Skin Manifestations of Intravascular Lymphoma Mimic Inflammatory Diseases of the Skin

J. Röglin; A. Böer

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

Materials and Methods

Reports in the literature were reviewed to identify patients with IVL who had skin manifestations of the disease. Articles were retrieved via Medline search for the terms 'intravascular lymphoma' and 'angioendotheliomatosis' and from references listed in those articles. Descriptions of clinical lesions were analysed for features in common among them in regard to site of involvement, colour of lesions, shape and consistency of lesions, and associated symptoms. Lesions of patients who presented only with skin lesions were compared with those in patients who also had involvement of other organs in order to identify differences between both groups. Lesions of patients with proven IVL-BCL were compared with those encountered in proven IVL-TCL. Figures published in articles were collected by us and are reproduced here in order to show the morphological spectrum of the disease.

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Table 1. Comparison of Clinical Lesions in Patients With or Without Involvement of Organs Other Than The Skin
	Lesions restricted to the skin (n = 29)	Skin lesions preceding lesions in other organs (n = 26)	Lesions in other organs preceding lesions in the skin (n = 26)	Total (n = 81)	%
Site involved
Trunk	8	12	5	25	31
Buttock	2	2	2	6	7·5
Thigh	14	13	6	33	41
Leg	10	13	5	28	35
Arm	3	4	5	12	15
Others	2	2	2	6	7·5
Type of lesions
Nodules/ plaques	14	17	8	39	49
Macules	9	2	7	18	22·5
Telangiectases	5	7	4	16	20
Induration	12	6	4	22	27·5
Colour of lesions
Red	7	8	11	25	31
Blue/livid	6	6	3	15	19
Grey/brown	2	4	1	7	9
Oedema	11	8	3	22	27·5
Pain	12	7	0	19	24
Lesions waxing and waning	5	7	1	13	16
Fever/malaise	10	14	14	38	47·5

Table 2. Reported Patients With Intravascular Lymphoma (IVL) and Lesions in The Skin. Comparison of Patients With Proven B-cell Lymphoma (BCL) With Those With T-cell Lymphoma (TCL)
	IVL-BCL	IVL-TCL	Differentiation not known	Total
Lesions restricted to the skin	8	6	15	29
Skin lesions preceding lesions in other organs	6	7	13	26
Lesions in other organs preceding lesions in the skin	11	3	12	26
Total	25	16	40	81
Pictures of lesions available	10	6	16	32

Table 3. Patients Reported to Have Died From Intravascular Lymphoma (IVL) That Presented With Lesions In The Skin. Comparison of Patients With Proven B-cell Lymphoma (BCL) With Those With T-cell Lymphoma (TCL) and Comparison of Patients With Lesions Restricted to The Skin With Those in Whom Lesions in The Skin Preceded Involvement of Other Organs and With Those in Whom Lesions in The Skin Appeared After Involvement of Organs Other Than The Skin.
	IVL-BCL	IVL-TCL	Differentiation not known	Total	Percentage
Lesions restricted to the skin	1	2	0	3 (of 29)	10%
Skin lesions preceding lesions in other organs	3	6	8	17 (of 26)	65%
Lesions in other organs preceding lesions in the skin	9	3	10	22 (of 26)	85%
Total	13 (of 25)	11 (of 16)	18 (of 40)	42 (of 81)	52%
Percentage	52%	69%	45%	52%

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