Necrobiotic xanthogranuloma without paraproteinaemia: marked improvement with psoralen ultraviolet A treatment.

Necrobiotic xanthogranuloma (NXG) is a rare granulomatous condition that is often associated with a paraproteinaemia and in some cases multiple myeloma. Treatment is therefore aimed at the underlying associated haematological abnormality. However, isolated NXG cases have been reported. We report a case of isolated NXG that responded very well to systemic psoralen ultraviolet A (PUVA) treatment. The rationale for this treatment was the successful use of PUVA treatment in other necrobiotic conditions such as granuloma annulare and necrobiosis lipoidica.

Antiatherogenic property of triterpenoids-enriched extract from the aerial parts of Salvia miltiorrhiza.

This study investigated the antiatherogenic activity and the chemical constituents of a triterpenoids-enriched extract from the aerial parts of Salvia miltiorrhiza Bunge. The extract displayed a significant, dose-responsive antiatherogenic effect on LDLR(-/-) mice after a 24-week application in a daily dose 50, 100, 200, 1000 mg/kg, respectively. Pronounced systemic inflammation and cutaneous xanthomatosis inhibition effects of the extract, combined with hypocholesteremic and plaque stable properties, were shown in this study. At 200 mg/kg in the high dose treated group, the percent inhibitions of two inflammatory markers in serum, CRP and MCP-1, were 47.6 +/- 4.2% and 36.8 +/- 5.0%, and significant reductions in aortic atherosclerotic lesion areas were 62.3 +/- 3.9% (en face) and 77.8 +/- 3.1% (cross section) compared with the model group. No abnormal behavior or lethality were observed with the extract up to 1000 mg/kg. Fifteen triterpenoids were separated and identified from the extract. This study provided new evidence of the antiatherogenic property of S. miltiorrhiza correlated with triterpenoids by an antiinflammatory mechanism.

Lapaquistat acetate, a squalene synthase inhibitor, changes macrophage/lipid-rich coronary plaques of hypercholesterolaemic rabbits into fibrous lesions.

BACKGROUND AND PURPOSE: Inhibition of squalene synthesis could transform unstable, macrophage/lipid-rich coronary plaques into stable, fibromuscular plaques. We have here treated WHHLMI rabbits, a model for coronary atherosclerosis and myocardial infarction, with a novel squalene synthase inhibitor, lapaquistat acetate (TAK-475). EXPERIMENTAL APPROACH: Young male WHHLMI rabbits were fed a diet supplemented with lapaquistat acetate (100 or 200 mg per kg body weight per day) for 32 weeks. Serum lipid levels were monitored every 4 weeks. After the treatment, lipoprotein lipid and coenzyme Q10 levels were assayed, and coronary atherosclerosis and xanthomas were examined histopathologically or immunohistochemically. From histopathological and immunohistochemical sections, the composition of the plaque was analysed quantitatively with computer-assisted image analysis. Xanthoma was evaluated grossly. KEY RESULTS: Lapaquistat acetate decreased plasma cholesterol and triglyceride levels, by lowering lipoproteins containing apoB100. Development of atherosclerosis and xanthomatosis was suppressed. Accumulation of oxidized lipoproteins, macrophages and extracellular lipid was decreased in coronary plaques of treated animals. Treatment with lapaquistat acetate increased collagen concentration and transformed coronary plaques into fibromuscular plaques. Lapaquistat acetate also suppressed the expression of matrix metalloproteinase-1 and plasminogen activator inhibitor-1 in the plaque and increased peripheral coenzyme Q10 levels. Increased coenzyme Q10 levels and decreased very low-density lipoprotein cholesterol levels were correlated with improvement of coronary plaque composition. CONCLUSION AND IMPLICATIONS: Inhibition of squalene synthase by lapaquistat acetate delayed progression of coronary atherosclerosis and changed coronary atheromatous plaques from unstable, macrophage/lipid accumulation-rich, lesions to stable fibromuscular lesions.

Normolipemic plane xanthoma associated with adenocarcinoma and severe itch.

Normolipemic plane xanthomas are yellow-red-colored flat patches or plaques with barely palpable borders, under normolipemic conditions usually involving the eyelids, the lateral sides of the neck, the upper aspect of the trunk, or the flexural folds. Histologically the lesions are characterized by an infiltrate consisting of foamy macrophages in the papillary and middermis with a distinct perivascular localization. Plane xanthoma has been associated with monoclonal gammopathy, cryoglobulinemia, and myeloproliferative disorders. We present a patient in whom plane xanthoma developed on the upper aspect of the back, which was accompanied by severe itch in the affected area. These symptoms started 1 month after resection of an adenocarcinoma of the rectum that was complicated by recurrent abdominal abscesses and, currently, by ongoing inflammatory bowel disease. A hypothetic pathophysiologic scheme of events leading to xanthoma formation in this patient is presented.

Treatment of xanthelasma by excision with secondary intention healing.

Twenty-eight patients with cosmetically compromising xanthelasma were treated by excision of the involved skin, the areas then being allowed to heal by secondary intention. All patients had a good cosmetic-result, those having previously undergone treatment with trichloroacetic acid chemical peels considering the new method more effective in clearing their lesions despite increased treatment morbidity in the first few weeks. Complications included recurrence in two patients, easily dealt with by re-treatment, and scarring in one patient, which settled with massage and was not cosmetically compromising. It is suggested that treatment of xanthelasma by excision and secondary intention healing is an inexpensive, effective and safe treatment for this common cosmetic problem.

Verruciform xanthoma in a psoriatic patient under PUVA therapy.

A patient with psoriasis vulgaris developed verruciform xanthoma (VX) on the scrotum during psoralen photochemotherapy (PUVA). Although it is uncertain whether VX was induced by PUVA therapy, we report the first case of VX which appeared in a patient with psoriasis during PUVA therapy. We speculate that UV light may be one of the etiologic factors triggering VX in this case.

Magnetic resonance imaging of Achilles tendon xanthomas in familial hypercholesterolemia.

The demonstration of tendon xanthomas is helpful in diagnosing heterozygous familial hypercholesterolemia, but in many patients lipid may accumulate without clinical abnormality being present. We investigated the possibility of detecting the lipid element with magnetic resonance (MR) imaging in seven patients with familial hypercholesterolemia and six controls. Although the mean relative signal intensities measured on long TR/TE spin echo sequences of the tendon were significantly higher in patients than in controls, the lack of such elevation does not rule out the presence of such lesions. In vitro measurements indicated that the signal intensity of triglycerides was quenched by cholesterolesters. The anatomic findings of MR imaging were compared with those of ultrasonography (US), showing excellent correlation in measurements between MR images and US [r(S) = 0.95 and 0.97 respectively]. MR imaging and US provide equal information on the anatomy of the Achilles tendon; as an abnormally increased signal intensity within the xanthoma on MRI was found in only a minority of our patients, the value of MRI in the demonstration of Achilles tendon xanthomas is limited when using conventional T1 and T2 spin echo sequences.

Xanthogranulomatous abscess of a mullerian duct remnant. A rare lesion of the rectum and anus.

A rare example of xanthogranulomatous inflammatory mass and abscess of a Mullerian duct remnant involving the anorectal area is reported. A barium enema showed a bilobed precoccygeal mass of moderate size involving the posterior aspect of the distal rectum. Computed tomography (CT) showed two nonfatty, round masses, one of which contained a small central cystic area. The other mass had a homogeneous appearance and was believed to be in the wall of the rectum. The pathologic specimen showed organizing abscesses and a chronic xanthogranulomatous inflammation in tissue compatible with urogenital tissue, presumably a Mullerian duct remnant. This is the first documented report of anorectal xanthogranulomatous abscess in a Mullerian duct remnant with radiologic findings and histopathologic correlation. Though rare, this lesion should be considered in the differential diagnosis of extrinsic and intramural rectal masses seen on barium enema and CT examinations.

[Posterior fossa syndrome terminating the course of histiocytosis X. Study of lesions of the central nervous system. Association with polyvinylpyrrolidone thesaurismosis]. / Syndrome de la fosse postérieure terminant l'évolution d'une histiocytose X. Etude des lésions du système nerveux central. Association d'une thésaurismose à la polyvinyl-pyrrolidone

The authors report the pathological and clinical findings in a case of histiocytosis X in a child. A rapidly progressive neurological syndrome of brain stem damage, occurred 5 years after the onset. Blockage of the 3rd ventricle led to surgical exploration of the posterior fossa. Lesions of the brain stem and cerebellum, in addition to the granuloma of the hypothalamus, are described. The neurological symptoms in histiocytosis X often suggest a space-occupying lesion but usually occur in the later prolonged forms in the adult. Association of thesaurismosis due to P.V.P., of therapeutic origin, produced numerous clinical skin and joint signs.