefit ratio seem appropriate for the treatment of this benign condition.

Alopecia↗
PubMed2000

[Acquired naevus flammeus (Fegeler syndrome)].

We report the development of an acquired nevus flammeus following a ski accident. This disorder was first described by Fegeler 1949, who reported the case of a 43-year-old soldier who acquired a nevus flammeus in the face following a cranial trauma. Since then, a number of similar case reports have been published. Differential diagnosis is discussed, such as the unilateral nevoid teleangiectasia syndrome, eruptive nevoid teleangiectasia and eruptive spider nevi.

Adult↗
PubMed2000

An autosomal dominant form of hereditary hypotrichosis simplex maps to 18p11.32-p11.23 in an Italian family.

We report on a three-generation Italian family with dominant transmission of a form of hereditary hypotrichosis simplex (HHS). The nine affected adults presented with sparse, thin and short hair. Somewhat less sparse and longer hair was observed in the two affected young children in the third generation. Reduced hair growth affected the scalp and body, although normal eyelashes, eyebrows and growth of men's beards were observed. No associated abnormality was detected and the overall psychomotor development of the affected individuals was normal. A phenotypic variation was observed amongst the family members and is suggestive of a reduced penetrance of the trait or the effect of a modifying factor. After exclusion, in our family, of linkage to loci previously described in other forms of atrichia or hypotrichosis, we performed a genome-wide linkage analysis, which resulted in a positive lod score at 18p11.32-p11.23. We defined a critical region of about 35 cM flanked by markers D18S853 and D18S40. The highest two-point lod score was obtained with the microsatellite markers D18S1376, D18S53 and D18S453 (lod score of 3.31 at theta = 0.00). The 18p11.32-p11.23 locus represents the first chromosome region shown to be associated with hereditary hypotrichosis simplex.

Adult↗
PubMed2000

Congenital hypotrichosis due to short anagen.

Two children with congenital hypotrichosis are described. Both presented with persistent short, fine hair since birth. Evidence is provided that the short hair observed in these patients is due to a short anagen phase of the hair cycle, with a normal rate of hair growth. Shortening of the anagen phase of the scalp hair cycle leads to a decrease in the maximal hair length and an increase in the number of hairs in telogen, resulting in an increase in hair shedding. Scanning electron microscopy showed a widely spaced cuticular pattern, a finding typically seen in hair of thin calibre. One patient had affected family members with an apparently autosomal dominant mode of inheritance. The disorder appears to resolve spontaneously during puberty and adulthood.

Cell Division↗
PubMed2000

Male-pattern baldness in men with X-linked recessive ichthyosis.

BACKGROUND: X-linked recessive ichthyosis (XRI) is a genetic disorder of keratinization with extracutaneous manifestations due to deficiency of steroid sulfatase (STS). Because STS plays an important role in androgen metabolism, and elevated levels of dehydroepiandrosterone sulfate have been reported in young men with andogenetic alopecia (AGA), the hypothesis was advanced that men with XRI do not show AGA or develop only mild forms of common baldness. METHODS: Patients with a diagnosis of XRI confirmed by analysis of the microsomal sulfatases in our clinic between 1984 and 1998, and in whom study of the case histories depicted the typical clinical presentation of XRI, were sent a questionnaire with the Hamilton-Norwood scale of patterns of hair loss in men, inquiring them to designate the condition of their scalp hair. RESULTS AND CONCLUSIONS: Of 15 questionnaires returned, 7 indicated Hamilton-Norwood I, 3 Hamilton-Norwood II, 3 Hamilton-Norwood III-vertex, 1 Hamilton-Norwood IV and 1 Hamilton-Norwood VII. The results of this survey do not support the hypothesis that XRI and AGA are mutually exclusive, in as much as advanced AGA was found among these men.

Adolescent↗
PubMed2000

Bowen's disease of the glans penis (erythroplasia of Queyrat) in plasma cell balanitis.

We report a case of a persistent penile plaque on the glans penis of allegedly more than 20 years' duration, which was refractory to circumcision and local treatment. Over the years, the patient repeatedly presented with a circumscribed inflammatory lesion of the glans penis, diagnosed as Zoon's balanitis on the basis of clinical aspects and two biopsies. Because of unresponsiveness of the lesion to circumcision and focal steroid infiltration, repeated biopsies were performed in an attempt to rule out malignancy. Two further biopsies were carried out. One again showed the features of a plasmacellular inflammation, while the other finally revealed the histopathologic features of erythroplasia of Queyrat (carcinoma in situ or Bowen's disease of the glans penis). We assume that either the former biopsy specimens were taken from a plasma cell-rich reactive infiltrate around the neoplastic lesion, or that carcinoma in situ may have arisen due to the chronic inflammation of Zoon's balanitis plasmacellularis. Radiotherapy was performed with good clinical response and subsequent histopathologic proof of complete remission of the lesion.

Aged↗
PubMed1999

[Cutaneous paraneoplastic syndrome as an immunologic phenomenon exemplified by paraneoplastic subacute cutaneous lupus erythematosus].

Two case reports and a review of the literature on paraneoplastic figurate erythemas with lupus erythematosus (LE)-specific histolopathology illustrate cutaneous paraneoplasia as an immunological phenomenon. Subacute cutaneous lupus erythematosus (SCLE) is characterized by photosensitive, annular or papulosquamous skin lesions with LE-specific histopathology in association with circulating anti-SS-A (Ro) auto-antibodies. The SS-A (Ro) antigen is a complex of cytoplasmic ribonucleoproteins that translocate to the surface of keratinocytes under UV irradiation. Sequestered cytosolic antigen that is expressed on the cell surface is presented to T lymphocytes by professional antigen presenting cells in the context of specific MHC class II molecules. The initiation of the specific immune response, the expansion of antigen-specific T helper cells, and the differentiation of B cells to antibody producing plasma cells depend on the further interaction with antigen. The risk of a self-perpetuating autoimmune response relies on further release of auto-antigen. It is conceivable that in paraneoplastic SCLE tumor antigen with homology to SS-A (Ro) leads to photosensitive autoreactivity. The review of the literature reveals 9 other cases of SCLE associated with internal malignancy. The most common associated tumors were bronchial and mammary carcinoma. The latency between the appearance of skin lesions and the diagnosis of an underlying tumor was between one month and three years (mean: 10 months). In all cases, skin lesions improved with cancer therapy, in three cases they (re)appeared in association with relapse of tumor.

Adenocarcinoma↗
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↗