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

Results

Articles reporting on a total of 224 patients with IVL were reviewed. In articles before 1986 and many thereafter no differentiation is made by the authors between B-cell and T-cell lymphoma (105 patients).[1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,29,30,36,49,51,53,77,92] Beginning in 1986, authors increasingly included immunohistochemical data of the patients studied by them.[19,20,23,25] The majority of patients had IVL-BCL (86 patients)[31,32,34,35,38,39,40,42,47,50,53,55,56,57,58,60,61,62,64,67,68,73,74,77,78,80,82,83,86,87,88,89,93,95,97] whereas a minority had IVL-TCL (33 patients).[25,37,40,44,45,47,48,52,53,54,57,59,63,66,69,71,72,73,75,78,79,81,91,95,96,97]

In 59 patients no signs and symptoms of the skin were mentioned, and in another 75 patients authors stated specifically that the skin had been uninvolved in the lymphomatous process. In 90 of 224 patients, the disease presented with lesions in the skin; nine of these had insufficient data to be assessed in detail in this review.[78,92,97] The remaining 81 patients comprised 47 women and 34 men, mean age 60 years. Twenty-nine had lesions restricted to the skin,[1,3,16,22,25,26,27,40,49,50,54,57,60,68,74,75,87,89,92] whereas in the other 52 patients organs other than the skin were also involved. In 26 of these 52 patients, lesions in the skin preceded those in other organs[5,7,8,9,10,11,13,21,24,26,30,34,37,39,40,44,48,60,61,64,66,69,93] whereas in the remaining 26, signs and symptoms of other organs preceded the appearance of lesions in the skin.[2,6,17,19,20,24,26,34,36,38,42,51,52,54,56,57,58,81,82,88]

Skin manifestations were said to be localized in decreasing order of frequency on the thigh (41%), leg (35%), trunk (31%), arm (15%) and buttock (7·5%). When the thighs were affected, lesions were mentioned to have favoured the anteromedial aspect in nine patients.[16,21,27,30,34,39,40,60,88] Lesions were said to have involved both sides of the body in 47 patients.[1,3,5,7,8,9,13,16,21,25,30,34,37,40,42,44,45,48,49,50,52,54,56,58,60,61,64,68,69,72,74,82,87,88,89,93]

Most commonly lesions were described as being nodules and/or plaques (49%); macules were encountered in 22·5% of the patients. Lesions were said to be indurated or firm in 27·5% of the patients. Mention was made of prominent telangiectases in only 20% of the patients. The colour of lesions was described as red in 31% of the patients, blue to livid in 19% and brown to grey in 9%, no colour of lesions being given in the remaining 41%. A frequently accompanying sign was oedema of the legs, being mentioned in 27·5% of the patients. Pain was a relatively common complaint (24%). Lesions were reported to have been waxing and waning in 16% of the patients. Associated findings such as fever, malaise and weakness were described in 47·5% of the patients.

Comparison of clinical features in patients with lesions restricted to the skin with those in whom the disease involved other organs after or before lesions were manifest in the skin is presented in Table 1 . No significant differences between the three groups could be determined in regard to the clinical presentation of skin lesions.

Photographs of clinical lesions were included for only 32 patients,[1,2,3,7,9,10,13,16,19,21,22,24,27,30,34,37,39,45,48,49,50,52,60,66,68,74,82,87,89] 10 of those having IVL-BCL,[34,39,50,60,68,74,82,87,89] six IVL-TCL[37,45,48,52,66] and 16 not being characterized as either TCL or BCL.[1,2,3,7,9,10,13,16,19,21,22,24,27,30,59] Figure 2 (a–d) shows clinical lesions of patients with IVL from articles before 1986, when immunophenotyping was not performed. Figure 3(a–k) shows clinical lesions in patients with proven IVL-BCL (including one patient of our own). In Figure 4(a, b) lesions of patients with proven IVL-TCL are presented.

Figure 2.

(a–d) Clinical lesions of patients with IVL from articles before 1986, when immunophenotyping was not performed. (a) Reproduced from Pfleger L, Tappeiner J. 19591, with kind permission of Springer Science and Business Media Copyright© (1959). All Rights reserved; (b) reproduced with kind permission from Fievez M, Fievez C, Hustin J,7Copyright© (1971), American Medical Association. All Rights reserved; (c) reproduced with kind permission from Bhawan J, Wolff SM, Ucci AA, Bhan AK,19Copyright© (1985), Wiley & Sons, Ltd.; (d) reproduced with kind permission from Keahey TM, Guerry D, Tuthill R et al.,16Copyright© (1982), American Medical Association. All Rights reserved.

Figure 3.

(a–k) Clinical lesions in patients with proven IVL-BCL. (a) Reproduced with kind permission from Chang AB, Zic JA, Boyd AS,74Copyright© (1998), Elsevier Health Sciences. All Rights reserved; (b) reproduced from Williams REA, Seywright MM, Lever R, Lucie NP. 199039; (c) reproduced with kind permission from Perniciario C, Winkelmann RK, Daoud MS, Su WPD,60Copyright© (1995), Lippincott Williams & Wilkins. All Rights reserved; (d) reproduced with kind permission from Asagoe K, Fujimoto W, Yoshino T et al. 2003,88Copyright© (2003), Elsevier Health Sciences. All Rights reserved; (e) patient of our own; (f, g) reproduced with kind permission from Wilson BB,50Copyright© (1992), American Medical Association. All Rights reserved; (h, i) reproduced with kind permission from Erös N, Karolyi Z, Kovacs A et al. 2002,87Copyright© (2002), Elsevier Health Sciences. All Rights reserved; (j) reproduced from Özgüroglu E, Büyülbabani N, Özgüroglu M, Baykal C. 199768; (k) reproduced with kind permission from Petroff N, Koger OW, Fleming MG, Fishleder A et al.,34Copyright© (1989), Elsevier Health Sciences. All Rights reserved.

Figure 4.

(a, b) Clinical lesions of patients with proven IVL-TCL. (a) Reproduced with kind permission from Lopez-Gil F, Roura M, Umbert I, Umbert P. 1992,48Copyright© (1992), Elsevier Health Sciences. All Rights reserved; (b) reproduced with kind permission from Sepp N, Schuler G, Romani N et al. 1990,37Copyright© (1990), Elsevier Health Sciences. All Rights reserved.

Comparison of sure cases of IVL-BCL and IVL-TCL is provided in Table 2 . It did not reveal any major differences between both groups, an exception being the involvement of the face and the anogenital area in four patients with IVL-TCL.[37,40,57,66] Moreover, patients with IVL-TCL seemed to be younger at the time of diagnosis (mean 53 years, range 17–84) compared with patients with IVL-BCL (mean 66 years, range 54–84).[45,81]

Data on treatment and follow-up were available for 81 patients.[1,2,3,6,7,8,9,10,11,13,16,20,22,24,25,30,34,36,37,42,44,45,48,50,51,52,54,56,57,58,60,61,64,66,69,72,75,81,88,89,92,93] The vast majority of patients received combination chemotherapy such as CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone), and some received radiation treatment either as a single treatment or in combination with chemotherapy. Death from IVL that presented with lesions in the skin was explicitly mentioned in 42 patients and is summarized in Table 3 . Whereas patients with lesions restricted to the skin continued to do well during a follow-up period ranging from 3 months to 14 years, the majority of patients who had lesions in the skin together with lesions of IVL in other organs died within 2 years after the diagnosis of the disease. Patients with IVL-TCL compared with patients with IVL-BCL had a slightly worse outcome based on the few cases available for comparison (69% vs. 52% fatal outcome).

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