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Skin-Coloured Papules on Elbows and Knees with Foamy Cells

 Venkatcenter Bangalore, Karnataka, India

Date of Web Publication24-Jan-2020

Correspondence Address:
Venkatram Mysore,
Venkatcenter, #3437, 7th Main Road, 1st G Cross, Subbanna Garden, Vijayanagar, Bengaluru - 560 040, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/idoj.IDOJ_295_18

How to cite this URL:
Kantamuneni U, Kayarkatte MN, Mysore V. Skin-Coloured Papules on Elbows and Knees with Foamy Cells. Indian Dermatol Online J [Epub ahead of print] [cited 2020 Feb 17]. Available from: http://www.idoj.in/preprintarticle.asp?id=276571

A young female aged 36 yrs presented with complaints of multiple asymptomatic papules on the extensor surface of bilateral elbows and knees since 3 months. She also gave history of red raised small lesions on bilateral lower 1/3rd of the legs since 7 days. There was no history of fever, sore throat, or pain in the abdomen. She further informed about four previous episodes of similar red raised lesions on legs in the past 1 year, which had subsided with some tablets.

On examination, multiple skin colored to yellowish flat papules were noted in the aforementioned sites [Figure 1]. The papules were mildly tender, firm, and diascopy did not yield apple-jelly appearance. The lower third of legs [Figure 2] showed palpable erythematous purpuric lesions without any blanching. There were no other significant cutaneous findings. Systemic examination was normal. Biopsy was performed from the erythematous lesions on the legs [Figure 3] and skin colored papules on the knee [Figure 4] and [Figure 5].
Figure 1: Skin coloured to yellowish papules on the extensors of the joints

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Figure 2: Palpable purpura on bilateral legs

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Figure 3: Histopathology from the palpable purpuric lesion of leg at 10× magnification showing features of leucocytoclastic vasculitis (H and E)

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Figure 4: Histopathology from the yellowish papule on knee at 10× magnification showing vessel wall damage, infiltration of vessel wall by neutrophils, and a perivascular infiltrate of neutrophils and few lymphocytes and occasional eosinophil. Extravasation of RBCs and nuclear dust are noted. (H and E)

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Figure 5: Histopathology from the yellowish papule on knee at 40× magnification showing foamy cells around blood vessels amidst few lymphocytes and polymorphs in the vicinity of the vessel. (H and E)

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Histopathology of the lesion from the leg showed features of leucocytoclastic vasculitis [Figure 3]. The biopsy from skin colored lesion on knee displayed vessel wall damage, infiltration of vessel wall by neutrophils, and a perivascular infiltrate of neutrophils and few lymphocytes and occasional eosinophils. Extravasation of RBCs and nuclear dust were noted. Interestingly, an admixture of foam cells amidst the infiltrate near blood vessels is noted [Figure 4]. On high power view (40X), foamy cells around blood vessels amidst few lymphocytes and polymorphs in the vicinity of the vessel was confirmed [Figure 5].

   Question Top

What is your diagnosis?

   Answer Top

Final Diagnosis: Leukocytoclastic vasculitis-Erythema elevatum diutinum (EED) with extracellular cholesterolosis.

   Discussion Top

EED is a distinctive form of chronic cutaneous vasculitis, presenting as persistent, symmetrical, firm, tender, and red to brown papules or nodules that may coalesce to form plaques. They are commonly found on the extensor surfaces especially near joints such as the fingers, hands, elbows, ankles, and knees. After partial resolution, lesions may resemble xanthomata because of a yellowish or brown hue of the lesions.[1]

   Histopathologic Features Top

In the early stage, nonspecific leukocytoclastic vasculitis is observed. On low power, a busy dermis with a mild superficial and dense perivascular inflammatory infiltrate is observed. On further magnification, RBC extravasation, leukocytoclasis (neutrophil degeneration) forming nuclear dust and fibrinoid necrosis of the vessels can be noted. Chronic lesions show a mixture of lymphocytes, plasma cells and macrophages along with the hemorrhagic, fibrinoid leukocytoclastic vascular changes. Nodular lesions may show areas of fibrosis and capillary proliferation within the inflammatory lesion. The capillaries may show deposits of fibrinoid material or merely fibrous thickening.[2] Lipid material also may be present as cholesterol clefts in old, fibrotic lesions of EED. Such cases have been documented as extracellular cholesterolosis in EED.[3],[4],[5]

EED may be associated with underlying bacterial infections or paraproteinemia. The drug of choice for treating EED is dapsone. Other treatments tried are colchicine, methotrexate, oral, and intralesional steroids. Our patient was treated with dapsone to which the patient responded well. Cases of extracellular cholesterolosis have become a rarity in recent years as EED is easily treatable with dapsone.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent form for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity.

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Conflicts of interest

   References Top

Gibson LE, el-Azhary RA. Erythema elevatum diutinum. Clin Dermatol 2000;18:295-9.  Back to cited text no. 1
Yiannias JA, Winkleman R. The classic angiovasculitis lesion of erythema elevatum diutinum. J Cutan Pathol 1992;192:558.  Back to cited text no. 2
Wilkinson SM, English JSC, Smith NP. Erythema elevatum diutinum: A clinicopathological study. Clin Exp Dermatol 1992;17:87-93.  Back to cited text no. 3
LeBoit PE, Benedict Yun TS, Wintroub B. The evolution of lesions in erythema elevatum diutinum. Am J Dermatopathol 1986;8:392-402.  Back to cited text no. 4
Kanitakis J, Cozzani E, Lyonnet S, Thivolet J. Ultrastructural study of chronic lesions of erythema elevatum diutinum: “Extracellular cholesterosis” is a misnomer. J Am Acad Dermatol 1993;29:363-7.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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