Topical tacrolimus in granuloma annulare and necrobiosis lipoidica.
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Administration, Topical↗
Biomedical subjects
W Harth
Publications and source records attributed to W Harth.
The borderline syndrome in psychosomatic dermatology. Overview and case report.
The borderline syndrome is one of the most severe disturbances of psychosomatic dermatology. Patients with borderline syndrome are situated 'on the border' of psychosis, neurosis and personality disorders. The skin as a borderline organ carries a symbolic role. The clinical picture includes artefactual skin diseases due to self-mutilation by conscious or unconscious cutting, and rubbing, scratching or para-artefactual manipulations of pre-existing dermatoses. Leading symptoms of the borderline syndrome are poor impulse control, emotional instability and poor ego strength with low frustration tolerance and unstable personal relationships. We present the case of a 38-year-old female patient with borderline syndrome suffering from para-artefactual skin diseases of the face and a massive hyperhidrosis of the hands and feet. Within 9 months she was treated in four acute psychiatric hospitals and by 12 psychiatrists and psychotherapists. Early and accurate diagnosis and high-quality, sophisticated long-term therapy are necessary.
Adult↗
[Prevalence of psychosomatic disorders in dermatologic patients. Experiences in 2 dermatology clinics with a liaison therapy model].
INTRODUCTION: The prevalences and differences of biopsychosocial disorders were investigated in the dermatologic clinics of Erfurt and Giessen, where a liaison-therapy model has been established. Different dermatological diseases were compared by a variety of psychological tests, and patients with the same diagnosis were compared between the two clinics. PATIENTS AND METHODS: We examined 406 patients for psychosomatic problems with diagnostic interviews and psychometric tests. The coping of skin disease questionnaire (CSD) and the Symptom Checklist (SCL-90R) served as diagnostic measures. Between 1995-2000, 71 patients were seen in Erfurt and 335 in Giessen. The distribution of skin diseases and the psychosomatic disorders are shown by ICD-10 diagnoses. RESULTS: The comparison of an East and a West German city showed no significant differences in the whole group or the parallel groups using the CSD and SCL-90R. There was only a tendency to a greater reduction of quality of life in Erfurt. Patients with glossodynia evaluated themselves in all psychometric tests as very "normal". By contrast, patients suffering from alopecia and acne felt very helpless and seemed to have a need of greater psychosocial care. CONCLUSIONS: The biopsychosocial characteristics of the patients treated in the dermato-psychosomatic units in Erfurt and Giessen show no significant differences. Patients with diseases such as alopecia and acne are underestimated in regard to the need for additional psychosocial care. The liaison therapy model is an effective approach to diagnose psychosomatic-dermatological disorders and decide if further psychosomatic treatment is indicated.
Adolescent↗
[Botulinophilia. The new life style venenophilia].
BACKGROUND AND OBJECTIVE: Botulinum toxin is effective in the treatment of hyperhidrosis and the demand for therapy is increasing. Simultaneously we have observed an increase in patients with body dysmorphic disorders who also want botulinum toxin therapy. This botulinophilie is a new variant of venenophilie. We investigated the prevalence of this new diagnosis in our patient population. PATIENTS/METHODS: In the first quarter of 2000 we studied the biopsychosocial features of 13 patients with hyperhidrosis. RESULTS: In 23.1% of our cases we were able to confirm a botulinophilie with body dysmorphic disorder and a normal Minor sweat test. CONCLUSIONS: Botulinophilie is not an indication for botulinum toxin therapy but for psychotherapy.
Adult↗
Darier disease--novel mutations in ATP2A2 and genotype-phenotype correlation.
Darier disease (DD) is with a frequency of up to 1 in 36,000 a relatively common genodermatosis with autosomal dominant inheritance and late age of onset. The progressive skin manifestations are variable, but often debilitating and disfiguring, and may be associated with a wide range of neuropsychiatric problems, such as epilepsy and depression. On histology, acantholysis and dyskeratosis are prominent findings, implicating impaired functionality of desmosomes. Recently, mutations in the ATP2A2 gene encoding SERCA2, a calcium pump of the endo/sacrcoplasmic reticulum, have been identified as the molecular basis of DD. This slow-twitched calcium ATPase has two splice variants, one of which is highly expressed in epidermis, and maintains low intracellular calcium levels by facilitating transport of cytosolic calcium into the endoplasmic reticulum. Thus, it may confer a direct effect on the established calcium-dependent assembly of desmosomes. We screened ATP2A2 in a cohort of 24 DD families using conformation sensitive gel electrophoresis and direct sequencing, and detected 14 distinct mutations, 9 of which were novel. The mutational spectrum included 9 missense mutations, 1 nonsense mutation, 3 small in-frame deletions, and a 19-basepair insertion. Mutations were scattered over the entire gene with a slight preponderance in the first 8 exons, and affected exclusively residues conserved among all SERCAs. In addition, we found 2 silent polymorphisms, 1 of which occurred in 4 unrelated families. Comparison of molecular data and phenotypic features, such as severity and type of disease, occurrence of mucosal involvement, or association with neuropsychiatric disorders, did not reveal an obvious genotype-phenotype correlation in our cohort.
Calcium-Transporting ATPases↗
Body dysmorphic disorder and life-style drugs. Overview and case report with finasteride.
The body dysmorphic disorder is the repeated preoccupation with a minimal or non-evident defect and includes a wide spectrum of imagined defects in appearance. These patients present themselves in every clinical practice and are extraordinarily difficult to treat. The focus of the preoccupation concerns head, face, chest and the genital area. Following the introduction of the new "life-style" drug, finasteride, we observed a dramatic increase in the number of patients suffering from body dysmorphic disorder attending our clinic for skin diseases in Erfurt. These patients frequently contact their doctor demanding specifically for prescription of a particular life-style drug. However, there is no indication for using life-style drugs for the treatment of a body dysmorphic disorder. The appropriate treatment includes psychotherapy and psychopharmacological treatment.
Adult↗
Botulinophilia: contraindication for therapy with botulinum toxin.
Botulinum toxin inhibits neuromuscular transmission and is one of the most potent toxins. It has proven to be effective in the treatment of hyperhidrosis and is being more frequently demanded for therapy. Patients with body dysmorphic disorder also seek costly treatment with botulinum toxin. This botulinophilia is a new venenophilia. Body dysmorphic disorder is defined as a preoccupation with an imagined defect in appearance. If a slight physical anomaly is present, the person's concern is markedly exessive. The patient's preoccupation causes clinically significant distress or impairment in socially, occupational, or other important areas of functioning. The sweat test according to Minor is negative. Patients with botulinophilia are among the most difficult patients managed by the dermatologist. They are demanding and time-consuming. In our clinic, 23.1% of a series of patients seeking treatment with botulinum toxin screened positive for body dysmorphic disorder. Botulinophilia is a contraindication for therapy with botulinum toxin but is an indication for psychotherapy.
Adult↗
[Dermatological stigmata in Rubinstein-Taybi syndrome].
The clinical features of the Rubinstein-Taybi syndrome include mental deficiency and broad thumbs and toes. Typical dermatological findings are capillary hemangioma of the forehead and hypertrichosis. Rubinstein-Taybi syndrome patients also seem to be prone to develop keloids. We present a case of a one year old girl with Rubinstein-Taybi syndrome and the typical dermatological features.
Diagnosis, Differential↗
Dermatological symptoms and sexual abuse: a review and case reports.
Dermatological symptoms in cases of sexual abuse can be very diverse. To establish a causal relationship between skin diseases and sexual abuse is particularly difficult. In dermatology, three main areas of presentation can be identified. ACUTE CONSEQUENCES: Direct injuries found on the genitalia and body. Behaviour and psychological changes seen. Sexually transmitted diseases (STD) may be identified, after an appropriate incubation period. LONG-TERM CONSEQUENCES: In the long term, even decades later, patients may manifest with a wide spectrum of psychosomatic manifestations of skin diseases, particularly factitious disorders. IMITATIONS: A group whose skin manifestations may mimic and be mistaken for sexual abuse. The initial suspicion of sexual abuse and the need for specific questioning and investigations can lead to a disturbance in the doctor-patient relationship.
Adult↗
[Keratosis follicularis spinulosa decalvans associated with patent ductus arteriosus and hypospadia in an Asiatic patient].
Keratosis follicularis spinulosa decalvans (KFSD) appeared sporadically in an Asian boy, who also presented with naevus teleangiectaticus lateralis, patent ductus arteriosus (Botalli) and hypospadia. The association of these findings raises the question of a new syndrome. In addition, this is the first report of KFSD in a patient of Asian origin.
Asian People↗
Molecular genetic analysis of two families with keratosis follicularis spinulosa decalvans: refinement of gene localization and evidence for genetic heterogeneity.
X-linked keratosis follicularis spinulosa decalvans (KFSD) is a rare disorder affecting both skin and eyes. In the two extended KFSD families analysed to date, the gene was mapped to Xp22.13-p22.2. By analyzing several new markers in this region, we were able to narrow the candidate region to a 1-Mb interval between DXS7161 and (DXS7593, DXS7105) in the large Dutch pedigree. In addition, we analyzed 23 markers in Xp21.2-p22.2 in a German family with KFSD. Haplotype and recombination analysis positioned the KFSD gene in this family most likely outside the candidate region on Xp22.13-p22.2. This finding is suggestive for genetic heterogeneity: in this pedigree there is either another locus on the X-chromosome, or KFSD is transmitted here as an autosomal dominant trait with variable expression.
Child, Preschool↗
Hailey-Hailey disease maps to a 5 cM interval on chromosome 3q21-q24.
Hailey-Hailey disease (HHD) is a rare autosomal dominant genodermatosis characterized by disturbed keratinocyte adhesion. The disease has recently been mapped to a 14 cM region on chromosome 3q. We have further refined the location of the HHD gene by linkage analysis in six HHD families from Germany and Italy using 11 polymorphic microsatellite markers and found no evidence for genetic heterogeneity. We observed complete cosegregation between HHD and marker D3S1587, with a maximal lod score of 4.54. Detailed haplotype analyses allowed us to narrow the interval containing the HHD locus to 5 cM, flanked by D3S1589 and D3S1290.
Chromosome Mapping↗
Fine mapping of the Darier's disease locus on chromosome 12q.
Darier's disease (DD) is an autosomal dominant genodermatosis characterized by epidermal acantholysis and dyskeratosis. We have performed genetic linkage studies in 10 families with DD (34 affected) by analyzing 14 polymorphic microsatellite markers. Our results confirm recent reports mapping the DD gene to chromosome 12q23-q24.1. Haplotype analysis of recombinant chromosomes in our families, along with previously reported data, narrow the location of the DD gene to a 5 cM interval flanked by the loci D12S354 and D12S84/D12S105. This localization allowed exclusion of two known genes, PLA2A and PAH, as candidate loci for DD. Three other gene loci (PPP1C, PMCH, PMCA1), mapping in 12q21-q24, remain potential candidates.
Adolescent↗
[Retinoids in therapy of granuloma anulare disseminatum].
We report on four patients with granuloma annulare disseminatum successfully treated with etretinate as basic medication. In two cases there was complete remission after treatment with initial doses of up to 0.9 mg/kg body weight etretinate. One patient did not respond to etretinate alone but the condition was cured when Re-PUVA therapy was administered in addition. In another patient, with widespread granuloma annulare, primary treatment with Re-PUVA was instigated, followed by low-dose monotherapy with etretinate. According to these findings, we propose a three-stage regimen of therapy. After checking that the indications are appropriate, we first initiate monotherapy with etretinate and switch to a low-dose treatment over a longer period on response. If there is no response we switch to the second stage, i.e. Re-PUVA therapy; the third is then etretinate monotherapy to prevent relapse.
Aged↗
[Hailey-Hailey disease. Early detection of heterozygotes by an ultraviolet provocation tests--clinical relevance of the method].
An UV provocative test to identify genotypical carriers of Hailey-Hailey disease was developed, and performed on 30 non-affected family members of 8 families. The test revealed that 10 individuals were genotypical carriers without clinical signs. We checked the reliability of the UV provocative test during a 3-year follow-up period (family register method). In 50% of the carriers identified by the UV provocative test the first clinical manifestations of Hailey-Hailey disease developed in this period. All individuals with negative UV provocative test results remained clinically healthy. Up to now our results with the UV provocative test have been verified by the clinical development in 80% of cases. The UV provocative test is a practicable and reliable method of determining genotypical carriers of Hailey-Hailey disease.
Adult↗
[Keratosis follicularis spinulosa decalvans. Therapy with isotretinoin and etretinate in the inflammatory stage].
Keratosis follicularis spinulosa decalvans (KFSD) is a rare X-linked disorder of keratinization of the hair follicle associated with corneal dystrophy. The clinical picture is characterized by solid follicular hyperkeratosis, especially on the exposed skin, sparse eyebrows/eyelashes, follicular scaling and scarring alopecia of the scalp, dry skin and ocular symptoms with keratitis and photophobia. We describe the three stages of the disease: onset, inflammation and partial remission and the treatment appropriate in each. Two patients in the inflammatory stage of KFSD, with recurrent deep, fibrosing folliculitis and perifolliculitis followed by spreading and scarring alopecia on the scalp, responded to oral therapy with retinoids. In both cases there was a distinct and lasting remission of the inflammation and stabilization of the spreading alopecia after treatment with etretinate (Tigason), up to 0.8 mg/kg body weight, or isotretinoin (Roaccutan), 0.5 mg/kg body weight, for 12 weeks. The follicular spinulous hyperkeratosis became softer, but persisted. Thus, oral therapy with retinoids appears helpful in the inflammatory stage of KFSD, even though there is little improvement in the follicular hyperkeratosis.
Administration, Oral↗
Dermatologic problems for immigrant communities in a Western environment.
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Adult↗
Clinical pictures and classification of somatoform disorders in dermatology.
Somatoform disorders in dermatology are a heterogeneous group from a biopsychosocial point of view. Among the clinical patterns we find, for example, pruritus, pain, paresthesia as well as feelings of disfiguration, eco-syndromes, erythrophobia or psychogenic pseudoeffluvium. The multiple clinical symptoms are usually accompanied by psychosocial disorders, these are subjective complaints by the patient which cannot be medically objectified. The relevant somatoform disorders in dermatology can be differentiated as somatisation disorders, hypochondriacal disorders, somatoform autonomous disorders, persistent somatoform pain disorders and "other somatoform disorders". A precise differential-diagnostic division is necessary in order to initiate adequate therapy strategies. With this overview article, we would like to make an updated classification recommendation for dermatology and present experiences in therapy.
Humans↗