Xanthoma disseminatum (XD) is a rare benign mucocutaneous xanthomatosis that is classified as a benign non-Langerhans cell histiocytosis. We report a 62-year-old man who presented with widespread yellow-brown papulonodular and tumoral lesions on face, flexors, and trunk. Histopathological features of the cutaneous lesions were typical of XD.
Xanthoma disseminatum (XD) is a rare benign mucocutaneous xanthomatosis classified as a benign form of non-Langerhans cell histiocytosis [
A 62-year-old man was admitted to our department due to a gradually evolving disseminated papulonodular eruption on face, flexors, and trunk for 30 years ago. On examination, multiple, well-defined, yellowish brown papules and nodules and tumor-like lesions were seen symmetrically on upper and lower eyelids (Figure
Tumor-like lesions on periorbital area, the ulceration like lesion do to biopsy’s site.
Involvement of flexural regions of neck with papule that confluent to plaque lesion.
Hundreds of red to brown papules were distributed symmetrically on axilla, genitalia, poplitea, and trunk, some of which are confluent together and formed plaque or tumor-like lesions (Figures
Involvement of red-brown papules.
Papules, plaque, and tumor like lesions.
Involvement of nasal mucosa.
He had no history of polyuria or any previous medical history.
Analysis at that point did not reveal any abnormalities in urine osmolality. Laboratory findings revealed a white blood cell count of 10,300 cells/mL containing 74% neutrophils. C-reactive protein was 7 mg/dL (normal values of 0.5 mg/dL).
Fasting cholesterol was 148 (normal values up to 200) and triglyceride was 141 (normal values up to 150).
Chest X-ray was normal.
Magnetic resonance imaging of the brain and pituitary revealed no abnormalities.
Histopathologic examination of skin biopsies showed a dense dermal diffuse histiocytic infiltration interspersed with mixed inflammatory cells and giant cells (Figures
Dense dermal diffuse histiocytic infiltration.
A dense dermal diffuse histiocytic infiltration interspersed with mixed inflammatory cells and giant cells.
Foam cells were also seen.
Immunohistochemistry was positive.
Xanthoma disseminatum (XD) is a rare but distinct sporadic disorder, in which lipid deposition occurs secondary to a proliferation of histiocytic cells. This is usually seen before 25 years, as rarely reported in the elderly [
The etiology of XD is unknown. It has been suggested that XD represents a reactive proliferation of histiocytes with secondary accumulation of lipid. But it is not associated with hyperlipidemia.
Three patterns have been identified; the most common pattern is the persistent form. Rarely, lesions may regress spontaneously, and even more infrequently in the progressive form there may be significant internal organ involvement [
Mucous membrane involvements of XD have been reported in 40–60% of cases [
Meningeal involvement is common, leading to diabetes insipidus when infiltration at the base of the brain is present. This condition is encountered in about 40% of cases but usually is less severe than that associated with Langerhans cell disease. Characteristically, internal lesions other than diabetes insipidus are absent. Our patient did not have any sign or symptom of diabetes insipidus [
In a few instances multiple osteolytic lesions have been found, especially in the long bones, as well as lung and central nervous system infiltrates.
Histopathologically, in early lesions, scalloped macrophages dominate the histologic picture, with few foamy cells. Well-developed lesions may still show scalloped cells, but xanthomatization occurs in most cases. Most well-developed lesions contain a mixture of scalloped cells, foamy cells, and inflammatory cells, as well as Touton and foreign-body giant cells. XD histiocytes stain for lysozyme and aI-antitrypsin and also express CD68, CDllb, CD14, CDllc, and factor XIIIa [
The main differential diagnosis of XD is generalized eruptive histiocytosis (GEH) and progressive nodular histiocytosis (PNH). Multiple skin lesions occurring in adolescence or young adulthood with prominent involvement of flexural areas, as well as viscera and mucosa, and comprising mainly xanthomatous cells are XD; multiple lesions appearing in crops, generally sparing the flexures, and occurring in normolipemic patients are GEH, while multiple lesions arising in skin of an older patient and progressing to form large nodules, with no evidence of spontaneous regression and comprising mainly spindle-shaped histiocytes, are PNH [
We report this case for its unusual large tumor-like lesions around the eyes. In the literature, some authors have previously described cases of XD with eyelid or periocular accentuation of lesions [
There are various treatment modalities, like vasopressin, corticosteroids, antiblastic chemotherapy, radiotherapy, cryotherapy, CO2 LASER therapy, and surgical resection, used alone or in combination [
The authors declare that there is no conflict of interests regarding the publication of this paper.