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Nevus lipomatosus superficialis is a rare disorder characterized by a nevoid fatty growth within the papillary and reticular dermis. Lesions more often occur in the sacral, abdominal, or pelvic regions. A 36-year-old Brazilian female with giant nevus lipomatosus is reported. Our case seems to be the biggest reported in the literature.
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BACKGROUND: Lipodermatosclerosis (LDS) is characterized by a hardening and hyperpigmentation of lower leg skin as a consequence of chronic venous insufficiency. The degree of skin hardening or fibrosis associated with LDS is proposed to relate directly to skin breakdown and venous ulcer formation as well as to a subsequent delay in ulcer healing. OBJECTIVES: To determine whether elevated procollagen type I gene expression and increased cell proliferation are responsible for the fibrotic changes associated with LDS. METHODS: Skin biopsies were obtained from the legs of patients with varying degrees of chronic venous disease and were assessed for procollagen gene expression by in-situ hybridization and for cell proliferation by immunolocalization of proliferating cell nuclear antigen. RESULTS: The number of cells expressing procollagen type I mRNA (COL1A1) was significantly higher in the dermis of LDS-affected skin compared with samples from the other patient groups. In addition, there was a significant increase in the number of dermal fibroblasts undergoing proliferation in both LDS samples and skin samples prior to LDS changes compared with control samples. However, there was no significant difference in level of inflammation in biopsy samples between patient classes. CONCLUSIONS: These results suggest that enhanced cell proliferation and procollagen gene expression are both involved in LDS development. Furthermore, fibrotic changes may occur in the absence of, or subsequent to, any significant inflammatory response, indicating that additional profibrotic factors produced in the skin as a consequence of chronic venous insufficiency may play a role in LDS formation.
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A 54-year-old female developed tender, hot, erythematous skin at the apex of a large pendulous abdomen at the same time as she became symptomatic with congestive cardiac failure secondary to respiratory failure. Clinically and histologically the changes in the abdominal-wall skin resembled lipodermatosclerosis, a condition normally seen in the legs secondary to chronic venous insufficiency.
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Two typical cases of Jadassohn's Naevus Phakomatosis are reported, with full clinical details, history and the results of investigations. Neurosurgery was necessary in one case so that the macroscopical appearance of the brain and histological findings of portions of the brain removed are described.
Nevus lipomatosus cutaneus superficialis (Hoffman-Zurhelle) is a distinct clinical entity. A case is reported in which the lesions were large, appeared early in childhood and were linear in distribution along the natal cleft.
Large subcutaneous angiolipomata were observed bilaterally around the wrists, knees, and ankles in an adolescent boy. Growth had been slow since first noted at age 1 year. The tumors extended deeply between muscles, tendons and joint capsules, but without infiltration of these structures. The tumors recurred after subtotal excision. Muscular hypotrophy and deformation of bones near the affected joints were noted. An 8-year-old sister had similar tumors, suggesting a genetic etiology.
A patient with familial angiolipomatosis in whom an autosomal dominant mode of inheritance could be established is described. This patient also had coincidental autosomal dominant polycystic kidney disease.
A case of multiple intraosseous lumbar vertebral lipomata is reported occurring in the presence of Paget's disease. The pathology and radiology are correlated.
BACKGROUND: Colonic pseudolipomatosis is rare and the pathogenesis is controversial. The purpose of the present paper was to clarify endoscopic and histological characteristics of colonic pseudolipomatosis and to discuss the etiology. METHODS: A total of 15 lesions from 14 patients was reviewed. They were able to be histologically classified into two groups on the basis of variety in size of the vacuoles: Group A, the ratio of largest vacuole to smallest vacuole in size is less than three, Group B, the ratio is more than four. RESULTS: Four of 15 lesions were group A, and were endoscopically polypoid or flat lesions covered with normal-looking mucosa. They were microscopically characterized by (i) predominant location in the upper portion of the lamina propria; (ii) no submucosal involvement; (iii) less variation in vacuolar size; and (iv) no association with lymph follicles. The vacuoles of group A contained proteinaceous materials in two of four lesions. Group B (11 lesions) had small elevated mucosa with normal-looking surface or non-elevated reddish mucosa. Microscopically, the lesions were mainly located in the lower portion of the lamina propria, occasionally also in the submucosa, had variable-sized vacuoles, and were related to lymph follicles. CONCLUSION: It is suggested that the vacuoles in group A contain fluid, and may indicate an abnormal stagnation of interstitial fluid. Microscopic appearance of group B is essentially similar to that of pneumatosis coli. It is thought that group B probably results from penetration of gas from the crypts into the mucosa during colonoscopy. It is unclear why group B had a preference for ileocecal valve and an association with lymph follicles.
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Gastric lipomas are uncommon lesions, usually single and located in the antrum. Common symptoms include upper digestive bleeding, anemia, and intestinal obstruction. Alternatively, they may remain asymptomatic, and detectable only at autopsy. A case of a 72-year-old man, who died of myocardial infarction, was found at autopsy to have multiple gastric lipomas of the corpus and antrum, with a single lesion measuring 10 x 6.5 x 3 cm.
A case of fatty replacement of the pancreatic body and tail associated with leiomyosarcoma of the pancreatic head is presented. Clinicopathological features as well as differential diagnoses are described with a review of the literature on both leiomyosarcoma and fatty replacement of the pancreas. A possible correlation between the two lesions is also discussed.
Lipomatous pseudohypertrophy of the pancreas was found at autopsy in a 52-year-old Japanese woman with cirrhosis due to chronic hepatitis B. Clinically, there were no clear symptoms of pancreatic insufficiency during the entire course. Marked atrophy and fat deposition of the pancreas had already been detected by computed tomography (CT) at least 6 years before her death. She died of hepatic failure due to decompensated cirrhosis. Autopsy revealed uniform enlargement of the pancreas due to massive fat replacement (lipomatous pseudohypertrophy): the exocrine glandular elements showed marked atrophy and loss, while the islets of Langerhans were preserved. Although the etiology and pathogenesis of lipomatous pseudohypertrophy is still unclear, this case suggests that this condition is causally related to chronic hepatitis B or other chronic advanced hepatic lesions.