Skin Manifestations of Intravascular Lymphoma Mimic Inflammatory Diseases of the Skin

J. Röglin; A. Böer

Disclosures

The British Journal of Dermatology. 2007;157(1):16-25. 

In This Article

Summary and Introduction

Summary

Background: Intravascular lymphoma (IVL) is fatal when it is diagnosed late in the course. Sometimes skin lesions enable early diagnosis, but criteria for diagnosis are not well established.
Objectives: To demonstrate the clinical spectrum of skin lesions of IVL and to correlate it with clinical outcome; to identify features differentiating between B-cell and T-cell IVL with skin involvement.
Methods: Review of 97 articles reporting on total of 224 patients with IVL.
Results: Skin lesions were mentioned in 90 of 224 patients. They were nodules and/or plaques (49%) or macules (22·5%) of red (31%) or blue to livid (19%) colour on the leg (35%), the thigh (41%) and the trunk (31%). Telangiectases were present in only 20% of the patients. Oedema (27·5%) of the legs and pain (24%) were often accompanying. No criteria enabled distinction between lesions restricted to the skin and skin lesions concurrent with IVL in other organs, but when the disease was restricted to the skin, the prognosis was favourable (10% vs. 85% fatal outcome). Skin lesions of T-cell IVL are indistinguishable from those of B-cell IVL.
Conclusions: Forty per cent of all patients with IVL have skin lesions, these being red, sometimes painful plaques located typically on the lower extremities, accompanied by oedema. A clinician risks misinterpreting these changes as thrombophlebitis, erythema nodosum or erysipelas. Neither clinical course nor differentiation of the lymphoma can be predicted from the morphology of skin lesions, but involvement of other organs at the time of diagnosis indicates a poor prognosis.

Introduction

Intravascular lymphoma (IVL) was described first in 1959 by Pfleger and Tappeiner under the designation 'Angioendotheliomatosis proliferans systemisata'.[1] The disease was for long considered to be a malignant neoplasm of endothelial differentiation[2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18] but immunohistochemical studies finally revealed the lymphocytic differentiation of the proliferations of atypical cells within vessels.[19,20] Histopathological findings are stereotypical and consist of infiltrates of large blasts within vessels of the superficial and deep plexus in the dermis sometimes also involving vessels in the upper part of the subcutaneous fat (Fig. 1). Reactive inflammatory infiltrates and haemorrhage as well as various amounts of haemosiderophages may also be present.

Figure 1.

Findings histopathologic of IVL: Infiltrates of large blasts within vessels of the superficial and deep plexus in the dermis, sometimes also involving vessels in the subcutaneous fat. In this patient, blasts were CD20 positive, proving IVL-BCL.

Intravascular lymphoma is a rare disease that often has a fatal course because patients do not present with signs and symptoms that allow a correct diagnosis to be made before the disease is well advanced.[1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91,92,93,94,95,96,97] Sometimes, however, skin lesions enable a diagnosis to be made early, but diagnostic criteria for such lesions are not well established, patients being reported almost exclusively in the form of case reports.

Several authors of reports on IVL included a review of patients published prior to their own work,[12,13,14,26,43,57,58,63,73,92,96,98,99,100] but none of them focused specifically on the clinical appearance of lesions in the skin, a reason for that being that authors of these articles were mainly oncologists, neurologists and pathologists, but not dermatologists.

Recently, we diagnosed one patient of our own with IVL. The patient, a man aged 59 years, had presented with recurrent erythema tender to the touch on the inner aspects of the thighs and the legs. For several weeks he had been misdiagnosed clinically as having thrombophlebitis migrans, when a biopsy specimen taken to exclude chronic erysipelas finally revealed the correct diagnosis. Figure 1 shows photomicrographs from a biopsy taken from our patient. Large blasts were seen in the lumen of medium-sized vessels in the subcutis. Blasts were negative for T-cell markers CD3, CD4, CD8 and CD30 but were positive for the B-cell marker CD20. IgH rearrangement studies by polymerase chain reaction revealed a clonal B-cell population.

Our patient prompted us to undertake a review of 224 patients published in the literature,[1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91,92,93,94,95,96,97] with the aim of improving criteria for clinical diagnosis of skin lesions in IVL. Clinical appearance was evaluated on the basis of course and outcome of the disease in order to identify morphological characteristics of prognostic value. Additionally, we attempted to identify criteria differentiating between B-cell (IVL-BCL) and T-cell (IVL-TCL) variants of the disease, when presenting with lesions in the skin.

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