Search

Aim Familial cases of Kaposi's sarcoma (KS) are rare, and have all been described in patients with the classical variant of the disease. The predisposition of classical Kaposi's sarcoma among Jews is well known. We herein describe five families, all Jews, in which two members have Kaposi's sarcoma. To our knowledge, this has been the largest reported series of familial Kaposi's sarcoma. Patients and methods The clinical course, management and response to therapy were described and compared with other published cases. Results No similarity was found in any of the families in time and age of onset of the disease, or in the severity and course of the disease among the members of the same family. There was a high incidence of second neoplasms among these familial cases. Conclusions We discuss the potential implications of second neoplasms based on our understanding of the pathogenesis of the disease, as well as the influence or predisposal of some genetic mechanisms to the development of Kaposi's sarcoma.

Livedo reticularis is associated with collagen vascular diseases and other vaso-occlusive disorders in a substantial number of cases. In the remaining cases the cause of livedo reticularis is still unknown. (i.e., idiopathic). We sought to determine a possible causal relationship between idiopathic livedo reticularis and autoimmune factors associated with the coagulation system, including antiendothelial cell antibodies. Nine patients with idiopathic livedo reticularis were studied. All patients were found to have normal platelet count, fibrinogen levels, and prothrombin and activated partial thromboplastin times, as well as negative results for Venereal Disease Research Laboratory and D-timer tests. Anticoagulant activity was detected in 2 patients: one had positive results of thromboplastin titration index and Russell's viper venom test, as well as increased levels of anticardiolipin antibodies and anti-endothelial cell antibodies; the other has positive thromboplastin titration index, mildly increased levels of anti-endothelial cell antibodies, and markedly increased levels of antinuclear antibodies. A third patient had mildly increased levels of anti-endothelial cell antibodies alone, and a fourth patient had mildly increased levels of antinuclear antibodies only. The clinical outcome was uneventful in all of the patients during an 18-month follow-up period. These findings suggest involvement of autoimmune factors associated with the coagulation system in some patients with idiopathic livedo reticularis, whose clinical significance remains to be determined.

The lipid compositions of 8 normolipidemic xanthelasma palpebrarum (XP) lesions were analyzed using thin-layer chromatography, with the adjacent uninvolved skin used as control. The lesions were found to be composed predominantly of cholesterol, mostly cholesteryl ester, whereas in the control specimens phospholipids predominated. The degradation rates of 125I-low-density lipoprotein (LDL), oxidized LDL, and acetyl LDL, and the rates of intracellular cholesterol synthesis from 1,2-(14)C-acetate, in blood monocyte-derived macrophages (MDM) from 3 normolipidemic patients, were similar to those of MDM from 3 normal control subjects. The mean levels of lipid peroxides and conjugated dienes under basal conditions, as well as following the addition of a free radical-generating compound (2,2-azobis-2-amidinopropane hydrochloride) to the plasma of 14 normolipidemic XP patients were significantly higher than those of 14 age- and sex-matched normal controls. We conclude that the predominant lipid accumulated in normolipidemic XP lesions is cholesteryl ester, but there is no evidence for intrinsic cellular cholesterol metabolism derangement in blood MDM from patients which could account for this. Since macrophage cholesterol accumulation can also result from enhanced uptake of increased levels of oxidized LDL, the increased plasma lipid peroxidation (derived from oxidized LDL) might lead to accumulation of cholesterol in macrophages and formation of foam cells via this mechanism.

The histological findings in the first diagnostic biopsy of 50 patients with Kaposi's sarcoma (KS) were classified and were related to the clinical course. Forty-one patients had the "classic KS," of whom 31 had a benign course and 10 an aggressive disease. Nine patients had iatrogenically induced KS. Histologically the following subtypes were found: mixed type in 18 patients, spindle type in 16, early type in 14, and hemangiomatous and lymphangiomatous types in one each. We did not find any statistical relationship between the histological subtypes, degree of nuclear atypia, number of mitoses, and density of the inflammatory cell infiltrates in the KS lesions, and the clinical types and course of the disease. The results of this study do not support the possibility of a prognostic importance regarding the histological features of the early lesions in patients with classic or iatrogenic Kaposi's sarcoma.

Dermatofibrosarcoma protuberans is an uncommon skin tumor with a high potential for local recurrence if not adequately excised. There are only two reports in the literature describing dermatofibrosarcoma protuberans that enlarged considerably during pregnancy. On the other hand, the new development or enlargement of dermatofibromas in pregnancy is well documented. We present 2 additional patients in whom a dermatofibrosarcoma protuberans appeared and grew rapidly during pregnancy. Immunohistochemical studies were negative for estrogen and progesterone receptors. The possible pathophysiology and surgical management are presented and discussed.

The rate of success in treating onychomycosis with 40% urea ointment containing 1% bifonazole, with and without oral griseofulvin, was evaluated. Most patients had onychomycosis of the toes. 11 patients (group A: 5 men and 6 women, average age 48.5) were treated locally under occlusion with the ointment until dissolution of most of the nail, and then with 1% bifonazole cream for a total of 6 months. 11 others (group B: 3 men and 8 women, average age 42.5) received, in addition, griseofulvin 500 mg/day orally during that period. Treatment with the ointment alone did not give higher cure rates than the average of reported cure rates for griseofulvin alone. However, the results of treatment with 40% urea and 1% bifonazole ointment concomitantly with oral griseofulvin seemed superior to those of either of these agents alone, with full cure, partial cure and failure in group A of 22.2, 33.3 and 44.5%, respectively, and in group B, 45.4, 27.3 and 27.3%. This therapeutic experiment should be repeated in larger series of patients.

Hyperreactivity of the skin to intracutaneous injection or needle prick (the pathergy test) is a unique feature of Behçet's disease. This phenomenon has been demonstrated to be highly sensitive and even specific in patients with Behçet's disease from Turkey, Japan, and Israel. A lack of positive reaction to this test has been reported in British and American patients. To evaluate the specificity of the pathergy test's results in Israeli patients with Behçet's disease, we compared their prevalence in these patients to that in a group of patients with other diseases associated with vasculitis. Forty-six Jewish and Arab Israeli patients with Behçet's disease, during a period of peak activity of their disease, and forty-six patients with other diseases associated with vasculitis, were studied for the hyperreactivity phenomenon. A positive reaction was observed in forty-five of forty-six patients with Behçet's disease, while the test gave negative results in all those with other diseases. Our study indicates, therefore, that the results of the pathergy test are highly sensitive and specific for Behçet's disease in Israeli patients.

Langerhans' cells were studied in the epidermis of two patients with active Behçet's disease and compared with those in two normal controls. Ultrastructural morphology and the percentage of Langerhans' cells found were similar in patients' (1.88%) and the control epidermis (1.79%). The density of Langerhans' cells in adjacent sites of the same epidermis was not homogeneous, being in the range of 0.8-2.8% in Behçet's disease and 0.6-4% in the controls. In the controls, Langerhans' cells were distributed unevenly. Some were located near the basal layer of the epidermis while the rest were in the mid and upper layers. In Behçet's disease most Langerhans' cells were in the mid-epidermis, but some were immediately beneath the stratum granulosum. In the Behçet's disease epidermis the area occupied by Langerhans' cells was increased by about 25% and the number of granules found increased by about 44%. It is suggested that in Behçet's disease the Langerhans' cells are in a more active state.

Two cases with circumscribed calcinosis of skin are described. One is an obvious case with scleroderma, whereas the second is apparently idiopathic. However, a striking clinical similarity is seen in both. This together with other noncontributory clinical and laboratory findings observed in the second case seem suggestive that idiopathic type of calcinosis may present a preceeding or presenting symptom of a subclinical underlying disease.

SUMMARY The carbohydrate tolerance was routinely investigated in fifty-two patients with granuloma annulare. Thirteen of the fifty-two cases presented the generalized type and the remaining thirty-nine had the localized form of the disorder. The results obtained in these patients were compared with those revealed in two control groups of patients. The same number of patients of the same sex, age and ethnic group with skin diseases other than granuloma annulare were similarly investigated as were 386 patients with conjunctival micro-aneurysms. Some degree of carbohydrate intolerance was detected in 36.5% of patients with both types of granuloma annulare. However, the incidence of this disturbance in patients with the localized form was no greater than in those with other skin diseases. The important finding in this study was the 76.9% incidence of carbohydrate intolerance in generalized granuloma annulare. This finding was supported by the histopathological changes in the small cutaneous blood vessels of these patients.

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.

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.

Leukocyte inhibitory factor (LIF) was investigated in 9 patients with localized granuloma annulare and 8 patients with the generalized form of the disease. The control group consisted of 10 matched, apparently healthy subjects. LIF values were significantly higher in patients with granuloma annulare of both groups than in controls (p less than 0.05 and 0.01). However, no significant difference was revealed between the two groups of patients. Though the results seem to speak for a cell-mediated immune response in granuloma annulare, they do not add a further support to the previously demonstrated differences between the two forms of granuloma annulare.

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.

One hundred patients with viral warts and 108 apparently healthy matched controls were typed for HLA-A and B antigens. No significant difference in the frequencies of antigens was revealed, which suggested that a possible genetic trend in warts is at least not related to A and B loci of the histocompatibility system.

Seborrheic keratosis (Leser-Trélat sign) and extensive florid papillomatosis of the skin and the mucous membranes of the nose, mouth, throat, and external genitalia were associated with adenocarcinoma of the breast in a 61-year-old woman.

Acquired ichthyosis is known to be associated with a variety of systemic diseases and to occur secondarily to the intake of some drugs. A 57-year-old woman with acquired ichthyosis and polycythemia rubra vera is described.

Erythema ab igne (EAI) has not been common since coal stoves have no longer been in general use for heating. Nevertheless, each winter several patients with macular-reticular skin lesions considered to be EAI are seen in our clinic. Correct diagnosis depends on awareness of the condition. 3 women, aged 17, 34 and 50, respectively, in whom EAI was induced by prolonged sitting close to an electrical heating device are presented. When this was discontinued the skin lesions disappeared. In the majority of reported cases of EAI the course was benign, similar to that in our patients in whom there were no additional related lesions. However, isolated cases of squamous cell carcinoma developing in patients with EAI have been described. In other cases the local heating which caused the EAI was applied for relief of pain and the appearance of EAI was the first clue to an underlying internal 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.

SUMMARY Thirth-five patients who received thirty-seven kidney transplants (two patients received live; the rest cadaver kidneys) were followed up for skin complications. There were thirty-nine instances of overt skin infection. In twenty-one instances we encountered fungal, in ten pyogenic and in eight viral infection. The course of these infections was more severe and more protracted than is usual in other patients. In nine instances, the skin infection contributed directly to the death of the patient. One patient developed Kaposi's sarcoma and another pyoderma gangrenosum. This study suggests that immunosuppressive treatment, which all our patients were continuously receiving, enhances their susceptibility to infection and to malignancy and may have been an aetiological factor in pyoderma gangrenosum.

Summary Clinical or chemical (latent) diabetes was discovered in 7 of 8 patients with generalized granuloma annulare; all were women. The average age of onset of their disease was 50 years. Treatment with chlorpropamide and low carbohydrate diet seemed to alleviate the skin manifestations in 6 of 7 patients so treated.