[New aspects in dermatitis herpetiformis].
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Biomedical subjects
Publications and source records attributed to R Friedman-Birnbaum.
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A patient with a cyclic premenstrual eruption, clearing spontaneously with the menstrual flow, is reported. Hypersensitivity to endogenous progesterone in this case was confirmed by positive intradermal and leukocyte migration inhibition tests. Treatment with conjugated estrogens was followed by remission of symptoms. The importance of awareness to the possibility of progesterone autoimmune mechanisms is emphasized.
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Mast cells in the upper dermis and mucosal subepithelial layer were counted in thirty consecutive light microscopy oil-immersion fields of the following: thirty-four skin and oral specimens from lesions in patients with active Behçet's disease, eight specimens of apparently uninvolved skin of the same patients, and 102 lesions of a variety of other skin diseases. The results revealed a significant increase in the number of mast cells in Behçet's lesions. Furthermore, the histamine content of five reactive lesions showed a two-fold increase compared with that in apparently uninvolved skin of patients with active Behçet's disease. It is suggested that mechanisms similar to cutaneous basophil hypersensitivity may be involved in the production of Behçet's lesions.
This is a report of generalized familial benign chronic pemphigus in a Negro male. The rich staphylococcal and streptococcal flora detected on the skin of this patient may have been a factor in the generalization of the process.
Eosinophilic fasciitis is an additional clinical entity to many others manifested by sclerodermic changes in the skin. A case with this process is reported. It presents vast cutaneous sclerodermic changes associated with eosinophilia, signs of muscular exertion, and a massive inflammatory infiltrate involving the lower part of the dermis, subcutaneous tissue and the muscle fascia. Prednisone therapy was followed by rapid and marked improvement.
This is a report on two cases with pemphigus vulgaris effectively controlled by dapsone. In one, dapsone was added to the treatment after a failure of combined prednisone and methotrexate therapy to control the disease completely. It is suggested that dapsone may offer a means in an attempt to minimize adverse reactions met with during the long corticosteroid regimen required in pemphigus.
This is a report of three cases of associated tuberculous and neoplastic processes. The first is a case of lymphosarcoma complicated by cutaneous tuberculosis. The remaining two are cases of cutaneous tuberculosis complicated by squamouse cell carcinoma. Due to clinical similarity, the diagnosis of the secondary process in these cases was evoked by its failure to respond to treatment of the primary disease.
Histocompatibility antigens (HL-A) were studied in a family with Behçet's disease (BD). All who had symptoms or signs of BD had either HL-A B5 or BW35, or both. The increased number of families with BD recently reported supports the suggestion that positive family history may assist the diagnosis of BD.
Thirteen patients with localized granuloma annulare and 19 with the generalized form of the disease were typed for HLA antigens. The control group consisted of 70 matched apparently healthy subjects. No significant difference in the frequencies of the 26 tested antigens was revealed between patients with localized granuloma annulare and controls. Patients with the generalized form of the disease showed a highly significant increase in the frequency of HLA-Bw35 as compared with both controls and patients with the localized form of the disorder (P = 0.001 and 0.01 respectively). The results seem suggestive of a possible different pathogenesis of the two forms of the disease.
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The mechanism of skin hyperreactivity (pathergy) in Behçet's disease is unknown. It has been suggested that the response is due to an Arthus-like reaction. In 18 patients with active Behcet's disease, pathergy was induced and the skin biopsied after 24 h. The histological reaction was that of mold to intense round cell infiltration. perivascular in type. Direct immunofluorescence failed in all 18 patients to demonstrate the local presence of immunoglobulins and complement at the site of lesion. A marked increase in the number of mast cells was observed at the site of reaction and scattered throughout the dermis, the role of which is to be further investigated. The study failed to demonstrate a humoral mechanism in the production of the cutaneous hyperactivity.
Two cases with pyoderma gangrenosum are presented. The course, in both cases, suggested that immunosuppressive therapy may play an etiological role in the disease. The first was a kidney recipient receiving prednisone and azathioprine and the second, a patient with pemphigus vulgaris who was treated with prednisone and methotrexate.
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