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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.

To emphasize the importance of early diagnosis in cases of müllerian adenosarcoma that appeared as benign-looking cervical polyps.We examined seven cases of müllerian adenosarcoma of the uterus in patients 14-63 years of age (median 39 years). Tissue protruding from the external os and an initial diagnosis of a cervical polyp were common findings for all patients. On repeated examination, all lesions were interpreted as müllerian adenosarcomas.Histologic examination demonstrated benign glands with a sarcomatous stroma, which typically formed periglandular cuffs of increased cellularity. The sarcomatous stroma was homologous in four cases and contained heterologous elements such as striated muscle, lipoblast, and cartilage in three cases; one patient had a sarcomatous overgrowth of stromal elements. The question of a müllerian adenofibroma versus adenosarcoma was raised in three cases with the general appearance of slit-like glands surrounded by a stroma with fibrosis and a low mitotic rate. Using the criteria of stromal cellularity--marked stromal atypia and a mitotic index of two figures per ten high-power fields--the cases were classified as adenosarcomas. The sarcomatous overgrowth, the presence of heterologous elements, and a high mitotic rate seem to be important prognostically.Gynecologists and pathologists should be aware of the difficulties and delay in the diagnosis of müllerian adenosarcoma when the tumor presents as a benign-looking cervical polyp.

The fibrotic lateral rectus muscle and the medial rectus muscle of 4 patients with esotropia and myopia who underwent surgical correction were studied ultrastructurally. All patients revealed underactivity of the lateral rectus muscle. In 3 patients the underactive lateral rectus was completely fibrotic; in 1 patient there was 80% fibrosis, and the rest of the muscle showed different stages of degeneration. The fibrotic lateral recti were composed of collagen fibrils and remnants of degenerated organelles. The collagen fibrils of the lateral recti were of different sizes, and the mean diameter varied from 65 to 92 nm. On the other hand, the interstitial collagen fibrils of nonfibrotic medial rectus muscles of these patients and also of control muscles showed less variation in size, and the mean diameter was smaller as compared with the fibrotic lateral rectus. It is suggested that the collagen fibrils that compose the fibrotic lateral rectus muscle probably develop under conditions which differ from the normal development of collagen fibrils.

Fechtner syndrome, a variant of Alport syndrome, was first reported by Peterson et al. [1985]. It is characterized by nephritis, hearing loss, eye abnormalities, macrothrombocytopenia, and leucocyte inclusions, present in varying combinations in several members of the same family. This is the second family reported; 16 relatives are affected. The clinical manifestations of the syndrome are delineated. The pattern of inheritance is autosomal dominant. The hematologic abnormalities are similar to those detected in May Hegglin anomaly. They are present in every affected relative and may be present at birth. The feasibility of prenatal diagnosis is discussed.

SUMMARY The mechanism of skin hyperreactivity (pathergy) in Behcet'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 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.

10 of 16 (62.5%) patients with granuloma annulare presented microangiopathic changes in the skin. The incidence in control groups was 100% in diabetic patients, 40% in old-age patients not known as diabetics and 0 in young victims of road accidents. The frequency of the vascular findings does not seem to be shared equally by the two types of granuloma annulare, the generalized and the localized type. In the first, as in the diabetic group, microangiopathy was observed in all patients examined. Whereas, in the second it was detected in only 2 of the 8 patients. It is concluded, therefore, that from the point of view of their relation to a diabetic state, the two forms of the disorder differ from each other.

A case of a newborn child with extensive congenital absence of epidermis is presented. Following our review of the literature, this appears to be the most extensive case described. Some possible etiological causes of this rare disease are briefly discussed.

While immigrants from Morocco and Romania make up 7 and 6%, respectively, of the Israeli population, one-third of all postnecrotic cirrhosis patients come from Morocco and another third from Romania. The rate of hepatitis B virus (HBV) infection, as expressed by serum hepatitis B surface antigen, anti-HBs, and anti-HBc, was compared in immigrant patients with postnecrotic cirrhosis and in age and sex matched controls from the same countries. Among 19 Moroccan immigrants with postnecrotic cirrhosis 74% showed evidence of HBV infection. This was not significantly different from the prevalence of HBV infection (64%) in a matched control group of 61 Moroccan individuals. In the 26 Romanian cirrhotics, however, a significantly higher prevalance of HBV infection than in the 60 matched controls was observed (54 and 23%, respectively, p less than 0.01). The difference was detected only when anti-HBc was looked for in addition to hepatitis B surface antigen and anti-HBs. These results indicate that: 1) HBV infection and postnecrotic cirrhosis are associated in Romanian immigrants to Israel. 2) Association between HBV infection and cirrhosis in Moroccan immigrants cannot be shown because of the high infection rate in the control population. 3) Anti-HBc is often the only marker of HBV infection in cirrhotic patients, and should be examined in addition to hepatitis B surface antigen and anti-HBs in order to get meaningful results.

A rare case of malignant melanoma metastasizing to the kidney is described. Five other cases were found in the English literature, this being the sixth. A review of the subject is brought up.

Light microscopy findings in early Wilson's disease (hepatolenticular degeneration) may be normal even when special cytochemical stains are used. We present a case of Wilson's disease in which light microscopy was negative, while electron microscopy showed the characteristic changes in the hepatocytes. Since low serum ceruloplasmin levels and high urinary copper excretion are not by themselves definite proof of Wilson's disease, and since copper content of the liver is not universally measured, electron microscopy examination of liver tissue appears to be a worthwhile additional tool for the early diagnosis of Wilson's disease.

Distant bone metastases in cases of intracranial meningiomas are uncommon. Two patients with meningiomas showing malignant histological features are described, in both of whom bone metastases were observed many years after cranial surgery.

Two patients in whom various immunological phenomena obscured and delayed the diagnosis of subacute bacterial endocarditis (SBE) are described. Immunological abnormalities usually correlate well with the chronicity and severity of the disease, as is demonstrated here. The mechanisms underlying the various immunological phenomena are discussed. The importance of alertness to the possibility of SBE in cases of disease manifesting itself as an immunological disorder, thus achieving early diagnosis and early antimicrobial treatment, is stressed.

A case of immunoblastic lymphadenopathy and systemic amyloidosis in a 21-year-old man is presented. The basic defect in immunoblastic lymphadenopathy is believed to be an abnormal immune reaction, most probably of the B cell system. Although systemic amyloidosis is common in some disorders of the B cell system, no cases of systemic amyloidosis complicating immunoblastic lymphadenopathy have been previously reported. Immunoblastic lymphadenopathy includes elements known to be associated with amyloidosis and we believe that this association is not merely coincidental.

An unusual case of a müllerian adenosarcoma of the peritoneal mesothelium in a 32-year-old pregnant woman is described. On emergency laparotomy, a tumor was found in the pelvis connected with the right broad ligament only, and was removed. Light and electron microscopic examination showed a rare example of an extrauterine müllerian adenosarcoma composed of a stromal sarcoma, muscle tissue resembling primitive myoblasts, and sex-cord-like tubular lumens. The patient was followed, and after 22 months the primary surgery was reexplored. On laparotomy, no evidence of disease was found, and the ovaries were tumor-free. Histologic examination of the pelvic peritoneum demonstrated small areas of adenosarcoma resembling elements of the previous tumor. Müllerian adenosarcoma is a rare tumor of müllerian origin, and its less aggressive behavior as compared with mixed müllerian sarcoma is warranted. The better prognostic outlook of müllerian adenosarcoma is probably related to the histologic structure, but depends in addition on the size of the tumor and the site of origin. No case of peritoneal origin of müllerian adenosarcoma has been previously reported; we discuss this unusual location.