In some cases, it is difficult to determine a single cause of death even after conducting full autopsy and additional tests. A 49-year-old man, reportedly having diabetes mellitus, was found unconscious by his mother and revealed to be dead. He had several contusions all over his body, including the right periocular area, but they did not appear fatal. A focal area of polymicrogyria and cortical dysplasia was found on the right preoccipital notch, accompanied with dystrophic calcification and leptomeningeal angiomatosis. These findings were considered indicative of Sturge-Weber syndrome, a rare neurocutaneous disorder, of atypical type without facial lesions. Blood level of beta-hydroxybutyrate was 859 microg/mL, implying that he also had diabetic ketoacidosis. His ketoacidosis may not have been corrected appropriately because of status epilepticus in association with brain lesion, resulting in his death, but neither direct evidence nor statement was obtained. In cases with several apparent causes of death, the examiner's assumption should be based not on imagination but on evidence, and logic should not be overlooked. It is more helpful for the investigators or the bereaved to obtain more detailed information rather than come to a hasty conclusion.
3-Hydroxybutyric Acid ; Angiomatosis ; Autopsy ; Brain ; Cause of Death ; Contusions ; Diabetes Mellitus ; Diabetic Ketoacidosis ; Humans ; Imagination ; Ketosis ; Logic ; Malformations of Cortical Development ; Middle Aged ; Mothers ; Neurocutaneous Syndromes ; Research Personnel ; Status Epilepticus ; Sturge-Weber Syndrome*

Angiomatosis ; pathology ; Breast ; pathology ; Breast Diseases ; pathology ; Female ; Humans ; Hyperplasia ; pathology

Diffuse dermal angiomatosis is an acquired, benign vascular proliferation with poorly circumscribed, violaceous, and livedoid plaques with frequent ulceration. Histologically, there is diffuse and interstitial proliferation of CD31-positive endothelial cells and myofibroblasts within the dermis. Endothelial atypia, mitoses, and vasculitis are lacking. Here we describe a 52-year-old male who presented with erythematous and purpuric patches on the trunk and lower extremities for 2 years. Histologic examination demonstrated a dense proliferation of endothelial cells in the papillary and reticular dermis. Small vascular spaces and extravasated erythrocytes were noted. Immunohistochemical staining using anti-CD31 antibody was positive.
Angiomatosis ; Dermis ; Endothelial Cells ; Erythrocytes ; Hemangioendothelioma ; Humans ; Lower Extremity ; Male ; Mitosis ; Myofibroblasts ; Skin Neoplasms ; Ulcer ; Vasculitis

Tumoral form of pseudoangiomatous stromal hyperplasia (PASH) of male breast is a rare disease entity. We report on a case of PASH that presented as a palpable subareolar breast mass in a 46-year-old male who has been on hemodialysis due to chronic renal failure. To the best of our knowledge, no case of PASH in a male with chronic renal failure has yet been reported. We describe its mammographic and sonographic findings with correlative histopathologic features, confirmed by surgical excision.
Angiomatosis ; Breast ; Breast Diseases ; Humans ; Hyperplasia ; Kidney Failure, Chronic ; Male ; Rare Diseases ; Renal Dialysis

Sclerosing angiomatoid nodular transformation (SANT) of the spleen, a newly defined primary lesion of the spleen, is very rare. Immunohistochemistry is the only way to confirm the diagnosis of SANT. We present the clinical characteristics and postoperative outcomes of five SANT cases that underwent splenectomy from January 2007 to October 2010. Although SANT had specific imaging findings, differential diagnosis from other splenic tuomrs or malignant lesions preoperatively was difficult. The hand-assisted laparoscopic splenectomy was a useful and effective technique for the management and postoperative diagnosis of SANT. All SANT patients had good prognosis without recurrence after splenectomy.
Adult ; Angiomatosis ; diagnosis ; pathology ; surgery ; Female ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Spleen ; pathology ; surgery ; Splenic Neoplasms ; diagnosis ; pathology ; surgery

Erythema elevatum diutinum (EED) is emerging as a specific HIV-associated dermatosis which can be easily misdiagnosed as Kaposi's sarcoma or bacillary angiomatosis. Until now, no case of HIV-associated EED had been reported in Korea. We report a case of EED in a 49-year-old man with HIV infection. The patient was diagnosed with HIV-infection and treated with a combination of anti-retroviral agents and dapsone. Two years after the start of treatment the lesion had regressed.
Angiomatosis, Bacillary ; Anti-Retroviral Agents ; Dapsone ; Erythema ; HIV ; HIV Infections ; Humans ; Korea ; Middle Aged ; Sarcoma, Kaposi ; Skin Diseases ; Vasculitis, Leukocytoclastic, Cutaneous

A tumoral pseudoangiomatous stromal hyperplasia (PASH) that causes huge breast enlargement is very rare. Only two cases of huge tumoral PASHs have been reported in the English medical literature. We report here on a surgically confirmed case of bilateral huge tumoral PASH in a 47-year-old woman, and we present the imaging and histopathology findings. We also review the relevant medical literature.
Angiomatosis/*pathology/surgery/ultrasonography ; Biopsy, Needle ; Breast/cytology/pathology/surgery ; Breast Diseases/*pathology/surgery/ultrasonography ; Contrast Media/diagnostic use ; Diagnosis, Differential ; Female ; Gadolinium DTPA/diagnostic use ; Humans ; Hyperplasia ; Image Enhancement/methods ; Magnetic Resonance Imaging/methods ; Mammography/methods ; Middle Aged ; Stromal Cells/pathology

Angiomatosis ; complications ; pathology ; Brain ; blood supply ; Brain Diseases ; complications ; pathology ; Brain Neoplasms ; complications ; pathology ; Epilepsy ; etiology ; pathology ; surgery ; Ganglioglioma ; complications ; pathology ; Humans ; Malformations of Cortical Development ; classification ; complications ; pathology ; Meninges ; blood supply

OBJECTIVETo study the clinicopathologic features of sclerosing angiomatoid nodular transformation of spleen and its differential diagnosis.

METHODSThe clinicopathologic characteristics and immunophenotype of 4 cases of sclerosing angiomatoid nodular transformation of spleen were studied.

RESULTSHistologically, all cases were characterized by multiple angiomatoid nodules of various sizes in a fibrosclerotic stroma. The nodules were round and sometimes convoluted. They were composed of slit-like, irregular-shaped or slightly dilated vascular spaces lined by plump endothelial cells and interspersed with a population of spindly or ovoid cells. Immunohistochemical study showed a heterogeneous staining pattern, with the lining cells of the small capillaries expressing CD34 and those of the sinusoid-like structures expressing CD8. CD31 highlighted both the lining cells and interspersed cells, resulting in a complex meshwork. The lining cells were also focally positive for CD68. Smooth muscle actin revealed conglomerates of spindly shaped cells around and between the vascular channels. These spindly shaped cells in the intervening stroma were focally positive for actin, but negative for desmin, CD21 and CD35.

CONCLUSIONSSclerosing angiomatoid nodular transformation is a rarely encountered benign lesion of the spleen, which should be distinguished from other angiomatoid tumors and tumor-like lesions.

Adult ; Angiomatosis ; metabolism ; pathology ; surgery ; Antigens, CD34 ; metabolism ; CD8 Antigens ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hamartoma ; pathology ; Hemangioma ; pathology ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sclerosis ; pathology ; Spleen ; pathology ; Splenectomy ; Splenic Diseases ; metabolism ; pathology ; surgery ; Splenic Neoplasms ; pathology

Adult ; Angiomatosis ; complications ; pathology ; surgery ; Colonoscopy ; Diagnosis, Differential ; Gastrointestinal Hemorrhage ; complications ; pathology ; surgery ; Humans ; Male ; Skin Diseases, Vascular ; complications ; pathology ; surgery ; Treatment Outcome