Differential diagnosis of bullous pemphigoid and epidermolysis bullosa acquisita.
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Biomedical subjects
Publications and source records attributed to P Duschet.
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Administration of etretinate in a 29-year-old female patient suffering from severe pustular psoriasis caused a dramatic increase in liver enzymes. Liver biopsy revealed changes characteristic for drug-induced hepatitis. After normalization of liver parameters following withdrawal of etretinate, isotretinoin was administered during a severe pustular relapse. In contrast to etretinate, isotretinoin was well tolerated and resulted in a good therapeutic response. Thus, isotretinoin can be considered as an effective and safe therapeutic alternative for pustular psoriasis even after the occurrence of etretinate-induced hepatitis.
Elastofibroma dorsi is a rare, benign tumor in elderly persons that usually occurs in the subscapular region. It represents a degenerative pseudoneoplastic process that resembles a malignant neoplasm. We report a patient with an elastofibroma dorsi of unusual size that ulcerated. This is a rare if not unique finding in elastofibroma dorsi.
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Apocrine chromhidrosis is an extremely rare and impressive skin disorder. Primarily after emotional stress patients complain of coloured sweat secretion (black, green, blue or yellow). The deposition of lipofuscin in the apocrine glands may be the cause of this disease, the different colours of sweat being due to various oxidation stages of lipofuscin. We report two patients, one with blue and one with green axillae; the clinical features, histopathology, differential diagnosis, and pathogenesis of apocrine chromhidrosis are discussed.
Plasmapheresis represents a new and powerful treatment for solar urticaria and has been performed in a couple of patients so far. Since therapeutic responses have been observed exclusively in cases exhibiting a pathogenetically important serum factor, plasmapheresis seems to work specifically by elimination of this photoallergen. It is easy to demonstrate a serum factor by in vitro irradiation of the patient's serum and subsequent autologous reinjection into the skin, inducing the generation of wheals. In contrast to transfer tests, this method does not bear the risk of HIV infection. In view of the extreme difficulty in administering conventional therapy for solar urticaria, the present encouraging results suggest that plasmapheresis should be considered in patients who show a serum factor.
Systemic mastocytosis associated with urticaria pigmentosa seems to be strikingly more common than previously assumed. The diagnosis can be established by the investigation of bone marrow sections, whereas bone marrow smears are less reliable. Some mediators are produced by the enhanced number of mast cells; telemethyl imidazole acetic acid is the most suitable mediator to calculate the size of the mast cell pool. Investigations like this might offer an alternative to the examination of bone marrow sections in future.
Hypereosinophilic dermatitis represents a clinically distinct disorder in the spectrum of eosinophilic dermatoses. Its major clinical symptoms include pruriginous papular skin eruptions associated with blood eosinophilia. Histological examination reveals a diffuse, dense infiltration with eosinophils. We discuss the clinical picture, differential diagnosis, and therapy in the light of two cases.
Electron beam therapy applied to a lymph node metastasis from a squamous cell carcinoma was followed by the development of histologically and immunologically typical bullous pemphigoid, the lesions being initially strictly confined to the irradiation area. This observation suggests that the bullous pemphigoid antigen may be altered or unmasked by electron beam radiotherapy, leading subsequently to the production of autoantibodies. The disease in this case effectively responded to the administration of tetracycline and niacinamide, a therapeutic regimen described recently.
Sixty-four patients received systemic alpha-interferon (10 million units subcutaneously daily) and 63 received systemic acyclovir (5 mg/kg body weight intravenously thrice daily) in a randomized study of acute herpes zoster. Start of healing, complete healing, development of new skin lesions in the primarily affected and in other dermatomes, and degree and duration of pain were evaluated. Both drugs proved equally clinically efficient without statistically different findings between the two groups; herpes zoster neuralgia was not prevented by either interferon or acyclovir therapy. Minor clinical side effects occurred slightly more frequently during interferon treatment and included fever and nausea. Transient and moderate leukopenia was observed in nearly all patients in the interferon group.
A 53-year-old male patient who had suffered for several years from severe persistent light reaction possibly due to tribromsalan photosensitivity was treated with cyclosporin A after long-term low-dose administration of corticosteroids which had to be discontinued. PUVA therapy was impracticable due to the extraordinarily high UVA sensitivity. When cyclosporin A blood concentrations between 100 and 200 ng/ml were reached, the patient was nearly free from symptoms; the excellent clinical response was also documented by phototesting performed prior to and during therapy. Cyclosporin A may be a valuable therapeutic alternative to systemic corticosteroids for severe cases of persistent light reaction which cannot be controlled by photoprotective measures.
Lichen planus/lupus erythematosus/overlap syndrome (OS) comprises those dermatoses which show the clinical, histologic, and immunopathologic characteristics of both diseases. On account of this heterogeneity, the diagnosis of OS may be difficult. About 35 cases have been reported on in the literature so far. We are going to discuss the clinical, histologic, and immunofluorescence findings in OS in detail.
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A 34-year-old female patient presented with the classical features of keratosis lichenoides chronica, a very rare dermatosis. Typical hyperkeratotic papules in a linear and reticular pattern were located on the upper extremities and the trunk. The nails and the perionychial areas showed marked vegetating lesions. After administration of a combined retinoid-PUVA therapy impressive remission was observed. The clinical picture, the differential diagnosis, in particular from lichen ruber planus, and the therapeutic aspects are discussed.
A patient with the typical features of Dowling-Degos disease (reticulate pigmented anomaly of the flexures) is described; the clinical picture and differential diagnosis are discussed. Systemic retinoids were used for treatment, but no essential improvement could be observed.
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A patient with figurated, erythematous skin lesions which histologically showed eosinophilic spongiosis and an epithelial intercellular staining with IgG, but no acantholysis, developed erythromelanoderma. This case and a review of the pertinent literature indicate that the diagnosis of pemphigus does not seem to be justified even in the presence of eosinophilic spongiosis and epidermal intercellular antibodies, when the clinical signs of this disease and the typical histological feature, i.e., acantholysis, are lacking.