Panniculitis in familial Mediterranean fever. Case report with histopathologic findings.

A 79-year-old Armenian-born woman with stable, long-term familial Mediterranean fever had progression of chronic renal failure concurrently with two types of skin lesions. One lesion resembled erysipelas, which is quite common in familial Mediterranean fever, whereas the other was panniculitis, only occasionally described in familial Mediterranean fever. The unique histopathologic features of the latter are presented. The onset of acute cutaneous disease in this patient coincided with worsening renal disease and preceded a flare of disease activity in other sites after a 14-year period of quiescence. The severe and unremitting pain from the skin lesions and their tendency to form nonhealing ulcers were substantial causes of morbidity in this patient with familial Mediterranean fever.

Surgical pathology of hyperparathyroidism. Usefulness of fat stain and problems in interpretation.

Thirty-six adult patients with classical hyperparathyroidism had parathyroidectomy in which tissue evaluation included oil red O stains. Neutral lipid staining has been reported to distinguish hyperfunctioning parathyroid from suppressed or normal tissue. In normal glands, parathyroid chief cells show abundant coarse and fine intracytoplasmic neutral lipid droplets. In contrast, the cytoplasm of chief cells is essentially free of neutral lipid droplets in adenomatous, hyperplastic, or carcinomatous glands. This study shows that to limit or alter surgical exploration solely on the basis of the findings of the oil red O technique will lead to significant judgmental error (6/36 patients). Such errors are especially likely in the patient who has enlarged parathyroid glands containing a normal distribution of parenchymal to adipose tissue. There was excellent agreement between clinical outcome, surgical-pathological findings, and the oil red O stain in 30 cases of parathyroid adenoma, nodular hyperplasia, and a control group (12 patients) who had biopsies of normal parathyroid tissue during the course of thyroidectomy.

Pulmonary artery-bronchial fistula complicating chronic lymphocytic leukemia.

A 52-year-old man with chronic lymphocytic leukemia (CLL) entered the hospital because of repetitive bouts of massive hemoptysis requiring a right pneumonectomy. A bronchus intermedius-pulmonary artery fistula was found and felt to be due to necrosis of a peribronchial lymph node which was infiltrated by chronic lymphocytic leukemia cells. This represents another pulmonary complication in the clinical course of advanced CLL.

An experimental model of pulmonary arterial granulomatous inflammation.

An experimental method to investigate pulmonary granuloma formation in the guinea pig was established. Animals sensitized to human serum albumin (HSA) and challenged intravenously with HSA covalently linked to Sepharose 2B beads developed a specific granulomatous response. This intense pulmonary arterial, focally necrotizing, but mainly granulomatous inflammatory reaction developed 5 to 7 days after the administration of the HSA-bead conjugate. Unsensitized animals did not show such extensive inflammation, but rather exhibited a typical foreign body reaction to the bead. In both the experimental and control groups, the inflammatory response was indistinguishable at 14 and 21 days. Thus the initial acute inflammatory reaction was seen only in specifically sensitized animals. On the other hand, HSA alone produced no demonstrable inflammatory lesion. These observations suggest that locally retained antigen may trigger antigen-specific T (thymus-dependent) lymphocytes to release mediators which contribute to granuloma formation.

The "disappearance" of residual basal cell carcinoma of the skin.

Three patients with histological evidence of incompletely removed basal cell carcinoma underwent reexcision; no residual tumor was found. Other reports of this phenomenon are discussed and some speculative explanations are offered.

The validity of Pap smear parameters as predictors of endometrial pathology in menopausal women.

Cytopathologists recognize that certain Pap smear findings are suspicious for endometrial pathology in menopausal women. To study their prognostic importance in the directed cervical smear, six parameters were used to evaluate smears for evidence of an endometrial lesion: the presence of (1) histiocytes, (2) multinucleated histiocytes, (3) nonspecific inflammation, (4) bleeding, (5) elevated squamous cell maturation index, and (6) the degree of cytologic atypicality of endometrial glandular cells, expressed as a "score" from 0 to 6. Clinical pathologic correlation of 102 women with these parameters was undertaken. A multivariable statistical analysis determined which of these six parameters was most predictive of an endometrial lesion. Cytologically "scored" endometrial glandular cells was the only parameter predictive of endometrial pathology. Its emergence as the sole prognostic cervical Pap smear parameter for endometrial pathology has not been previously reported. This predictive value was consistent even with control for bleeding history and age. Appropriate interpretation of abnormal Pap smear findings contributes substantial diagnostic information in the evaluation of the endometrium.

Albumin uptake by skin, skeletal muscle and lung in living and dying patients.

Quantitative changes in albumin and water content which occur in skin and muscle tissue obtained by biopsy from clean surgical incisions at the time of skin incision and at the time of wound closure were assessed in seven patients who had major abdominal and vascular operations. Biopsies from skin, muscle and pulmonary tissue were obtained in a second group of nine patients who had thoracotomy for suspected bronchogenic carcinoma. The intravascular albumin mass decreased linearly with the duration of the operation (r = 0.69, p <0.01) and was correlated significantly with a linear increase of extravascular albumin content in muscle (r = 0.63, p < 0.01). A significant increase of extravascular albumin occurred from the time of incision to the time of wound closure in the skin and muscle samples from both groups (p < 0.05-p < 0.001). Extravascular albumin and water content were assessed in a third group of three postoperative patients who died with acute respiratory failure. Although the concentration of extravascular albumin in pulmonary tissue taken from this group was not different from the thoracotomy group, calculations based on the mean combined post mortem lung weights showed two to three times more extravascular albumin content. The histopathological findings are consistent with these changes. Thoracotomy was not associated with an abnormal increase in pulmonary extravascular albumin and water content, possibly because albumin does not gain access to the pulmonary extravascular space in increased amounts during operation in this clinical setting.

The long-term outcome of restorative operation in Crohn's disease: influence of location, prognostic factors and surgical guidelines.

The course of all 113 patients with Crohn's disease whose initial procedure involved an anastomosis operated upon from 1942 to 1972 was followed through 1980. The calculated cumulative 30-year total mortality was 23.4%, 16.7% disease-related. The cumulative recurrence rate was 29% at five years, 52% at ten years, 64% at 15 years and 84% at 25 years, with no important differences between disease locations and types of operation. Sex, age, duration, granulomas, enteral or perirectal fistulas and length of the resection, the disease, and the proximal resection margin had no significant influence on the rates of development of recurrent disease or on functional outcome. By far the most common site of recurrence was the neo-terminal ileum, but in ileocolitis compared with ileitis, recurrence was 5.2 times more likely (p = 0.0001) to involve the adjacent or remote colon as well. Moreover, only 1/63 ileitis patients eventually required ileostomy, whereas 15/47 patients with ileocolitis or colitis ultimately required this procedure (p less than 0.001). The current status of the patients was excellent or good in 64% and unwell or dead related in 24%. Urolithiasis developed in 19%.