S. Haim

Histological and direct immunofluorescence study of cutaneous hyperreactivity in Behçet's disease

The mechanism of skin hyperreactivity (pathergy) in Behcets disease is unknown. It has been suggested that the response is due to an Arthus‐like reaction. In 18 patients with active Behçets disease, pathergy was induced and the skin biopsied after 24 h. The histological reaction was that of mild 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 hyperreactivity.

Polymorphonuclear leucocyte function in Behçet's disease.

Polymorphonuclear leucocyte function was investigated in 19 patients with active Behçets disease. Spontaneous free leucocyte migration was found to be significantly reduced, yet after stimulation the leucocytes chemotactic activity was considerably increase (p less than 0-05) when compared to control leucocytes. Control leucocytes migrated more rapidly when incubated in serum taken from patients with Behçets disease (p less than 0-005). The enhanced chemotactic activity in Bechçets disease appears to be due to both serum and intrinsic leucocyte factors. Spontaneous nitroblue tetrazolium reduction was found to be normal, although after stimulation leucocyte nitroblue tetrazolium reduction was lower than in the control group (P less than 0-025), as was leucocyte oxygen utilisation. It is suggested that the hyperreactive cellular inflammatory response that characterises Behçets disease may be due to increased chemotactic activity and minor alterations in functional metabolic activity of leucocytes.

The quantification and significance of mast cells in lesions of Behçet's disease.

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 Behçets 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 Behçets 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 Behçets disease.

Muscle Relaxing Agent in Cutaneous Leiomyoma

The management of pain in cutaneous leiomyoma involving large areas of the body surface is problematic. The surgical approach is not practical and the pain in these cases is refractory almost to all analgesic agents. This is a description of a case in which a muscle relaxing agent proved to be the only effective therapeutic approach.

Evaluation of Direct Microscopic Examination Versus Culture in the Diagnosis of Superficial Fungal Infections

Summary: Five hundred eighty six samples of skin and nail scrapings or hair clinically suspected of superficial fungal infection were studied by direct smears and cultures. 366 (62.5%) of these samples were negative by both methods. Of the remaining 220 (37.5%) samples, 114 (51.8%) were positive by the direct KOH preparation, 6 (2.7%) by culture and 100 (45.5%) by both methods. Most (72.7%) of the 214 (97.3%) KOH positive smears showed a low to high hyphal content, and 4 (1.9%) of them were culturally negative although they possessed a very high amount of hyphae. Discrepancies between the direct microscopic examination and the subsequent culture are well known in the literature and therefore this study strongly suggests the necessity of both tests for the diagnosis of superficial mycoses.

Comparative Ultrastructural Study of Mast Cells in Mastocytoma and Mastocytosis

The ultrastructural aspects of mast cells from skin lesions in 2 patients with mastocytoma were compared to those from lesions in 4 patients with mastocytosis, 3 with cutaneous and 1 with systemic involvement. In mastocytoma, the mast cells accumulated in a large mass. They revealed short cytoplasmic villi and were similar, morphologically, to mast cells of normal skin and gingiva. In the diffuse types of the disease, cutaneous and systemic, the cells were arranged in small groups. In addition to normal mast cells, there were also irregular or bizarre-shaped cells presenting long and twisted cytoplasmic protrusions. In the case with the systemic involvement and in 2 of 3 cases with apparently cutaneous diffuse lesions, the villi of adjacent cells interlaced and showed a tendency to form mast cell aggregates. The pathognomonic value of the above observation seems worthy of further investigations.

Mast cells in Behçet's disease: ultrastructural and histamine content studies.

In order to assess the role of mast cells in the pathogenesis of mucocutaneous lesions of Behçets disease, the following investigations were performed: (1) a comparative study of ultrastructural features of mast cells in spontaneous and reactive (pathergic) lesions of the process and in apparently uninvolved skin of patients in the active stage of the disease; (2) comparative estimation of the histamine content at the site of cutaneous hyperreactivity lesions and in apparently uninvolved skin of the same patients. Various forms and degrees of degranulation were revealed among the mast cells. These forms were obviously more frequent in spontaneous and reactive lesions than in the uninvolved skin, 66 and 56% as compared with 7.7%. Also the histamine content was twofold more in the reactive lesions than in the unaffected skin of the same patient, 43 ng/mg tissue as compared with 21 ng/mg tissue. The results of our study seem suggestive of an active role of mast cells in this process. The role is probably similar to that of basophils in cutaneous basophilic hypersensitivity.

PLATELET FUNCTION IN BEHÇET'S DISEASE

The coagulopathy observed in Behçets disease has defied explanation. No pathology in concentration or function of coagulation parameters has been demonstrated. We have studied platelet adhesion function in 13 patients with established Behçets disease, 5 with venous thrombosis and 8 with no involvement of veins, as well as in 30 age and sex matched healthy controls. Platelets derived from 10 of the 13 Behçets patients exhibited significantly increased adhesion as compared to the controls (p<0.01). The platelets of 11 of the 13 tested patients showed significantly increased aggregation in response to adenosine diphosphate (ADP) and epinephrine incomparison to those from controls (p<0.01).

Primary Chancriform Syndrome Caused by Nocardia asteroides: Primäres schankriformes Syndrom durch Nocardia asteroides

Summary: This is a report of a case with Nocardial asteroides infection. The infection in this case is presented with cutaneous manifestations which are in keeping with the concept of primary cutaneous chancriform syndrome. To our knowledge, these forms of Nocardial infection are rare and the one which we are reporting may be the first to be reported from Israel.

Generalized Familial Benign Chronic Pemphigus

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.