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Biomedical subjects

G Bonsmann

Publications and source records attributed to G Bonsmann.

44 records · Page 3Linked to original sources

[Annular elastolytic giant cell granuloma--differential diagnosis of cutaneous granulomatosis].

Annular elastolytic giant cell granuloma (AEGCG) is a rare granulomatous skin disease characterized by phagocytosis of elastic fibres by multinucleated giant cells. A 46-year-old female patient developed multiple annular lesions with elevated borders and central atrophy on the limbs. Histological examination revealed the absence of elastic material in the granulomatous infiltrate and fragments of elastic fibrils in the cytoplasm of the multinucleated giant cells. Immunohistological investigation revealed that the ratio of CD4+ to CD8+ T-lymphocytes was 3:1. Our patient was treated with clofazimine orally, 200 mg daily for the first 10 days, then 100 mg daily. After 12 weeks a definite improvement was achieved. Our case demonstrates that AEGCG, which is mostly localized on head and neck, has to be taken into account in the differential diagnosis of generalized cutaneous granulomatous disease.

Biopsy↗

[Squamous epithelial carcinoma at the site of lichen ruber hypertrophicus of the glans penis].

Reports of cancer development in lichen planus mostly concern the oral erosive form. The reported frequency of malignant transformation ranges between less than 1% and 10%. There have been a few isolated observations of malignant transformation of cutaneous lichen planus. In these cases, the hypertrophic type located on the lower limbs predominated. We report an unusual case of squamous cell carcinoma developing on the glans penis in the presence of hypertrophic lichen planus. Systemic treatment of the lichen planus with dapsone, retinoids, corticosteroids and topical cyclosporin A failed to prevent this development.

Carcinoma, Squamous Cell↗

Antigens of the major histocompatibility complex in patients with chronic discoid lupus erythematosus.

The frequencies of the major histocompatibility complex class I, class II and class III antigens were determined in 130 patients (88 women and 42 men) with chronic discoid lupus erythematosus, and compared with those of 764 healthy controls. A significant increase in HLA-B7 (38.0% in the patients vs. 25.8% in the control group), HLA-B8 (29.5% vs. 17.4%), HLA-Cw7 (58.9% vs. 26.1%), HLA-DR2 (46.9% vs. 29.7%), HLA-DR3 (32.0% vs. 19.4%), HLA-DQw1 (76.6% vs. 60.5%), and a decrease in HLA-A2 (41.9% vs. 55.7%) was found. The calculated relative risk values for the respective antigens markedly increased when two or more antigens were present in one patient, with a maximum relative risk value of 7.4 for the combinations of HLA-Cw7, DR3, DQw1 and HLA-B7, Cw7 and DR3, which were found in 17.2% of the patients and in only 2.3% of the controls.

Chronic Disease↗

Histiocytosis X in an elderly patient. Ultrastructure and immunocytochemistry after PUVA photochemotherapy.

The clinical features of a 76-year-old man with histiocytosis X of the Letterer-Siwe type, with extensive skin involvement, are described. The patient's lesions responded dramatically to PUVA-photochemotherapy. Light microscopic, immunocytochemical and immunoelectron microscopic findings, before and after PUVA, are reported. Birbeck granules in phagolysosomes of dermal macrophages indicated uptake of destroyed HX cells. Residual HX cells in the skin and HX cells in a recurring lesion expressed the same membrane antigens as in the primary lesions.

Aged↗

[Behçet disease with inner ear involvement].

Behçet's disease may also insolve the inner ear. We report on a 24-year-old man having suffered from defective hearing already since two years after onset of Behçet's disease. During the follow-up period, vertiginous attacks, tinnitus, as well as feeling of pressure in both ears have been additionally observed.

Adult↗

The Tay syndrome (congenital ichthyosis with trichothiodystrophy).

We report a 5-year-old boy affected with the Tay syndrome, and give a review of 12 pertinent cases previously reported under various designations. The Tay syndrome is a distinct type of congenital ichthyosis characterized by a peculiar anomaly of hair growth which has been termed trichothiodystrophy. The hair shafts are extremely brittle, and they show alternating light and dark banding when examined microscopically between polarizing filters. Other features of this syndrome are low birth weight, short stature, mental retardation, delayed neuromuscular development and other CNS anomalies, dysplasia of nails, hypoplasia of subcutaneous fatty tissue, prematurely aged facial appearance, hypogonadism, cataracts, osteosclerosis, dysphonia, and increased susceptibility to infections. The syndrome is inherited as an autosomal recessive trait. We delineate the criteria for distinguishing this gene defect from other types of congenital ichthyosis associated with disturbed hair growth, as well as from other types of trichothiodystrophy which are not associated with ichthyosis.

Growth Disorders↗

Thalidomide in the treatment of sixty cases of chronic discoid lupus erythematosus.

The therapeutic effect of thalidomide in chronic discoid lupus erythematosus (CDLE) was studied in sixty patients who were followed up for 2 years. In fifty-four patients (90%) a complete or marked regression of the disease was observed, but when the thalidomide was stopped, thirty out of forty-one (71%) patients relapsed. Patients undergoing a second course of thalidomide treatment again responded well. Nine of the patients in whom the disease recurred after successful treatment with thalidomide and who had been unresponsive to intermittent treatment with antimalarials, showed a good response to a second or third course with thalidomide. Mild side-effects were common and 25% of patients complained of slight to moderate polyneuritic symptoms. Since electroneurological examinations had not been performed before the thalidomide therapy, the frequency of neurological side-effects cannot be accurately calculated but we recommend neurological examinations before and periodically during thalidomide treatment. Thalidomide is a very effective drug in CDLE, but in most cases it exerts its effect only whilst treatment is continued. Its use should be restricted to patients resistant to topical steroids and systemic antimalarials.

Chronic Disease↗