Biomedical subjects
Publications and source records attributed to J H Boss.
The authors report on a patient with an undescribed constellation of lymph nodal plasmacytosis, perinodal epithelioid cell granulomas and fibrosis as well as anti-Sm and anti-phospholipid antibodies. The illness does not meet the criteria of well-known nosologic entities, but it is thought to represent an unrecognized nosological entity within the group of undifferentiated immune disorders.
An elderly woman receiving long term treatment with prednisone and azathioprine for cryptogenic chronic active hepatitis developed granulomatous lipophagic panniculitis and temporal arteritis. The lymphoplasmahistiocytic inflammatory reaction pattern is common to this patient's three diseases. It is suggested that an aberration of the defence mechanisms, immunological or otherwise, is responsible for this unusual occurrence. The triple association of chronic active hepatitis, granulomatous panniculitis and temporal arteritis has not been reported previously.
The fasciitis-panniculitis syndrome is histologically characterized by fibrous thickening and inflammation of the subcutaneous septa, fascia and perimysium. These morphological features were the common denominator in 13 patients with Shulman's eosinophilic fasciitis, scleroderma profunda, venous lipodermatosclerosis, induration following recurrent erysipelas and erysipeloid erythema nodosum. Small to medium-sized vessel phlebitis was found in the subcutaneous and perimysial tissues of 11 of the patients and subcutaneous phlebosclerosis was encountered in all cases. These data contrast with the low incidence of phlebopathy reported by others in these disorders.
A solitary cervical metastasis of a typical carcinoid tumor was found in the subcutaneous tissue of an asymptomatic 38-yr-old woman. Investigations failed to disclose the primary site until the 5th yr, when she presented with carcinoid syndrome. Multifocal ileal carcinoid tumors were resected and debulking of abdominal metastases performed. Interferon and somatostatin analogue treatment resulted in remission. Solitary cervical metastasis is an exceedingly rare initial manifestation of a mid-gut carcinoid tumor, and poses a therapeutic dilemma. There are no directions in the literature as to whether a "wait-and-see" approach or exploration surgery is the preferred management when one is confronted by a cervical metastasis of typical carcinoid tumor of unknown primary site.
Massive and diffuse subcapsular fibrosis of the liver was detected in two patients with chronic liver disease. The subcapsular layer consisted of inflamed fibrous tissue containing rare portal tracts and myriads of bile ducts, indicating loss of subcapsular hepatocytes and their replacement by connective tissue. In the early evolutive phase, massive and diffuse subcapsular fibrosis of the liver appears on computed tomography (CT) scan as a low-density band, which merges with the hepatic parenchyma and is enhanced by contrast on dynamic imaging; it is undetectable by ultrasonography. In the advanced phase, it is easily recognizable by ultrasonography as a perihepatic sonolucent band and by CT scan as a low-density band, which is well demarcated from the hepatic parenchyma and is not enhanced with the dynamic technique.
Cortical Infantile Hyperostosis (Caffey's disease) is a benign and self-limited disorder. Late recurrence or persistence of symptoms with deformity seems exceedingly rare. Its occurrence in isolated cases or in multiple members in families suggests the existence of two different forms, namely a sporadic form and a familial form. The tibia is the predominant bone known to be affected in the familial form, while the mandible is mostly affected in the sporadic form. The different pathological processes found in these two forms add further to the heterogeneity of this disorder. We describe an Arabic-Christian, non-consanguineous family with two affected sibs. In one, recurrent Caffey's disease was diagnosed at the age of 11 years.
Two patients who presented with discomfort in the calves while walking down a slope were diagnosed as suffering from eosinophilic fasciitis of the lower limbs. Following therapy of the underlying disease, this peculiar discomfort remitted concomitantly with the disappearance of the subcutaneous abnormalities. The authors posit that downslope claudication of the calves in eosinophilic fasciitis constitutes an additional variant of claudication of the lower limbs.
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The interface membranes of both components from two isoelastic cementless hip arthroplasties, which were revised for aseptic loosening, were examined histologically. The membranes consisted of densely structured fibrous tissue interspersed with giant cell granulomas, sheets of histiocytes and chronic inflammatory infiltrates. The granulomas and the histiocytes contained polymeric and metallic wear products, respectively. The morphological features of these membranes were compared with those of cemented joint replacements. Apart from the deposits of acrylic cement bordered by macrophages, there were no distinctive differences between the membranes of failed cementless and cemented arthroplasties. The release and deposition of wear products, of whatever nature, are apparently responsible for the formation of exuberant interface membranes.
A patient with long-standing sarcoidosis underwent partial colectomy for diverticular disease. Whereas sarcoid granulomas were rare in the otherwise normal colonic mucosa, an unusually large number of granulomas were scattered within the chronically inflamed peridiverticular tissues. It is suggested that in the framework of the sarcoidotic background, activated macrophages, being nonspecifically attracted to areas of inflammation, elicit an exuberant sarcoid granulomatous response. Our observation represents a previously undescribed variant of the "homing" phenomenon in sarcoidosis, unrelated insults inducing formation of granulomas in overabundance.
Intramuscular haemangioma, though benign, may be a serious disorder, because its large size can cause functional impairment, pain and disfigurement, which may require surgical excision. These, as well as the baffling histopathological aspects, are exemplified by this report of an intramuscular haemangioma of the forearm in an adolescent girl. Clinical recognition of a cutaneous angioma overlying a deeply-situated soft tissue tumour may aid the diagnosis of an intramuscular haemangioma.
Three patients presented with septal fasciitis and panniculitis, associated with clinical and laboratory features which precluded straight-forward classification into eosinophilic fasciitis, localised scleroderma, or lupus erythematosus profundus. Treatment with cimetidine caused the remission of cutaneous manifestations and the extracutaneous abnormalities, such as nailfold capillary disturbances and the presence of antithyroid antibodies, improved. It is concluded that features of eosinophilic fasciitis or localised scleroderma and certain additional atypical elements should be categorised as atypical fasciitis-panniculitis syndrome.
In an elderly patient with oligosymptomatic giant splenomegaly, clinical and laboratory data were nondiagnostic, while nonhomogeneous splenic enlargement was the only finding detected by imaging procedures. Splenectomy was performed and diffuse hemangiomatosis of predominantly capillary-type found. The failure of imaging techniques to even hint at the nature of the underlying disorder is comprehensible in view of the organ being essentially replaced in toto by the abnormal vascular channels. Diffuse splenic hemangiomatosis, a rare condition, may cause hypersplenism, and its diagnosis may be elusive because of misleading patterns on imaging.
A patient with sinonasal malignant triton tumor (ie, malignant schwannoma with rhabdomyoblastic differentiation) is described. In spite of its recurrence, the tumor has behaved as a slow-growing, locally aggressive, low-grade sarcoma for 3 years. This deviatory behavior is ascribed to well-differentiated nature and the primary exophytic-polypoid growth of the tumor.
A leiomyoma of the fetal membranes was incidentally discovered on examination of a spontaneously expulsed placenta following an uneventful pregnancy and delivery of a healthy neonate. Perusal of the literature uncovered only a single report of a placental leiomyoma.
We report our experience of seven cases suffering from capillaro-venous malformation localized in the head and neck area, treated preoperatively by a fibrosing agent--ETHIBLOC. Four of the cases are presented and analysed. We suggest the pre-operative injection of Ethibloc as the treatment of choice for capillaro-venous malformations in the head and neck region.
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