Ines Misselevitch

Familial Caffey's disease and late recurrence in a child

Cortical Infantile Hyperostosis (Caffeys 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 Caffeys disease was diagnosed at the age of 11 years.

Acute neutrophilic myositis in sweet's syndrome: Late phase transformation into fibrosing myositis and panniculitis

Early in the course of myeloblastic leukemia a patient concurrently developed febrile neutrophilic dermatosis and sterile acute myositis. The dermatitis and myositis were unresponsive to antibiotic therapy but remitted within a few days of institution of steroid treatment. The patient died of myocardial infarction. At autopsy the dermis was normal. Previously effected muscles were scarred. The overlying fascia and subcutaneous septa were fibrotically thickened. In addition, segmental acute aortitis was detected. Acute myositis and aortitis may reflect further organ manifestations of the Sweets reactivity pattern. It is proposed that Sweets myositis and dermatitis may evolve into a fibrosing myositis and panniculitis.

The Fasciitis-Panniculitis Syndrome: Clinical Spectrum and Response to Cimetidine

The term fasciitis-panniculitis syndrome (FPS) is proposed as a novel compilation encompassing several disorders, common to which is subcutaneous induration caused by cicatrizing fasciitis as well as septal and lobular panniculitis and perimysial fibrosis. Included herein are Shulmans eosinophilic fasciitis, morphea profunda, lupus profundus, venous lipodermatosclerosis, toxic oil syndrome, altered tryptophane-related eosinophilic myositis, graft-versus-host reaction, and fasciitis reactive to subjacent basal cell carcinoma. FPS should be differentiated from scleroderma, which primarily affects the dermal structures and in which arterioles are injured. In contrast, vasculopathy of the subcutaneous medium-sized veins accompanies the hypodermal lesions of FPS. The importance of recognizing and grouping these disorders lies in their different histopathology, characterization as reactive phenomena, enhanced responsiveness to treatment, and better prognosis than scleroderma. In view of the excellent prognosis of FPS, steroid treatment is not warranted. Long-term therapy with cimetidine appears to benefit the majority of patients.

Liposarcoma of the larynx.

Liposarcomas of the larynx are very rare. Only nine cases appear to have been recorded in the English literature. An additional case of liposarcoma of the larynx occurring in a young patient is reported and the relevant literature discussed.

Acute Cor pulmonale due to Tumor Cell Microemboli

Severe pulmonary hypertension due to tumor cell microemboli or lymphangitic carcinomatosis is a rare complication of malignant disease. In most of the reported cases, a clinical picture of subacute cor pulmonale developed. A 57-year-old man with deep vein thrombosis in his left calf developed acute progressive dyspnea with hypoxemia, cyanosis and the clinical picture of acute cor pulmonale, while he was on full heparinization. Respiratory failure with the need for mechanical ventilatory support developed within 2 days. Chest radiography revealed the development of acute cardiac enlargement, dilated pulmonary artery and diffuse opacities in the fields of both lungs. Open lung biopsy disclosed massive tumor cell microemboli and lymphangitic carcinomatosis. No parenchymal metastases were found. This case is extremely rare because of the rapid development of the patients respiratory signs and symptoms of acute cor pulmonale due to pulmonary tumor cell microemboli and lymphangitic carcinomatosis. It is also most atypical because of the rare pulmonary radiographic presentation.

Autosomal‐recessive omodysplasia: Prenatal diagnosis and histomorphometric assessment of the physeal plates of the long bones

Second-semester ultrasonography of a female fetus documented short femora and humeri and dislocation of the radii. Based on the clinical and postmortem radiological findings, autosomal-recessive omodysplasia was diagnosed. The physeal plates of the long tubular bones were assessed by computer-assisted image analysis. The dimensions and orientation of the chondrocytic lacunae in the physeal plates of the omodysplastic fetus were compared with those in the physeal plates of fetuses without gross limb abnormalities (oligohydramnios, n = 2; hydrocephalus, n = 2; Down syndrome, n = 1). The pathological characteristics of the omodysplastic physeal plates were an expanded zone of proliferating cartilage and an increased number of closely packed, small chondrocytes. We propose that a genetic, functional deficiency of the physeal cells, underlying the short-limbed dwarfism of autosomal-recessive omodysplasia, is partially compensated, albeit ineffectively, by an increased number of small chondrocytes in the proliferating zone of the physeal plate.

Intrahyoid thyroglossal cyst.

A rare case of an intrahyoid thyroglossal duct cyst in a 63-year-old woman is reported. The pertinent literature is discussed.

Downslope Claudication in Eosinophilic Fasciitis: A Fourth Type of Claudication—Case Presentations

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.

Colonic diverticulitis-related exuberant granulomatous reaction in a patient with sarcoidosis

SummaryA 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.