[Generalised eczema and palmoplantar hyperkeratosis].
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Acrodermatitis↗
Biomedical subjects
S Grabbe
Publications and source records attributed to S Grabbe.
Fabry disease in a female patient due to a de novo point mutation at position 691 of exon 5.
Fabry disease is an X-linked lysosomal disorder caused by deficiency of the lysosomal enzyme alpha-galactosidase A. We report on a 32-year-old female patient with an 8-year history of vascular lesions on the hips and periumbilical region and a presumed Fabry disease without positive family history. Ophthalmologic evaluation revealed whorl-like corneal opacities. Echocardiography revealed myxomatous degeneration and prolapse of the mitral valve. DNA analysis of the alpha-galactosidase A gene confirmed the diagnosis of Fabry disease, showing a de novo point mutation at position 691 of exon 5. The patient is now obtaining intravenous enzyme replacement therapy with agalsidase alfa and remains without drug-related reactions.
Adult↗
[Manifestation of Fabry disease in a heterozygous female patient. New perspectives using enzyme replacement therapy].
HISTORY AND ADMISSION FINDINGS: A 32-year-old woman with bilateral corneal opacities was at 9 years of age diagnosed to have reduced activity of the enzyme alpha-galactosidase A. She was admitted to our hospital because of skin lesions that had developed over the last 8 years. INVESTIGATIONS: The clinical features included angiokeratomas on the buttocks, hips, and periumbilical region, whorl-like corneal opacity (cornea verticillata), and mitral valve prolapse. Activity of alpha-galactosidase A was reduced to about a quarter of the normal value. Histological examination revealed lipid deposits within the endothelial cells of the skin. Molecular analysis of the alpha-galactosidase A gene revealed a point mutation at nucleotide-position 691 in exon 5 (p.Asp231Asn). TREATMENT AND COURSE: Enzyme replacement therapy with agalsidase alfa 0.2 mg/kg body-weight, infused over 40 min every other week, was initiated. So far no side effects due to the infusion therapy have been noted. The therapeutic success (reduction of lipid storage) cannot be assessed as yet. CONCLUSION: Fabry disease results from deficient activity of the enzyme alpha-galactosidase A. Affected (hemizygous) males often show the complete spectrum of symptoms and signs and have a deficient alpha-galactosidase A activity. Occasionally milder oligosymptomatic courses are observed, when residual enzyme activity is present. In contrast to previous belief, heterozygous females may be affected in the same manner as hemizygotes and may also have a significantly reduced enzyme activity.
Abdomen↗
[Pruritus without skin findings].
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Antidepressive Agents, Tricyclic↗
[Actinic keratosis].
Actinic keratosis is a precancerous lesion that is commonly seen in the elderly patient and requires treatment. It shows a predilection for areas of the skin that are exposed to the sun, and usually manifests as multiple skin-colored or discrete reddish scaly verrucous plaques. The diagnosis is usually based on the clinical appearance of the lesion. Therapeutic options include physical, immunological and chemical measures. The choice of treatment is determined by numerous factors, and can be adapted to the individual situation presenting.
Adjuvants, Immunologic↗
[Patient with vitiligo. Help, the white spots are becoming even larger!].
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Child↗
[Dermatitis artefacta (factitial dermatitis)--an interdisciplinary diagnostic and therapeutic challenge].
Factitial cutaneous lesions result from self-inflicted injury leading to objectifiable clinically relevant damage to the skin. Triggering factors are frequently psychiatric disorders or specific stress situations. Clinical manifestations range from superficial erosions to deep wounds.The establishment of the diagnosis is often a time-consuming and complicated process that precedes acceptance of the underlying pathology by both the therapist and patient. If lasting therapeutic success is to be achieved, the possibility of such cutaneous lesions must be included in differential diagnostic considerations, and early interdisciplinary cooperation is necessary.
Adult↗
[Disseminated herpes zoster in diabetes mellitus].
HISTORY AND ADMISSION FINDINGS: A 71-year old man presented with painful hemorrhagic vesicles and papules over the entire body that had persisted for three days. Type 2 diabetes mellitus type 2 had been diagnosed 20 years ago and had not been treated for the last 5 years. Therapy had been discontinued by the patient. INVESTIGATIONS: HbA1c (11,9%) and blood glucose levels (up to 360 mg/dl) were abnormal. Varicella-zoster-DNA was replicated by PCR from the vesicle fluid. DIAGNOSIS AND TREATMENT: After the clinical diagnosis of disseminated herpes zoster had been confirmed systemic therapy with aciclovir 10 mg/kg day was started. There was no evidence of malignancy. Insulin therapy was initiated. CONCLUSION: Dissemination is a rare complication of herpes zoster, aided by immunosuppression. In the presented case there was no evidence of malignancy or other cause of immunosuppression, but the patient also had type 2 diabetes with very high blood glucose levels. The diabetes was thought to be causally related to the ineffective immune response to varicella zoster virus. There has been no previous published report of this relationship.
Acyclovir↗
Dacarbazine (DTIC) versus vaccination with autologous peptide-pulsed dendritic cells (DC) in first-line treatment of patients with metastatic melanoma: a randomized phase III trial of the DC study group of the DeCOG.
BACKGROUND: This randomized phase III trial was designed to demonstrate the superiority of autologous peptide-loaded dendritic cell (DC) vaccination over standard dacarbazine (DTIC) chemotherapy in stage IV melanoma patients. PATIENTS AND METHODS: DTIC 850 mg/m2 intravenously was applied in 4-week intervals. DC vaccines loaded with MHC class I and II-restricted peptides were applied subcutaneously at 2-week intervals for the first five vaccinations and every 4 weeks thereafter. The primary study end point was objective response (OR); secondary end points were toxicity, overall (OS) and progression-free survival (PFS). RESULTS: At the time of the first interim analysis 55 patients had been enrolled into the DTIC and 53 into the DC-arm (ITT). OR was low (DTIC: 5.5%, DC: 3.8%), but not significantly different in the two arms. The Data Safety & Monitoring Board recommended closure of the study. Unscheduled subset analyses revealed that patients with normal serum LDH and/or stage M1a/b survived longer in both arms than those with elevated serum LDH and/or stage M1c. Only in the DC-arm did those patients with (i) an initial unimpaired general health status (Karnofsky = 100) or (ii) an HLA-A2+/HLA-B44- haplotype survive significantly longer than patients with a Karnofsky index <100 (P = 0.007 versus P = 0.057 in the DTIC-arm) or other HLA haplotypes (P = 0.04 versus P = 0.57 in DTIC-treated patients). CONCLUSIONS: DC vaccination could not be demonstrated to be more effective than DTIC chemotherapy in stage IV melanoma patients. The observed association of overall performance status and HLA haplotype with overall survival for patients treated by DC vaccination should be tested in future trials employing DC vaccines.
Cancer Vaccines↗
[Allergic contact dermatitis from a hydrocolloid dressing due to colophony sensitization].
A 62-year-old female patient with a venous leg ulcer developed massive eczema during wound bed preparation with the hydrocolloid dressing Varihesive. The patch testing confirmed a pronounced sensitization to the hydrocolloid dressing apart from the sensitization to colophony. After review of the current literature we found several case reports from the last 10 years about sensitization to hydrocolloids which were identical but distributed under different brand names in different countries. These dressings contain the pentaerythritol ester of hydrogenated rosin as the tackifying agent which is the substance retaining the sensitizing potential of colophony. Especially patients with chronic wounds frequently tend to contact sensitizations, and colophony currently represents the 4th most frequent allergen in Germany. Therefore, highly potent allergens such as colophony should be strictly avoided as a content material of modern wound dressings.
Allergens↗
[Childhood acne. Clinical expression, etiology, and relationship to juvenile acne].
Childhood acne has different clinical expressions which may be present from birth or manifest within the first weeks of life or after the third to sixth month of life. The condition may occur as a physiological phenomenon or may be pathological and require endocrinologic evaluation and treatment. It may be induced by drugs or ointments or due to intoxication. Severe courses with a tendency to scarring in childhood may be observed. Childhood acne may persist and develop into juvenile acne. It is likely that childhood acne may represent a risk factor for the development of severe acne in puberty.
Acne Vulgaris↗
[Role of MHC class I molecules in anti-tumoral mechanisms in human malignant melanoma].
Malignant melanoma is still the most frequent cause of death due to skin cancer with a rising incidence and mortality. Despite continued progress in understanding the pathophysiology of tumor progression and metastasis, curative therapeutic options are still missing for metastatic melanoma. The ability of a malignant melanoma to metastasize is partially derived from the capacity to avoid destruction by an intact immune system. Thus, a better understanding of the immunological processes that lead to the escape of melanoma cells from immune recognition could help to develop preventive strategies or effective new therapies. Therefore, an analysis of the MHC class I pathway and molecules involved in peptide loading of the MHC class I molecules could provide an important clue to future immune-based melanoma therapies, and might also help to select patients who could be expected to profit from T-cell-based immunotherapy. In this review article, we report on current data and concepts about the generation of MHC class I peptide complexes in human malignant melanoma.
Histocompatibility Antigens Class I↗
[Contact allergies in patients with chronic wounds: results of a study from 1999 to 2004].
Patients with chronic wounds frequently acquire clinically relevant contact sensitization. We examined the results of the skin patch tests in patients with chronic wounds, who were treated between 1999-2004 in the Department of Dermatology, University School of Medicine, Essen, Germany. Altogether 105 patients with chronic wounds underwent patch testing. In 68 individuals, contact sensitization to at least one substance was detected. The most frequent contact allergens were to balsam of Peru, Amerchol L-101, fragrance mix, wool wax alcohols and rosins. Contact sensitization to wound dressing materials was also identified in 4 patients. Our results demonstrate the current spectrum of contact sensitization of patients with chronic wounds. Our results show the importance of paying attention to the ingredients in wound dressings and performing patch testing if there is any clinical suggestion of allergic contact dermatitis.
Adult↗
[Successful therapy of a rheumatoid leg ulcer with intravenous immunoglobulins].
Rheumatoid arthritis (RA) is a chronic, systemic inflammatory disease. Leg ulcers in rheumatoid arthritis may be caused by vasculitis and are an interdisciplinary therapeutic challenge. A 69 year old women with rheumatoid arthritis and many other medical problems presented with widespread vasculitis-induced therapy-resistant ulceration on her right lower leg. Since previous therapeutic efforts had a negative effect on wound healing, we administered intravenous immunoglobulins which led to complete healing of the ulcer was achieved. Both the serological and rheumatologic features of her rheumatoid arthritis also improved. Intravenous immunoglobulins represented an effective therapeutic option with fewer side effects in the therapy of vasculitis-induced leg ulceration in this patient with rheumatoid arthritis.
Aged↗
[Ulcerated plaques and papules limited to one leg].
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Collagen Diseases↗
[Non-surgical therapy of basal cell carcinoma of the head-neck region].
Basal cell carcinoma is a semi-malignant epithelial skin tumour with a locally destructive growth pattern but a very low tendency to metastases. Basal cell carcinoma represent the most frequent malignant cutaneous tumour worldwide in white population with a lifetime risk of 30 % and still increasing incidence. Lifetime cumulative UV-exposure, as well as frequent sunburns are the most common exogeneous risk factors, others are ionising radiation, exposure to chemical carcinogens, genetic predisposition, and chronic immunosuppression. Basal cell carcinoma are predominantly located in the central region of the face. The characteristic clinical feature is a single nodule with multiple teleangiectasia, as well as an irregular shaped ulceration with and elevated border. The very rarely observed occurrence of metastases seems to be a result of insufficient therapy or a metatypic basal cell carcinoma type. Therapy of first choice of basal cell carcinoma is the complete, micrographically controlled excision. In addition, radiotherapy, topical immunotherapy with Imiquimod or photodynamic therapy may be considered as therapeutic alternatives in selected cases.
Adjuvants, Immunologic↗
[Take of mesh grafts in chronic leg ulcer patients improves by vacuum-assisted closure device].
Vacuum-assisted closure (V.A.C.) is an established therapeutic option in the management of acute and chronic granulating wounds. In recent years, little data has been published concerning skin graft transplantation and postoperative V.A.C. therapy. We report a consecutive case series of 54 patients with chronic leg ulcer who received a total of 74 mesh grafts. A postoperative V.A.C. therapy was performed in 28 mesh grafts, and 46 mesh grafts were treated with standard gauze therapy. In the V.A.C. group, 92.9 % grafts showed complete healing. In the treatment group without postoperative V.A.C. therapy, 67.4 % of the mesh grafts had taken. Differential analysis revealed a correlation in patients over 70 years of age or in patients suffering from diabetes mellitus or dermato-lipo-sclerosis. Particularly patients with diabetes mellitus and of greater age exhibited improved take rates in the V.A.C. group. The results of our study demonstrate for the first time the significant benefit of V.A.C. therapy after mesh-graft-transplantation in chronic leg ulcer patients as evaluated in a clinical trial with a control group.
Aged↗
[3-dimensional documentation of wound-healing].
The objective evaluation of the course of wound-healing represents a substantial parameter for the quality assurance of a modern wound management in chronic wounds. Established procedures exclusively based on a two-dimensional measurement of the wound surface with planimetry or digital photo documentation in combination with a metric statement of size. Thus so far an objective method is missing for the evaluation of the volumes of chronic wounds. By the linkage of digital photography, optical grid by means of digital scanner and an image processing software in co-operation with the company RSI we were able to do an accurate 3-dimensional documentation of chronic wounds (DigiSkin). The generated scatter-plots allow a visual, computer-assisted 3-dimensional measurement and documentation of chronic wounds. In comparison with available systems it is now possible for the first time to objectify the volume changes of a chronic wound. On the basis of a case report of a female patient with an venous leg ulcer, which has been treated with a vacuum closure therapy before and after performing a mesh-graft transplantation, we would like to describe the advantages and the resulting scientific use of this new, objective wound documentation system in the clinical employment.
Combined Modality Therapy↗