Case Of Xanthoma Disseminatum Research Articles

The Importance of Clinicopathological Correlation: A Case of Xanthoma Disseminatum

Xanthoma disseminatum (XD) is a rare disease that presents with pruritic papules, classically involving the extensor surfaces and mucosal areas. We present a case of XD that was misdiagnosed as eruptive xanthomas and eventually self-resolved. Self-resolving XD may be within a spectrum of adult-onset juvenile xanthogranuloma. However, as there may be systemic consequences of XD, it is critical to ensure these patients obtain a complete evaluation and close follow-up.

A Case of Xanthoma Disseminatum with Spontaneous Resolution over 10Years: Review of the Literature on Long-Term Follow-Up

A Case of Xanthoma Disseminatum with Spontaneous Resolution over 10Years: Review of the Literature on Long-Term Follow-Up

Xanthoma Disseminatum

Xanthoma disseminatum is a rare nonfamilial disease characterized by lipid deposition in skin and internal organs due to histiocytic cell proliferation, classified as a benign non-Langerhans cell histiocytosis. This chronic disease has no known established treatment. We report 8 cases of xanthoma disseminatum with treatment outcomes in long-term follow-up and provide a review of the medical literature. We studied clinical manifestations; disease course; histopathologic and immunopathological findings; and responses to treatment in 8 consecutively seen patients with xanthoma disseminatum, including follow-up. The follow-up period ranged from 3 months to 8 years. Five patients received 2-chlorodeoxyadenosine. Therapy with 2-chlorodeoxyadenosine was effective at inducing remission and long-term control of cutaneous lesions of xanthoma disseminatum in 5 cases. Adverse effects were minimal, and treatment was well tolerated. No improvement was noted in untreated patients. Among 8 cases of xanthoma disseminatum, a positive response to treatment with 2-chlorodeoxyadenosine was seen in 5 cases.

Non-Langerhans Cell Histiocytoses

Based on our series of 111 cases of non-Langerhans cell histiocytoses, we present a new unifying concept for this rare group of disorders. The common denominator is the monocyte/ macrophage, which presents with various histologic features probably due to the influence of cytokines. Non-Langerhans cell histiocytoses are classified according to the predominant mononuclear (vacuolated, spindle-shaped, xanthomatized, scalloped, and oncocytic) and/or multinucleate (Touton, ground-glass appearance, Langhans, and foreign body) histiocytic cell types. Variable mixtures of these cell types produce common polymorphous patterns with prominence of vacuolated, spindle-shaped, and xanthomatized histiocytes in juvenile xanthogranulomas and of scalloped and oncocytic histiocytes in adult xanthogranulomas. Rarely, unusual monomorphous reaction patterns are observed: mostly vacuolated histiocytes are seen in the mononuclear variant of xanthogranulomas, (early benign cephalic histiocytosis, and generalized eruptive histiocytoma. Xanthomatized histiocytes predominate papular xanthoma and rarely xanthoma disseminatum, whereas spindle-shaped histiocytes are evident in spindle cell xanthogranuloma and progressive nodular histiocytosis, scalloped histiocytes are evident in most cases of xanthoma disseminatum, and finally oncocytic histiocytes are evident in reticulohistiocytoma and multicentric histiocytosis. Immunohistochemical, ultrastructural, and clinical findings can rationally be adjusted to this unifying concept of non-Langerhans cell histiocytoses. The time course of lesions, the age of the patients, and the presence or absence of underlying internal diseases are, or may, at least partially, be related to and thus explain variations on the theme of the non-Langerhans cell histiocytic reaction.

Bone Lesions in Xanthoma Disseminatum

A case of xanthoma disseminatum occurred in association with progressive bone lesions. This association has not been previously emphasized.

Xanthoma Disseminatum

A rare case of xanthoma disseminatum producing deformity of the head, neck, and extremities is reported. This pathologically benign disease, considered to represent one of the histiocyte proliferative disorders, is manifested by numerous cutaneous xanthomas primarily in flexion areas. Progressive enlargement of these lesions accompanied by marked fibrosis has produced the severe cosmetic and functional deformities described in this patient. Treatment in this case involved excision of xanthomatous contraction bands with split-thickness skin grafting, followed by intensive physical therapy.

Xanthome bei Normolipämie

A short summary of normolipemic xanthomatoses is exposed. A case of xanthoma disseminatum is reported. The typical clinical picture is described and numerous investigations are recorded. No association with any systemic disorder neither diabetes insipidus nor rheumatoid arthritis could be found. Histologically the granulomas consist of many multinuclear giant cells embedded in an infiltration of histiocytic cells and lymphocytes. Fat staining demonstrates lipid accumulation as a secondary phenomenon. Systemic corticosteroid therapy was effective in preventing the regrowth of the granulomas after their surgical removal.

Tissue lipid patterns in a case of xanthoma disseminatum.

Xanthoma disseminatum is a rare disease characterized by xanthomatous lesions composed of proliferating mature histiocytes, foam cells with high cholesterol content, eosinophils, Touton cells, and fibrosis. Plaquelike skin lesions are usually seen over flexor surfaces of joint areas with multiple organ systems involved. Serum cholesterol and other serum lipid fractions are typically normal. A case of xanthoma disseminatum in a Negro female with widespread xanthomatous involvement and the finding of associated diabetes mellitus with secondary hyperlipemia is presented as the first reported case in a Negro and as an illustration of the lipid analysis of xanthomatous lesions, including fatty acid distribution by the gas chromatographic technique. Report of a Case History.— First admission: A 33-year-old Negro housewife was admitted to the Medical College of Virginia Hospitals on July 12, 1960, with the history that 10 to 12 years prior to admission she had noted raised nontender pea-sized nodules of the