Dabska tumor developing within a preexisting vascular malformation.

Malignant endovascular papillary angioendothelioma, first described by Dabska in 1969, is a rare vascular tumor that primarily affects children and is characterized by papillary proliferations of endothelial cells into vessel lumens. We report a case of this rare neoplasm in a boy with angiomatosis who developed Kasabach-Merritt syndrome. The tumor evolved as an ulcerated lesion superficially within a previous vascular malformation on his buttock. A review of the literature is presented.

Pleomorphic angioleiomyoma.

A 72-year-old man presented with a single nodule in the right thigh of 12 years' evolution. Histopathology confirmed the diagnosis of angioleiomyoma. However, unlike other tumors of this type reported in the literature, marked nuclear pleomorphism without mitotic figures was noted.

Partial lipodystrophy associated with juvenile dermatomyositis: report of two cases.

A 27-year-old woman and a 13-year-old girl diagnosed with juvenile dermatomyositis in childhood developed clinical findings of partial lipodystrophy 10 years after diagnosis. Exhaustive clinical and laboratory examinations showed an association with other abnormalities: hypertrichosis, steatohepatitis, and an abnormal insulin response to the glucose loading test in the first patient. Hypertrichosis, steatohepatitis, insulin-resistant diabetes mellitus, and acanthosis nigricans were observed in the second patient. Renal function was normal in both patients. Although a localized form of lipodystrophy has been reported associated with connective tissue disease (connective tissue lipoatrophy), the partial form has been infrequently described in association with juvenile dermatomyositis.

Acute exanthem and pancreatic panniculitis in a patient with primary HIV infection and haemophagocytic syndrome.

We report a patient with an acute infection with the human immunodeficiency virus (HIV), who initially presented due to a mononucleosis-like illness that included a rash on the upper trunk and limbs, and oral ulceration. The patient developed a haemophagocytic syndrome with severe systemic involvement. Three weeks after the initial presentation, lesions of a pancreatic panniculitis appeared on both legs.

Efficacy of lipectomy and liposuction in the treatment of multiple symmetric lipomatosis.

BACKGROUND: Multiple symmetric lipomatosis (MSL) is a rare disease characterized by enlarging, symmetric, nonencapsulated, fat deposits mainly on the neck and upper trunk. Liposuction and lipectomy, although palliative, are the treatments of choice, especially indicated when vital structures are compromised. OBJECTIVE: Our purpose was to evaluate the efficacy and safety of liposuction and lipectomy in the treatment of MSL. METHODS: We have examined two patients diagnosed with MSL who presented with symptoms derived from the compression of vascular, nervous, and/or respiratory tract structures. One was treated with lipectomy and the other with liposuction. RESULTS: A rapid resolution of the clinical symptoms was achieved with both therapies. The patient who was treated with lipectomy suffered from a compression of the left brachial plexus by scar tissue as an adverse effect, requiring a second surgical procedure. Liposuction only provoked a mild autoinvolutive hematoma in the other case. No clinical recurrences were observed at 3 and 2 years of follow-up respectively. CONCLUSIONS: We consider both lipectomy and liposuction as safe and effective techniques for the treatment of MSL patients. Although liposuction is usually associated with less adverse effects than lipectomy, location of the lipomas must be carefully considered before choosing one technique over another.