Normolipemic papular xanthoma with xanthelasma
Atul Singla MD
Dermatology Online Journal 12 (3): 19

atulsingla14@rediffmail.com

Abstract

Xanthomas are a common presentation of disorders of lipid metabolism, usually associated with abnormalities of cholesterol metabolism. A 35-year-old woman presented to us with the lesions of xanthelasma on the upper eyelids and papular xanthomas on the rest of the body. Routine investigations and systemic examination were normal. The lipid profile was within normal range and serum protein electrophoresis showed normal pattern. Histopathology from a papular lesion showed foamy histiocytes with Touton giant cells. We present a case of normolipemic papular xanthoma with xanthelasma, a very rare occurrence.


Xanthomatosis is a widespread disorder in which localized infiltrates of the lipid-containing cells appear in the skin and visceral organs. They may or may not be associated with abnormal lipid metabolism [1]. Xanthelasma palpebrum is the most common cutaneous xanthoma. It occurs as bilaterally symmetrical yellow plaque most commonly on the upper eyelid near the inner canthus. This lesion may represent a localized cutaneous phenomenon or signify a systemic hyperlipidemia and be associated with elevation of low density lipoprotein (LDL) as in pure hypercholesterolemia (such as familial hypercholesterolemia, FH) or type-III hyperlipoproteinemia [2]. Papular xanthoma is a rare normolipemic non-Langerhans-cell histiocytosis affecting both children and adults. Clinically, it is represented by monomorphic papular eruption seen in the 13 to 57-year age group and has a biphasic occurrence in the young adolescence and in middle age. It is predominately located on the trunk, extremities, and rarely on the head. Histologically, it is characterized by a regular epidermis and a dense distribution of the xanthomitized macrophages interspersed by numerous Touton giant cells [3]. Papular xanthoma may be associated with a systemic disorder or may be a cutaneous manifestation of an underlying malignancy. We describe a female patient with xanthelasma and normolipemic papular xanthoma unassociated with systemic disease.


Clinical synopsis

A 35-year-old woman presented to the outpatient department of Dayanand Medical College & Hospital, Ludhiana, North India with a history of yellowish papular lesions on the arm, face, and upper trunk since age 6 years. The lesions first appeared as small, slightly elevated plaques that were well circumscribed on the upper eyelids near the inner canthi (Fig. 1). After 2 years, papular lesions appeared on the rest of the face, head, back, arms, legs, posterior part of the thighs, and axillae (Fig. 2). Except for the skin lesions the physical examination was normal. Routine investigations including complete hemogram and urinalysis were normal. Lipid profile was within normal limits . Serum bilirubin was normal and serum protein electrophoresis showed a normal pattern.


Figure 1 Figure 2
Pre-treatment

Figure 3
Histopathology of neck lesion demonstrating Touton giant cell

Biopsy of a skin lesion of the neck showed localized collection of histiocytes with foamy vacuolated cytoplasm, few lymphocytes and neutrophils in the dermis. Touton giant cells were also noted.

Based upon the clinical profile and histopathological changes, diagnosis of xanthelasma palpebrum with papular xanthoma was made. Repeat lipid profile after 1 month was essentially unchanged.

The patient was started on oral antioxidants. Trichloroacetic acid was applied to xanthelasma lesions and cryotherapy with nitrous oxide was done for papular xanthomas. The patient is responding well to the treatment. The skin lesions are healing and she continues to be followed (Figs. 4, 5).


Figure 4 Figure 5
Patient following treatment of xanthelasma and papular xanthoms

Discussion

We present a rare case of normolipemic papular xanthoma with xanthelasma in a young female. Xanthomas are lesions characterized by accumulation of lipid-laden macrophages. They can be a reflection of altered lipid metabolism or a result of local cell dysfunction. Xanthelasma palpebrum is the most common of the xanthomas and presents as asymptomatic, usually bilaterally symmetric soft, velvety, yellow, flat, polygonal papules around the eyelids. Xanthelasmas are most common in the upper eyelid near the inner canthus. Usually, the lesions evolve for several months and enlarge slowly from small papules. Xanthelasma may be associated with hyperlipidemia in which any type of primary hyperlipoproteinemia can be present. Some secondary hyperlipoproteinemias, such as cholestasis, may also be associated with xanthelasma [4]. Papular xanthomas are normolipemic, nonconfluent, eruptive xanthomas located on the face, trunk, and mucous membranes with no internal involvement. Histologically, they are characterized by foamy cells and Touton giant cells. Electron microscopy shows macrophages packed with free lipid vacuoles and lacking specific markers [5]. Papular xanthoma has been reported to be associated with systemic disorders and may be a cutaneous sign of internal malignancy. Diffuse normolipemic xanthomatosis have normal lipid levels but are often associated with serious hepatic disease or hematological dyscrasia, especially multiple myeloma [6]. Hu et al. reported two cases of unusual normolipemic cutaneous xanthomatosis [7]. One patient presented with widespread normolipemic papular xanthomas in which histiocytes containing Langerhans granules were found. Vail et al. reported a case of chronic myelomonocytic leukemia with cutaneous xanthomas [8]. Darwin et al. reported a case of generalized papular xanthoma in a patient with Sezary syndrome [9]. The xanthomas were composed of foamy histiocytes that were shown by immunoperoxidase staining to be of the monocyte/macrophage lineage. Goerdt et al. described a case of normolipemic papular xanthomatosis in a patient with long-standing erythrodermic atopic dermatitis [10]. Papular xanthoma has also been reported to be associated with angiokeratoma of Fordyce [11]. Some normolipemic xanthomatosis are found to be associated with either a systemic disease or malignancy. Our case showed xanthelasma with papular xanthomatosis but without any lipid disorder,associated systemic disease, or malignancy. Normolipemic eruptive cutaneous xanthomatosis has been reported previously also but its occurrence is very rare [1, 6]. In our case two types of xanthomas were seen in the same patient. Although papular xanthomas have been reported to involute spontaneously [1], in our case they were persistent for 6 years at the time when the patient first presented to us. This case is being reported because of its rare occurrence.

References

1. Caputo R, Monti M, Berti E, Gasparini G. Normolipemic eruptive cutaneous xanthomatosis Arch Dermatol. 1986 Nov;122(11):1294-97

2.Seymour C.A. Xanthomas and abnormalities of lipid metabolism and storage. In:Champion RH, Burton JL,Burns DA, Beathnach SM, editors Rook/ Wilkinson/ Ebling Textbook of Dermatology. 7th ed. Oxford: Blackwell Science; 2004 p.57.65 -57.67

3.Breier F, Zegler B, Reiter H, Gschnait F,Zegler BW. Papular xanthoma; a clincopathological study of 10 cases. J Cutan Pathol. 2002 Apr;29(4):200-6.

4.Horenstein MG. Xanthomas .www.medicine.com/derm

5.Bundino S, Zina AM, Aloi F Papular Xanthoma. Clinical, Histological and Ultra structural study. Dermatologica 1988; 177 (6): 382-85.

6. Rudolph RL. Diffuse "essential" normolipemic xanthomatosis. Int J Dermatol. 1975 Nov;14(9):651-56

7. Hu CH, Winkelmann RK. Unusual normolipidemic cutaneous xanthomatosis: a comparison of two cases illustrating the differential diagnosis. Acta Derm Venereol. 1977;57(5):421-29

8. Vail JT Jr, Adler KR, Rothenberg J. Cutaneous xanthomas associated with chronic myelomonocytic leukemia. Arch Dermatol. 1985 Oct;121(10):1318-20.

9. Darwin BS, Herzberg AJ, Murray JC, Oslen EA. Generalized papular xanthomatosis in mycosis fungoides. J Am Acad Dermatol. 1992 May;26(2):828-32.

10. Goerdt S, Kretzschmar L, Bonsmann G, Normolipemic papular xanthomatosis in erythrodermic atopic dermatitis J Am Acad Dermatol 1995 Feb;32(2):326-33

11. Caputo R, Passoni E, Cavicchini S. Papular xanthoma associated with angiokeratoma of Fordyce: considerations on the nosography of this rare non- Langerhans cell histiocytoxanthomatosis. : Dermatology. 2003;206(2):165-68.

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Title:

Normolipemic papular xanthoma with xanthelasma

Journal Issue:

Dermatology Online Journal, 12(3)

Author:

Singla, Atul

Publication Date:

2006

Publication Info:

Dermatology Online Journal, UC Davis

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