Successful grafting with EpiDex in pyoderma gangrenosum.
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Aged↗
Biomedical subjects
Ralph M Trüeb
Publications and source records attributed to Ralph M Trüeb.
[Prescriptions for the unhealthy scalp. How to treat itching and dandruff].
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Animals↗
Dermocosmetic aspects of hair and scalp.
Hair cosmetic agents are preparations intended for placing in contact with the hair and scalp, with the purpose of cleansing, promoting attractiveness, altering appearance, and/or protecting them in order to maintain them in good condition. Current shampoo formulations and styling products are tailored to the variations associated with age, gender, hair quality, hair care habit, and specific problems relating to the superficial condition of the scalp. The great amount of variables to be accounted for, some of them contradictory and hard to bring into accord, makes adequate product formulation a challenge and requires continuous research. Recent hair cosmetic developments include topical hair growth stimulants, photoprotectors, and anti-aging compounds. The advances in molecular technology have opened the avenue to the study of the molecular basis of hair growth and its deviations, as well as of the aging process. The discovery of potential pharmacological targets and the development of selective and effective delivery systems following topical application indicate further strategies for maintenance of healthy hair and scalp in the young and old.
Hair Diseases↗
Pyoderma gangrenosum.
Pyoderma gangrenosum (PG) is a non-infectious reactive neutrophilic dermatosis which typically starts with pustules which rapidly evolve to painful ulcers of variable size and depth with undermined violaceous borders. Since its first description in 1930, the pathogenesis of PG has remained elusive even as an ever-widening range of systemic diseases has been described in association with it. The diagnosis of PG is based on clinical and pathologic features and requires exclusion of other conditions that produce ulcerations, since misdiagnosis exposes patients to risks associated with treatment. Critical to proper management are correct diagnosis, identification and treatment of any underlying disorder, and the appropriate choice of topical and systemic therapy. PG has four distinctive clinical and histologic variants, and the specific clinical features of the lesion may provide a clue to the associated disease. The most common associated diseases are inflammatory bowel disease, rheumatological or hematological disease or malignancy. Although there is no single successful treatment for PG, certain type of PG lesions are recognized to respond more readily to accepted therapies than others. Local treatment may be sufficient for mild disease, while systemic immunosuppressive therapy is necessary for severe cases. The treatments with the best clinical evidence are oral or pulse intravenous corticosteroids, and cyclosporine. Surgical therapy is useful in selected cases in conjunction with immunosuppression. Wound stabilization is obtained only through control of the systemic and local inflammatory process. Emerging therapies include use of platelet-derived growth factor and cell culture grafts when re-epithelialization is slow, and the TNF-alpha blocking agent infliximab for refractory disease. Despite advances in therapy, the long-term outcome for patients with PG remains unpredictable, because relapses are common.
Administration, Oral↗
Hermansky-Pudlak syndrome type 4 in a patient from Sri Lanka with pulmonary fibrosis.
Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by oculocutaneous albinism and a platelet storage pool deficiency. Some patients also develop fatal pulmonary fibrosis and some have granulomatous colitis. Six human genes HPS1, ADB3A, HPS3, HPS4, HPS5, and HPS6 have been identified as cause of the six known subtypes of HPS. While there exist nearly 500 Puerto Rican and non-Puerto Rican HPS-1 patients, very few HPS-4 patients have been reported, and most of these have not been described in detail. We now delineate the clinical characteristics of an HPS-4 patient homozygous for a novel HPS-4 mutation, P685delC. The patient, the first individual with HPS reported from Sri Lanka, had severe pulmonary fibrosis, typical of HPS-1 disease, without granulomatous colitis. We conclude that pulmonary fibrosis occurs as part of HPS-4 and that HPS should be considered in all ethnic groups.
Albinism, Oculocutaneous↗
Micronodular Kaposi's sarcoma - a new variant of classic-sporadic Kaposi's sarcoma.
Kaposi's sarcoma (KS) in its classic-sporadic form is a rare vascular neoplasm affecting predominantly elderly men of Jewish or Mediterranean origin. Women are very rarely affected by KS. The tumor manifests itself most commonly with brownish macular or infiltrated oval lesions on the lower legs in both genders. Human herpesvirus 8 (HHV-8) has recently been demonstrated to be etiologically linked to KS. We report a 51-year-old HIV-seronegative Caucasian woman with an unusual, previously not described variant of KS which presented with small (3 mm in diameter) scattered firm red papules on her right arm. The histology with nodular spindle cell tumor expressing endothelial markers, the demonstration of serum antibodies against HHV-8 and the presence of viral nucleic acids in the lesional tissue proved the diagnosis of the nodular stage of KS. An indolent course characterized by clinically identical seasonal recurrences, even after surgical treatment and cryosurgery, was observed during the follow-up period of 9 years. We propose the term 'micronodular KS' for this unusual clinical variant of KS mimicking capillary hemangioma.
Antibodies, Viral↗
Finasteride treatment of patterned hair loss in normoandrogenic postmenopausal women.
BACKGROUND: Finasteride, an inhibitor of type 2 5alpha-reductase, inhibits conversion of testosterone to dihydrotestosterone, resulting in a decrease in serum and scalp dihydrotestosterone levels believed to be pathogenic in androgenetic alopecia. Oral finasteride has been shown to be effective in the treatment of hair loss in men, while its efficacy in women has remained controversial. METHODS: 5 postmenopausal women without clinical or laboratory signs of hyperandrogenism were given 2.5 or 5 mg/day oral finasteride for the treatment of pattern hair loss. Efficacy was evaluated by patient and investigator assessments, and review of photographs taken at baseline and at months 6, 12 and 18 by an expert panel. RESULTS: Finasteride treatment improved scalp hair by all evaluation techniques. The patients' self-assessment demonstrated that finasteride treatment decreased hair loss, increased hair growth and improved appearance of hair. These improvements were confirmed by investigator assessment and assessments of photographs. No adverse effects were noted. CONCLUSIONS: Oral finasteride in a dosage of 2.5 mg/day or more may be effective for the treatment of pattern hair loss in postmenopausal women in the absence of clinical or laboratory signs of hyperandrogenism.
5-alpha Reductase Inhibitors↗
Intravenously applied IgG stimulates complement attenuation in a complement-dependent autoimmune disease at the amplifying C3 convertase level.
Intravenously applied normal human immunoglobulin G (IgG) has anti-inflammatory effects in the treatment of autoimmune diseases. Systemic inflammation can originate from an overreacting amplification loop of the complement system. In blood, C3b2-containing complexes maintain complement amplification much better than the extremely short-lived C3b. Therefore, in patients with the complement-dependent autoimmune disease, dermatomyositis, we studied whether intravenously applied normal human IgG (IVIG) stimulated in vivo inactivation of these complexes. In the course of IVIG treatment, clinically effective in 6 of 8 patients, the concentration of C3b2-containing complexes dropped to 37% +/- 14% (n = 6) of the pretreatment level when having infused 0.5 g IgG/kg body weight, increased marginally and in parallel to factor Bb thereafter until full-dose IgG was infused. By day 14 following infusion of 2 g IgG/kg body weight the concentration of C3b2-containing complexes was 66% +/- 19%. The plasma concentration of C3 remained constant in myopathic or increased by 15% to 20% in amyopathic patients. In contrast to this, IVIG infusion was associated with consumption of up to 40% of plasma C4 at day 1 to 2 after completion of IVIG infusion. Thus, IVIG had an immediate and long-lasting attenuating effect on complement amplification in vivo, despite the fact that it induced classical complement pathway activation.
Autoimmune Diseases↗
[Worms and allergy excluded. What is the etiology of eosinophilia?].
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Diagnosis, Differential↗
Direct immunofluorescence of plucked hair in pemphigus.
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Autoantibodies↗
[Trichoteiromania].
BACKGROUND: The physical influences leading to traumatic hair injury may be the result of cosmetic treatments, may be accidental or self-inflicted. The most frequent cause of self-inflected hair loss is trichotillomania, in which the hair is plucked, while trichotemnomania, in which the hair is deliberately cut, is less frequent. Freyschmidt-Paul et al. proposed the term trichoteiromania for yet another type of artificial hair loss, which results from perpetual rubbing of the scalp with fracturing of the hair shafts. PATIENTS AND METHODS: Four patients with trichoteiromania are further characterized on the basis of clinical, morphological and psychopathological criteria. RESULTS: In contrast to trichotillomania, trichoteiromania has no diagnostic histopathological features and has a normal trichogram. Traumatic changes to the hair shaft are more conspicuous, with splitting at the ends of the hairs, giving the impression of white tips. The underlying mental disorder varis among the patients, though scalp dysaesthesia, not explained through any specific dermatological disorder, is a common denominator in all cases. CONCLUSIONS: While trichotillomania is considered to be an obsessive-compulsive disorder, the underlying mental disorder in trichoteiromania represents a more heterogeneous group. Cooperation with the psychiatrist is indicated, as much as the management and prognosis of trichoteiromania will depend on recognition of the underlying mental disorder and its specific psychotherapeutic and pharmacological treatment.
Aged↗
Immediate-type hypersensitivity reaction to ingestion of mycoprotein (Quorn) in a patient allergic to molds caused by acidic ribosomal protein P2.
BACKGROUND: Quorn is the brand name for a line of foods made with so-called "mycoprotein," which springs from the mold Fusarium venenatum. Since the introduction on the food market, there have been complaints from consumers reporting adverse gastrointestinal reactions after ingestion of mycoprotein. To date, it is not clear whether the reported symptoms are IgE-mediated. OBJECTIVE: The aim of the study was to describe for the first time a case history of an asthmatic patient with severe hypersensitivity reactions to ingested mycoprotein and to identify and characterize the potential allergen that might be responsible for this. METHODS: The sensitization pattern of the asthmatic subject was characterized, and food allergy to mycoprotein was assessed by double-blinded placebo-controlled food challenge. Afterward, specific IgE antibodies of the serum of this patient were used to screen a Fusarium culmorum cDNA expression library. The coding sequence of one enriched cDNA-clone was expressed in Escherichia coli to produce a recombinant protein that was further purified and immunologically characterized. RESULTS: The patient showed high sensitization to many known aeroallergens but apart from Quorn not to any other tested food samples. The deduced amino acid sequence of the enriched cDNA-clone (Fus c 1) showed large identity to the 60S acidic ribosomal protein P2 which is highly conserved among several species and also described as minor allergen in other mold species. The frequency of IgE reactivity of sera from F culmorum -sensitized subjects to rFus c 1 was approximately 35%. By enzyme allergosorbent test inhibition, we found 65% inhibition of mycoprotein IgE reactivity by rFus c 1. On the opposite we found reduced IgE reactivity of rFus c 1 of 68% by using mycoprotein as inhibitor. CONCLUSIONS: Sensitization to mold allergens by the respiratory tract and subsequent oral ingestion of cross-reactive proteins may lead to severe food-allergic reactions. Thus, the 60S acidic ribosomal protein P2 of F venenatum probably is the reason for the described severe hypersensitivity reactions of the patient to Quorn-mycoprotein because of its potential cross-reactivity to the F culmorum allergen Fus c 1.
Adult↗
Association between smoking and hair loss: another opportunity for health education against smoking?
Besides being the single most preventable cause of significant morbidity and an important cause of death in the general population, tobacco smoking has been associated with adverse effects on the skin. Smoke-induced premature skin ageing has attracted the attention of the medical community, while only recently an observational study has indicated a significant relationship between smoking and baldness. The mechanisms by which smoking causes hair loss are multifactorial and are probably related to effects of cigarette smoke on the microvasculature of the dermal hair papilla, smoke genotoxicants causing damage to DNA of the hair follicle, smoke-induced imbalance in the follicular protease/antiprotease systems controlling tissue remodeling during the hair growth cycle, pro-oxidant effects of smoking leading to the release of pro-inflammatory cytokines resulting in follicular micro-inflammation and fibrosis and finally increased hydroxylation of oestradiol as well as inhibition of the enzyme aromatase creating a relative hypo-oestrogenic state. In view of the psychological impact of androgenetic alopecia on affected men and women, increasing public awareness of the association between smoking and hair loss offers an opportunity for health education against smoking that may be more effective than the link between smoking and facial wrinkles or grey hair, since the latter can be effectively counteracted by current aesthetic dermatologic procedures, while treatment options for androgenetic alopecia are limited.
Alopecia↗
Is androgenetic alopecia a photoaggravated dermatosis?
Progressive thinning of the scalp hair in androgenetic alopecia (AGA) results in a gradual decline in natural protection of the scalp from ultraviolet radiation (UVR). A number of pathologic conditions of the scalp are evidently related to UVR, particularly photosensitive diseases and disorders of the chronically photodamaged bald scalp. The most important chronic effects of UVR are photocarcinogenesis and solar elastosis. Besides these, erosive pustular dermatosis and 'red scalp' are distinct disorders peculiar to the balding scalp. While the consequences of sustained UVR on the unprotected scalp are well appreciated, the effects of UVR on hair loss have widely been ignored. However, clinical observations and theoretical considerations suggest that UVR may have negative effects: acute telogen effluvium from UVR has been described, and the production of porphyrins by Propionibacterium sp. in the pilosebaceous duct, with photoactivation of porphyrins leading to oxidative tissue injury, has been implicated in follicular microinflammation. Alternatively, keratinocytes themselves may respond to physicochemical stress from UVR, besides irritants and pollutants, by producing radical oxygen species and nitric oxide and by releasing proinflammatory cytokines, eventually leading to injury of the putative site of follicular stem cells in the superficial portion of the hair follicle. Since all of these processes involved in hair loss share the common feature that they are induced or exacerbated by exposure to sunlight, it is proposed that AGA is a photoaggravated dermatosis that requires photoprotection.
Alopecia↗
Hair pain (trichodynia): frequency and relationship to hair loss and patient gender.
BACKGROUND: Patients complaining of hair loss frequently claim that their hair has become painful. OBJECTIVE AND METHODS: The aim of the study was to evaluate the frequency of this phenomenon and its relationship to hair loss. Patients seeking advice for hair loss either spontaneously reported or were questioned about painful sensations of the scalp. Hair loss activity was quantified by a hair pull, daily count and wash test. Telogen percentage was obtained by a hair pluck. The scalp surface was examined by dermatoscopy. RESULTS: Of 403 examined patients, 20% of women and 9% of men reported hair pain, irrespective of the cause and activity of hair loss. A minority presented scalp telangiectasia. This strongly correlated with hair pain. CONCLUSIONS: Hair pain (trichodynia) affects a significant proportion of patients complaining of hair loss and may increase the anxiety. The symptom neither allows discrimination of the cause nor correlates with the activity of hair loss. A higher prevalence of female patients might be connected to gender-related differences in pain perception in relation to anxiety. The role of vasoactive neuropeptides in the interaction between the central nervous system and skin reactivity is discussed. In the absence of any correlation with quantitative parameters of hair loss or specific morphologic changes of the scalp, management remains empiric and tailored to the individual.
Adolescent↗
Causes and management of hypertrichosis.
Hypertrichosis is the term used for the growth of hair on any part of the body in excess of the amount usually present in persons of the same age, race, and sex, excluding androgen-induced hair growth. In its generalized and circumscribed forms, hypertrichosis may either be an isolated finding, or be associated with other abnormalities. Therefore, accurate classification of hypertrichosis is mandatory. Excessive hair may cause cosmetic embarrassment, resulting in a significant emotional burden, particularly if extensive. Treatment options are limited, and the results of therapy not always satisfactory. Patients should, therefore, be adequately advised of the available treatment modalities for temporary or permanent hair removal. No single method of hair removal is appropriate for all body locations or patients, and the one adopted will depend on the character, area, and amount of hair growth, as well as on the age of the patient, and their personal preference. The currently available treatment methods include cosmetic procedures (bleaching, trimming, shaving, plucking, waxing, chemical depilatories, and electrosurgical epilation), and hair removal using light sources and lasers. Laser-assisted hair removal is the most efficient method of long-term hair removal currently available. The lack of comparative data make it difficult to choose the most effective system, however, although the color contrast between epidermis and the hair shaft will determine the type of laser to favor. A novel treatment for slowing excessive hair growth is topical eflornithine, an inhibitor of the enzyme ornithine decarboxylase present in hair follicles that is important in hair growth. In general, treatment of hypertrichosis is more satisfactory for patients with localized involvement, than for those with generalized hypertrichosis.
Androgen Antagonists↗
Hair and nail involvement in lupus erythematosus.
Lupus erythematosus (LE) is a systemic autoimmune disorder associated with polyclonal B-cell activation, resulting in diverse patterns of autoantibody production and a heterogeneous clinical expression constituting a spectrum extending from limited cutaneous disease (cutaneous lupus LE) to a life-threatening systemic disease process (systemic LE). While the clinical characteristics of cutaneous LE are well defined in terms of morphology, and for the classification of systemic LE (SLE), clinical and laboratory criteria are available, recent advances in basic and clinical research have contributed to a better understanding of the cellular and molecular events that lead to LE. The importance of genetic susceptibility, environmental factors, and hormonal elements are well appreciated, although we as clinicians are faced with relatively late-term manifestations of the immunologic events that lead to the clinical manifestations of disease. Nevertheless, combining our knowledge of the clinical patterns of LE with the advances in our understanding of the underlying immunology, has led to the concept of "subsets" of disease, where a correlation between immune genetic markers, autoantibody profiles, distinctive cutaneous morphology, and clinical outcome has become possible.
Hair Diseases↗
Molecular mechanisms of androgenetic alopecia.
Androgenetic alopecia (AGA) is hereditary and androgen-dependent, progressive thinning of the scalp hair that follows a defined pattern. While the genetic involvement is pronounced but poorly understood, major advances have been achieved in understanding principal elements of the androgen metabolism involved: androgen-dependent processes are predominantly due to the binding of dihydrotestosterone (DHT) to the androgen receptor (AR). DHT-dependent cell functions depend on the availability of weak androgens, their conversion to more potent androgens via the action of 5 alpha-reductase, low enzymatic activity of androgen inactivating enzymes, and functionally active AR present in high numbers. The predisposed scalp exhibits high levels of DHT, and increased expression of the AR. Conversion of testosterone to DHT within the dermal papilla plays a central role, while androgen-regulated factors deriving from dermal papilla cells are believed to influence growth of other components of the hair follicle. Current available treatment modalities with proven efficacy are oral finasteride, a competitive inhibitor of type 2 5 alpha-reductase, and topical minoxidil, an adenosine-triphosphate-sensitive potassium channel opener which has been reported to stimulate the production of vascular endothelial growth factor in cultured dermal papilla cells. Since the clinical success rate of treatment of AGA with modulators of androgen metabolism or hair growth promoters is limited, sustained microscopic follicular inflammation with connective tissue remodeling, eventually resulting in permanent hair loss, is considered a possible cofactor in the complex etiology of AGA.
Alopecia↗