1/176. Nodular amyloidosis treated with a pulsed dye laser.BACKGROUND: Nodular amyloidosis is a rare form of primary localized cutaneous amyloidosis which is characterized by single or multiple nodules located on the extremities, trunk, genitalia, or face. OBJECTIVE: To determine the clinical and histologic response of nodular amyloidosis to pulsed dye laser treatment. methods: biopsy-proven amyloid nodules were treated with a 585-nm pulsed dye laser (average fluence 5.25 J/cm2; 10 mm spot) at 6- to 8-week time intervals. Clinical and histologic examination of laser-irradiated nodules were performed before and 6 weeks after the final laser treatment. RESULTS: Clinical improvement in the color, size, and pliability of nodules was noted and maintained for 6 months. Histologic examination revealed decreased inflammation and improvement in dermal collagen after laser irradiation. CONCLUSIONS: Since amyloid fibrils may be formed in association with dermatan sulfate-an essential matrix component in collagen fiber formation, it is postulated that the improvement seen in amyloid nodules after pulsed dye laser treatment may be attributed to a mechanism similar to that seen with hypertrophic scars.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
2/176. immunoblastic lymphadenopathy-like T-cell lymphoma complicated by multiple gastrointestinal involvement.We report a rare case of immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma complicated by multiple gastrointestinal involvement, which appeared to be ameliorated by chemotherapy but resulted in perforative peritonitis. A 66-year-old Japanese woman who had generalized lymphadenopathy and eruptions was admitted to our hospital because of bloody stool. Colonoscopic examination revealed hemorrhagic ulcers in the terminal ileum and a saucer-like ulcer in the cecum. Gastrointestinal endoscopy revealed several ulcerative or elevated lesions in stomach and duodenum. biopsy specimens of these lesions and of a lymph node showed characteristic histological features of IBL-like T-cell lymphoma. The initial treatment with prednisolone (PSL) and cyclophosphamide (CPA) was effective. Six months after the treatment, however, she developed bloody stool again caused by multiple ulcerative lesions in the large intestine. The recurrence of the disease was determined histologically, and four courses of CPA, PSL, vinblastine sulfate and doxorubicin hydrochloride (CHOP) therapy were administered. One month after completing the CHOP therapy, she developed intestinal obstruction and then acute peritonitis resulting from perforation at an ulcer scar in the jejunum. Surgical treatment was successful, and histological examination demonstrated no lymphoma cells in the resected specimen. A gastrointestinal perforation should be recognized as a potential complication of IBL-like T-cell lymphoma, even during remission.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
3/176. Striae formation in two hiv-positive persons receiving protease inhibitors.Steroidal side effects such as "buffalo hump" and visceral abdominal fat accumulation have been reported in association with protease inhibitors. We report two cases of severe disfiguring striae formation in patients with hiv who recently started indinavir therapy. These changes occurred within 3 months of starting treatment.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
4/176. Necrotizing fasciitis after cosmetic blepharoplasty.PURPOSE: To report a case of necrotizing fasciitis after cosmetic blepharoplasty. METHOD: Case report. A 74-year-old woman with history of type II diabetes mellitus underwent bilateral upper eyelid blepharoplasty. Postoperatively she developed fever, grayish discoloration of the skin, violaceous bullae, and a right facial nerve palsy. Necrotizing fasciitis was diagnosed and treated with intravenous antibiotics, debridement of necrotic tissue, and hyperbaric oxygen therapy. RESULTS: The infection resolved, but the patient required reconstruction for correction of cicatricial ectropion. CONCLUSION: Necrotizing fasciitis is a potentially fatal infection that typically occurs in the setting of trauma. Early recognition of its pathognomonic signs and aggressive management are paramount.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
5/176. subcutaneous fat necrosis of the newborn associated with anemia.subcutaneous fat necrosis (SFN) of the newborn characteristically affects full-term infants who have experienced perinatal distress, such as hypothermia, obstetric trauma, or asphyxia. We report a newborn who had pallor, deep breathing, and severe anemia immediately after birth. She developed SFN on the fourth postnatal day. Her condition improved after blood transfusions and the skin lesions resolved in 6 weeks. This appears to be the first report of SFN associated with anemia.- - - - - - - - - - ranking = 5keywords = fat (Clic here for more details about this article) |
6/176. Cutaneous lesions as the presenting sign of acute graft-versus-host disease following liver transplantation.Acute graft-versus-host disease (GVHD) is a frequent complication of bone marrow transplantation but is only rarely observed after solid organ transplantation. We describe a 68-year-old man who developed a maculopapular eruption 7 days following orthotopic liver transplantation for cirrhosis with malignant transformation due to haemochromatosis. At day 20, the patient complained of nausea, vomiting, diarrhoea and fever. skin biopsy revealed a lymphocytic infiltrate at the dermoepidermal interface, vacuolization of basal cells and epidermal dyskeratosis. immunohistochemistry showed HLA-DR and intercellular adhesion molecule-1 expression of lesional keratinocytes. HLA-typing of peripheral blood lymphocytes demonstrated circulating lymphocytes of donor origin. endoscopy revealed extensive erosions of the oesophagus, stomach and duodenum that on histology disclosed multifocal loss of crypts, lymphocytic infiltrates and epithelial cell death. A diagnosis of acute GVHD was made, and high-dose immunosuppressive therapy with azathioprine and methylprednisolone was instituted. The skin and gastrointestinal symptoms subsided within 4 weeks, but the patient died from severe infectious complications 105 days after transplantation. We conclude that acute GVHD is a rare but potentially fatal complication of liver transplantation. skin lesions are an early sign of acute GVHD and thus represent an important tool for early diagnosis.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
7/176. Isolated cutaneous response to granulocyte-monocyte colony stimulating factor in fatal idiopathic disseminated bacillus-Calmette-Guerin infection.Severe disseminated bacillus-Calmette-Guerin (BCG) infection is very rare and has been regarded as idiopathic when no immunodeficiency is present. This entity seems to be due to several new types of inherited abnormalities in the pathways important in defence against Mycobacteria. Although improvement with interferon-gamma (IFN-gamma) has been reported in some patients, to our knowledge there are no reports on the effect of other cytokines in the treatment of these patients. We report here the clinical response to IFN-gamma and granulocyte-monocyte colony stimulating factor (GM-CSF) treatment in a patient with idiopathic disseminated BCG infection who failed to respond to multiple antimycobacterial agents. The patient showed partial and transitory response to IFN-gamma, however, GM-CSF treatment led to rapid improvement of skin lesions within 2 weeks without any effect on the progression of the disease in the other organ systems. CONCLUSION: The response of idiopathic disseminated bacillus-Calmette-Guerin infection to granulocyte-monocyte colony stimulating factor treatment was limited to cutaneous lesions. Granulocyte-monocyte colony stimulating factor may have acted to promote wound healing or the levels of this factor achieved in other affected organs may have been inadequate.- - - - - - - - - - ranking = 4keywords = fat (Clic here for more details about this article) |
8/176. Subcutaneous sarcoidosis associated with vitiligo, pernicious anaemia and autoimmune thyroiditis.We report a patient with pernicious anaemia, primary autoimmune hypothyroidism and vitiligo, who presented with subcutaneous nodules. Histopathology of the nodules revealed noncaseating granulomas, consistent with a diagnosis of sarcoidosis. Mild pulmonary sarcoid was also detected. Although an association between sarcoidosis and other autoimmune diseases is well-recognized, the presence of the particular autoimmune diseases in our patient and the involvement of subcutaneous fat in the sarcoidal inflammation, appears to represent a most unusual clinicopathological combination.- - - - - - - - - - ranking = 598.16273903669keywords = subcutaneous fat, fat (Clic here for more details about this article) |
9/176. Compound heterozygous group A xeroderma pigmentosum patient with a novel mutation and an inherited reciprocal translocation.The severity of neurological abnormalities in Japanese group A xeroderma pigmentosum (XP-A) patients correlates with the sites of non-sense mutation in the XP-A gene. We describe a patient who presented with a more severe photosensitivity and neurological abnormality than those in typical Japanese XP-A patients with a splicing mutation in intron 3. The patient was compound heterozygous for the splicing mutation in intron 3, which resulted in formation of a non-sense codon in exon 4, and a novel non-sense mutation at codon 208 in exon 5, a C to T transition creating a stop codon TAG. Although the combination of these mutations might have been thought to cause only mild neurological signs, the longer truncated XP-A proteins than those of typical XP-A patients may have resulted in severe neurological symptoms. This phenomenon may be explained by a translocation of chromosome (1;10)(q25.3;q22.3) inherited from his father.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
10/176. Congenital fascial dystrophy: abnormal composition of the fascia.BACKGROUND: A scleroderma-like genetic disease, congenital fascial dystrophy, probably a variant of stiff skin syndrome described by Esterly and McKusick, was found to be related to genetically determined fascial abnormalities. Our previous electronmicroscopic study disclosed as a main pathologic finding presence of giant amianthoid-like collagen fibrils in the affected fascia. OBJECTIVE: The aim of the present study was to disclose the collagen abnormalities in the affected and control fascias and in the patient's fibroblast cultures derived from the skin and fascia. methods: The study was performed by histologic, immunohistochemical, and electronmicroscopic techniques. Immunohistochemical studies were done with the use of monoclonal antibodies: anti-collagens I, III, IV, and VI, anti-laminin, anti-fibronectin, anti-desmin, anti-spectrin, anti-vimentin, anti-laminin, anti-heparan sulfate, and anti-alpha-actinin. Electronmicroscopic studies were performed on the fascia sections and on cultured fibroblasts. RESULTS: The main abnormality leading to giant collagen fibril formation was presence of myofibroblasts, absence of collagen III, and overproduction of spectrin and collagen type vi, mainly its filamentous form. CONCLUSION: Our findings suggest that the abnormal composition of the fascia could depend on modulation of fibroblasts into myofibroblasts capable of producing spectrin and long-spacing collagen.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
| | Next -> |