Pemphigus vulgaris--the potential for error: a case report.

The importance of correlation, referral, and collaboration between dermatology, or any other medical department, and the oral health profession is reported through a case presentation. A case of pemphigus vulgaris diagnosed from a tongue biopsy and confirmed by immunofluorescence is reported. The patient was treated for actinic keratosis of the scalp for 16 months due to inadequate biopsy material. The diagnostic value of proper biopsy material is stressed. The clinical manifestations leading to a misdiagnosis between actinic keratosis and pemphigus vulgaris are discussed.

Fatal pulmonary toxicity following oral etoposide therapy.

Oral etoposide has considerable activity in small cell lung cancer and the low risk of toxicity has resulted in the frequent prescription of this agent in elderly or infirmed patients. We describe a case of fatal pulmonary toxicity following the administration of oral etoposide. This is the first report of biopsy-proven pulmonary toxicity associated with this agent.

Tongue, primary amyloidosis, and multiple myeloma.

A case of macroglossia and resulting apertognathia because of primary amyloidosis in a 65-year-old man with multiple myeloma is described. In addition, a retrospective study of the oral manifestations of primary amyloidosis and multiple myeloma in the last 15 years is reported.

Malignant transformation in Paget disease of bone.

BACKGROUND: The previously reported incidence of malignant transformation of Paget disease up to 5.5% and its dismal prognosis have prompted the clinical investigation of a large population with Paget disease. METHODS: A chart review of symptomatic and asymptomatic patients with a diagnosis of Paget disease between 1970 and 1985 at four large Montreal Hospitals revealed 1078 patients. Eight patients with malignant transformation were studied in detail. RESULTS: The incidence of malignant transformation was 0.7%, and the most frequent histologic type was osteogenic sarcoma. The most prevalent site was the femur, and pathologic fracture with focal osteolysis was present in 50% of patients at the initial appearance of the tumor. Healing at the fracture site was demonstrated in one patient. Another patient died of uncontrollable hemorrhage. A case of malignant lymphoma in Paget disease is recorded. CONCLUSIONS: Surgery or biopsy should be preceded by preoperative scintigraphic bone blood flow evaluation and, if necessary, administration of a preoperative course of mithramycin and selective embolization to minimize bleeding. The prognosis of malignant transformation in Paget disease is poor, although one patient with malignant lymphoma survived after aggressive treatment. Early biopsy and aggressive treatment should not be delayed.

Minocycline-induced Sweet's syndrome.

This is the second reported case of minocycline-induced Sweet's syndrome (and the first such case to appear in the American literature). The syndrome developed in a 32-year-old man 10 days after minocycline therapy for acne was begun and resolved rapidly after discontinuation of the medication and start of oral prednisone therapy.

Hemothorax and hemopericardium in a patient with Bean's blue rubber bleb nevus syndrome.

A patient with Bean's blue rubber bleb nevus syndrome presented with a hemothorax, and 18 years earlier had presented with "idiopathic" hemopercardium and tamponade. Typical hemangiomas were found in the pleura and skin. This is the first report of intrathoracic bleeding with this disorder.

Pagetic arthritis. Pathophysiology and management.

A review of the records of 112 patients demonstrate modern concepts of pathogenesis and treatment of Paget's disease. Pain from Pagetic arthritis should initially be treated by medical treatment aimed at modulation of bone blood flow. Patients, even with impressive arthritic reactions, can respond dramatically. Pain persists in patients with end-stage destruction of articular cartilage and/or stress microfractures. Patients with deformity without joint space loss may be candidates for osteotomy. Advanced cartilage destruction is treated by total arthroplasty and continuation of anti-Pagetic therapy after surgery, possibly to reduce the incidence of component loosening. Articular cartilage erosion and subchondral bone destruction are attributable to the disease process. Vascular invasion and joint surface collapse are associated with cartilage thinning and sequestration.

Familial lichen planus.

We observed the simultaneous occurrence of generalized lichen planus in a woman and her mother. Both patients improved after therapy with topical steroid and salicylic acid ointment. Of the eighty-one cases of familial lichen planus previously reported, the vast majority (89 percent) occurred in blood relatives. The intervals of onset between familial cases were long, ranging from six weeks to thirty years (mean 73.4 months). These observations suggest that familial lichen planus may result from genetic predisposition, rather than from an infectious cause.

Vascular abnormalities in a fetus with multiple pterygia.

We describe a 16-week fetus with a lethal multiple pterygium syndrome and hydrops. No bony abnormalities were noted on radiographic or anatomical examination. A prominent meshwork of dilated, thin-walled vessels was present in the subcutis over the entire body. This abnormal vascularity may have caused pterygium formation and death of the fetus.

Eruptive vascular tumors associated with chronic graft-versus-host disease.

We report the occurrence of benign angiomatous and mesenchymal tumors following chronic graft-versus-host disease. Our patient is a 23-year-old man who had undergone allogenic bone marrow transplantation for chronic myelogenous leukemia in 1978. Over the past 3 years he has developed poikiloderma and sclerodermatous tightening of the upper and lower extremities. From 1981 until the present time, violaceous to black friable tumors have erupted on the lower extremities. Histopathologic findings show an active mesenchymal and vascular tumorous response of variable-sized channels.

The coexistence of rheumatoid arthritis and scleroderma: a case report and review of the literature.

We describe a patient with all the features of the CREST syndrome variant of scleroderma who developed a destructive arthropathy typical of rheumatoid arthritis (RA). A pulmonary rheumatoid nodule was found confirming the presence of RA. The rarity of the coexistence of these two diseases in the same patient suggests that, despite commonly found overlapping features, these illnesses are distinct entities.

Absent left hemidiaphragm, arhinencephaly, and cardiac malformations.

An infant is reported with absent left hemidiaphragm, hydrocephalus, arhinencephaly, and cardiovascular anomalies. The parents are second cousins.

Severe renal dysgenesis produced by a dominant gene.

A woman with the autosomal dominant syndrome of preauricular pits, cervical fistulae, and partial deafness gave birth to two children with preauricular pits and severe renal dysgenesis. The facies had some features of the Potter facies of renal agenesis. One child died soon after birth because of pneumothorax and immature development of the lungs. We suggest that all infants with renal agenesis or dysgenesis be examined for preauricular pits because of the high recurrence risk of renal anomalies in families with this syndrome.