Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020473 (hyperlipidemia)
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Eight patients had multiple xanthomatous plaques and subcutaneous nodules that had a predilection for the periorbital area, flexures, and trunk and that tended to ulcerate. Skin biopsy specimens showed a combination of xanthogranulomatous nodules with necrobiosis. All patients had an accompanying dysproteinemia, which was a monoclonal IgG paraprotein in six. Hyperlipidemia, low serum complement, and cryoglobulinemia were variable features. Five patients had leukopenia. Bone marrow examination showed myeloma in two patients, a lymphoproliferative process in one, and some atypical plasma cells in two. Cutaneous necrobiotic xanthogranuloma is a distinctive histologic pattern most frequently related to plasma cell dyscrasias, and it should be distinguished from normolipemic plane xanthoma and other necrobiotic granulomas.
J Am Acad Dermatol 1980 Sep
PMID:Necrobiotic xanthogranuloma with paraproteinemia. 745 93

A retrospective analysis of patients receiving isotretinoin for acne was performed, in order to determine the necessity for routine testing of lipid profiles and liver function tests during therapy. Data were analysed from 209 individuals, 113 (69 males, 44 females) of whom had been treated with 1 mg/kg/day, and 96 (67 males, 29 females) with 0.5 mg/kg/day. There were no significant changes in any of the tests of liver function. There were significant elevations in both plasma cholesterol and triglycerides at 8 and 16 weeks (P < 0.01) for both dose schedules, which were significant in both male and female subjects (P < 0.001). All the individuals with elevated cholesterol (> 6.5 mmol/l) at 16 weeks had elevated cholesterol at the onset of therapy. Triglyceride concentrations were elevated at 8 weeks, but there was no further increase thereafter. It was not possible to predict which subjects would become hypertriglyceridaemic from pretreatment lipid estimations. In conclusion, there appears to be little evidence to support the previously recommended regular biochemical monitoring of liver function and lipid profiles in patients who are treated with isotretinoin for 16 weeks. It would appear prudent to ensure that there is neither liver disease nor hyperlipidaemia prior to the onset of therapy, and to determine the triglyceride response to therapy on one occasion after 4 weeks' treatment. This change in patient management should result in considerable savings both in patient time and in blood collection and analysis.
Br J Dermatol 1993 Dec
PMID:Isotretinoin therapy for acne vulgaris: a re-evaluation of the need for measurements of plasma lipids and liver function tests. 828 55

The interrelationship between carotenemia and biliary dyskinesia was studied in eighty-two biliary dyskinesia patients and twenty-seven normal subjects. Incidence of serum carotene levels of more than 300 micrograms/dl was more frequent in biliary dyskinesia patients without liver damage or hyperlipemia. Among the carotenemia patients, no dietary cases were found, but metabolic and hyperlipemic ones were observed. Significant relationships were found between gall bladder contraction rate and the levels of serum carotene, vitamin A, and lipids in metabolic carotenemia. A close relationship between metabolic carotenemia and biliary dyskinesia was strongly suggested.
J Dermatol 1993 May
PMID:A correlation between carotenemia and biliary dyskinesia. 834 May 33

The Muir-Torre syndrome is a rare disorder characterized by sebaceous neoplasms of the skin and multiple visceral malignancies. The syndrome appears to be a familial, autosomal dominant condition. We diagnosed this syndrome in a previously unreported patient and found a personal and family history of malignancies and hyperlipidemia. The association of Muir-Torre syndrome with a family history of hyperlipidemia, another autosomal dominant condition, has not been previously reported. The possible genetic relationship between the two disorders is discussed.
J Am Acad Dermatol 1993 Feb
PMID:Muir-Torre syndrome associated with a family history of hyperlipidemia. 843 39

A case of acquired perforating dermatosis associated with diabetic nephropathy is described. The case is unusual in that the dermatosis first developed approximately 1 year after renal transplantation rather than at a time when renal function was more severely impaired or during haemodialysis. There was a partial response to treatment with isotretinoin but the use of this drug was limited by the development of hyperlipidaemia. The relevant literature is reviewed.
Clin Exp Dermatol 1997 Nov
PMID:Acquired perforating dermatosis and diabetic nephropathy--a case report and review of the literature. 960 58

Spiny keratoderma is a dermatosis consisting of multiple projections located on the palms and soles, with a distinct histology characteristic of a parakeratotic column above a hypogranular epidermis. We report six cases discovered within a year and review the present literature on spiny keratoderma. The average age of the patients was 57 years. Fifty-seven percent of the patients were male and forty-three percent were female. The duration of lesions ranged from 4 months to 40 years. Symptoms were variable, however, lesions were often unnoticed by the patient. The location of the lesions involved the palms and soles or the palms alone. Past medical history was significant for hypertension and hyperlipidemia treated with HMG-CoA reductase inhibitors. Lesions often occurred in patients involved in manual labor. Spiny keratoderma is a relatively common under-reported dermatosis found most often in older patients with history of manual labor and is possibly related to treatment with HMG-CoA reductase inhibitors.
J Dermatol 1998 Jun
PMID:Spiny keratoderma: a common under-reported dermatosis. 967 41

There is a very small number of patients who suffer from acne even in the sixth and seventh decades of life. These patients have suffered from acne for most of their lives, 30-60 years, and have often received multiple courses of antibiotics over many years. We saw 10 such patients over 4 years. One received oral isotretinoin 1 mg/kg per day, but was unable to tolerate the adverse effects of cheilitis and developed hyperlipidaemia. We subsequently treated nine others with oral isotretinoin, 0.25 mg/kg per day, for 6 months; in six the acne had virtually cleared by 3-4 months while the other three cleared by 6 months. Up to 36 months after therapy these patients have remained clear of acne except for one who relapsed after 11 months. Therefore, as these patients respond well with few side-effects both in the long- and short-term to low-dose isotretinoin, they should be treated with isotretinoin, although at the lower starting dose of 0.25 mg/kg per day compared with younger patients who are treated with 0.5-1 mg/kg per day, and the treatment maintained for 6 months.
Br J Dermatol 1998 Jul
PMID:Acne vulgaris in the elderly: the response to low-dose isotretinoin. 976 56

Renal transplant recipients have an increased risk of developing skin cancers, which are often multiple and aggressive. Frequently, these tumours develop on a background of widespread epidermal dysplasia. Systemic retinoids are known inhibitors of skin cancer but reports of their use in renal transplant patients are limited. We describe our experience using 0.3 mg/kg daily of acitretin in 16 patients over a 5-year period. Overall, there was a significant reduction in the number of new tumours excised in 12 of 16 patients during treatment compared with the same pretreatment interval. A significant chemoprophylactic effect was shown for up to 4 years of treatment. Patients with five or more tumours prior to acitretin benefited most. Two patients discontinued treatment because of side-effects and two patients developed hyperlipidaemia. Two patients with end-stage graft failure proceeded to haemodialysis. The introduction of low-dose acitretin proved to be a useful strategy in the long-term reduction of skin cancer in renal transplant recipients with multiple skin cancers and extensive epidermal dysplasia.
Br J Dermatol 1999 Apr
PMID:Skin cancer chemoprophylaxis in renal transplant recipients: 5 years of experience using low-dose acitretin. 1023 16

Xanthoma disseminatum (XD) is a rare benign non-X-histiocytic disorder of unknown aetiology. We report a 37-year-old man who presented with XD preceded by a decade of cranial diabetes insipidus, with associated type IIb hyperlipidaemia and computed tomographic evidence of hepatic involvement. A review of the literature is also included.
Clin Exp Dermatol 1998 Nov
PMID:Xanthoma disseminatum: a case with hepatic involvement, diabetes insipidus and type IIb hyperlipidaemia. 1023 26

We described a case of Werner's syndrome associated with osteosarcoma. A 37-year-old Japanese man was diagnosed as having Werner's syndrome by the presence of juvenile cataracts, skin sclerosis and hyperpigmentation of the feet, high-pitched voice, characteristic bird-like appearance of the face with beak-shaped nose, thinning of the entire skin and hyperkeratoses on soles, hyperlipemia, hyperuricemia, diabetes melitus, and the mutated responsible gene (WRN). He had a 3-month history of a tumor on his left forearm. Histologically, the tumor included four histological patterns; a malignant fibrous histiocytoma-like, a desmoid-like, a dermatofibrosarcoma protuberans-like, and a chondrosarcoma-like pattern. Tumoral osteoid formation was also found in the tumor. Therefore, the tumor was diagnosed as osteosarcoma.
J Dermatol 1999 Oct
PMID:A case of Werner's syndrome associated with osteosarcoma. 1055 36


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