Atypical Cutaneous Lymphoproliferative Disorder: A Fatal Mimic of Cutaneous T-Cell Lymphoma in a Patient with HIV Infection


Atypical cutaneous lymphoproliferative disorder (ACLD) is a rare condition that has been associated with HIV infection. Patients with ACLD present with diffuse, erythematous and pruritic skin lesions accompanied by generalized lymphadenopathy. The clinical characteristics of ACLD overlap most notably with several other conditions including Mycosis Fungoides/Sézary Syndrome (MF/SS), a cutaneous lymphoma of T-cell lineage. Unlike Mycosis Fungoides, the noxious infiltrates of ACLD are not monoclonal but polyclonal and consist of cytotoxic CD8+ T-cells instead of CD4+ T-cells or B-cells. Highly active antiretroviral therapy (HAART) has been reported to improve ACLD. We describe the case of a Caucasian man with longstanding HIV infection who presented with severe erythroderma. Skin and lymph node biopsies showed polyclonal CD8+ T-cell infiltrates. Gene rearrangement studies did not reveal an obvious clonal disorder. Hallmark peripheral blood findings consisting of a severe depletion of CD4+ T-lymphocytes and markedly elevated CD8+ cells provided an important diagnostic clue. Despite the purported benefits of HAART in ameliorating this disorder, erythroderma and extreme pruritus improved only after the patient began taking mycophenolate mofetil and hydroxyurea. Unfortunately, he succumbed to complications of methicillin-resistant Staphylococcus aureus septicemia. We alert readers to this rare HIV-associated condition which may mimic other benign and malignant skin conditions and briefly discuss diagnostic and therapeutic options.

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V. Nguyen, R. Dorer and D. Aboulafia, "Atypical Cutaneous Lymphoproliferative Disorder: A Fatal Mimic of Cutaneous T-Cell Lymphoma in a Patient with HIV Infection," World Journal of AIDS, Vol. 3 No. 1, 2013, pp. 10-15. doi: 10.4236/wja.2013.31002.

Conflicts of Interest

The authors declare no conflicts of interest.


[1] H. Bachelez, F. Hadida, C. Parizot, et al., “Oligoclonal Expansion of HIV-Specific Cytotoxic CD8 T Ly-mphocytes in the Skin of HIV-1-Infected Patients with Cutaneous Pseudolymphoma,” The Journal of Clinical Investigation, Vol. 101, No. 11, 1998, pp. 2506-2516. doi:10.1172/JCI1450
[2] E. Sbidian, M. Battistella, J. Rivet, H. Bachelez, et al., “Remission of Severe CD8+ Cytotoxic T Cell Skin Infiltrative Disease in Human Immunodeficiency Virus-Infected Patients Receiving Highly Active Antiretroviral Therapy,” Clinical Infectious Diseases, Vol. 51, No. 6, 2010, pp. 741-748. doi:10.1086/655895
[3] T. A. Longacre, K. Foucar, F. Koster and W. Burgdorf, “Atypical Cutaneous Lymphoproliferative Disorder Resembling Mycosis Fungoides in AIDS,” The American Journal of Dermatopathology, Vol 11, No. 5, 1989, pp. 451-456. doi:10.1097/00000372-198910000-00007
[4] S. Itescu, J. Dalton, H. Z. Zhang and R. Winchester, “Tissue Infiltration in a CD8 Lymphocytosis Syndrome Associated with Human Immunodeficiency Virus-1 Infection Has the Phenotypic Appearance of an Antigenically Driven Response,” Journal of Clinical Investigation, Vol. 91, No. 5, 1993, pp. 2216-2225. doi:10.1172/JCI116448
[5] R. G. Egbers, T. T. Do, L. Su, Y. R. Helfrich, J. E. Gudjonsson, “Rapid Clinical Change in Lesions of Atypical Cutaneous Lymphoproliferative Disorder in an Hiv Patient: A Case Report and Review of the Literature,” Dermatology Online Journal, Vol. 17, No. 9, 2011, p. 4.
[6] J. R. Golbus, G. Gallagher, G. Blackburn and S. Cinti, “Polyneuropathy Associated with the Diffuse Infiltrative Lymphocytosis Syndrome,” Journal of the International Association of Providers of AIDS Care, Vol. 11, No. 4, 2012, pp. 223-226. doi:10.1177/1545109712442798
[7] D. Basu, F. M. Williams, C. W. Ahn and J. D. Reveille, “Changing Spectrum of the Diffuse Lymphocytosis Syndrome,” Arthritis Care and Research, Vol. 55, No. 3, 2006, pp. 466-472. doi:10.1002/art.21980
[8] P. F. Levay and M. E. Motes, “Diffuse Infiltrative Lymphocytosis Syndrome (DILS),” South African Family Practice, Vol. 50, No. 2, 2008, pp. 42-44.
[9] S. Friedler, M. T. Parisi, E. Waldo, R. Wieczorek, G. Sidhu and M. J. Rico, “Atypical Cutaneous Lymphoproliferative Disorder in Patients with HIV Infection,” International Journal of Dermatology, Vol. 38, No. 2, 1999, pp. 111-118. doi:10.1046/j.1365-4362.1999.00417.x
[10] W. D. James, R. R. Redfield, G. P. Lupton, et al., “A Papular Eruption Associated with Human T Cell Lymphotropic Virus Type III Disease,” Journal of the American Academy of Dermatology, Vol. 13, No. 4, 1985, pp. 563-566. doi:10.1016/S0190-9622(85)70197-1
[11] N. S. Sadick and N. S. McNutt, “Cutaneous Hypersensitivity Reactions in Patients with AIDS,” International Journal of Dermatology, Vol. 32, No. 9, 1993, pp. 621-627. doi:10.1111/j.1365-4362.1993.tb04014.x
[12] M. R. Buchness, H. W. Lim, V. A. Hatcher, et al., “Eosinophilic Pustular Folliculitis in the Acquired Immunodeficiency Syndrome,” The New England Journal of Medicine, Vol. 318, No. 18, 1988, pp. 1183-1186. doi:10.1056/NEJM198805053181807
[13] S. A. Coopman, R. A. Johnson, R. Platt and R. S. Stern, “Cutaneous Disease and Drug Reactions in HIV Infection,” The New England Journal of Medicine, Vol. 328, No. 23, 1993, pp. 1670-1674. doi:10.1056/NEJM199306103282304
[14] T. Nagatani, “Adult T-Cell Leukemia/Lymphoma Cutaneous Type,” International Journal of Dermatology, Vol. 35, No. 6, 1996, pp. 400-401. doi:10.1111/j.1365-4362.1996.tb03018.x
[15] F. Gahongayire, “Mycosis Fungoides and Sézary Syndrome against a Human Immunodeficiency Virus-Positive Background: Case Report,” International Journal of Dermatology, Vol. 46, No. 1, 2007, pp. 32-35.
[16] R. Willemze, “Primary Cutaneous Lymphomas,” Annals of Oncology, Vol. 22, No. 4, 2011, pp. iv72-iv75. doi:10.1093/annonc/mdr181
[17] E. A. Kotz, D. Anderson and B. H. Thiers, “Cutaneous T-Cell Lymphoma,” Journal of the European Academy of Dermatology and Venereology, Vol. 17, No. 2, 2003, pp. 131-137. doi:10.1046/j.1468-3083.2003.00635.x
[18] T. Takahashi, H. Tsukuda, H. Itoh, M. Tsujisaki, et al., “Primary and Isolated Adult T-Cell Leukemia/Lymphoma of the Bone Marrow,” The Japanese Society of Internal Medicine, Vol. 50, No. 20, 2011, pp. 2393-2396. doi:10.2169/internalmedicine.50.5857
[19] J. J. Campbell, R. A. Clark, R. Watanabe and T. S. Kupper, “Sézary Syndrome and Mycosis Fungoides Arise from Distinct T-Cell Subsets: A Biologic Rationale for Their Distinct Clinical Behaviors,” Blood, Vol. 116, No. 5, 2010, pp. 767-771. doi:10.1182/blood-2009-11-251926
[20] K. Takatsuki, “Discovery of Adult T-Cell Leukemia,” Retrovirology, Vol. 2, No. 16, 2005, pp. 16-18.
[21] M. Duvic, M. Donato, B. Dabaja, C. Hosing, et al., “Total Skin Electron Beam and Non-Myeloablative Allogeneic Hematopoietic Stem-Cell Transplantation in Advanced Mycosis Fungoides and Sézary Syndrome,” Journal of Clinical Oncology, Vol. 28, No. 14, 2010, pp. 2365-2372. doi:10.1200/JCO.2009.25.8301
[22] M. Janier, C. Katlama, B. Flageul, et al., “The Pseudo-Sézary-Syndrome with CD8 Phenotype in a Patient with the Acquired Immunodeficiency Syndrome (AIDS),” Annals of Internal Medicine, Vol. 110, No. 9, 1989, pp. 738-740.
[23] P. Zhang, L. Chiribogy, M. Jacobsen, et al., “Mycosis Fungoides-Like T-Cell Cutaneous Lymphoid Infiltrates in Patients with HIV Infection,” American Journal of Dermatopathology, Vol. 17, No. 1, 1995, pp. 29-35.
[24] H. Bachelez, F. Hadida and G. Gorochov, “Massive Infiltration of the Skin by HIV-Specific Cytotoxic CD8+ cells,” The New England Journal of Medicine, Vol. 335, No. 1, 1996, pp. 61-62. doi:10.1056/NEJM199607043350118
[25] M. E. Moreno-Fernandez, W. Zapata, J. T. Blackard, G. Franchini and C. A. Chougnet, “Human Regulatory T Cells Are Targets for Human Immunodeficiency Virus Infection, and Their Susceptibility Differs Depending on the HIV Type 1 Strain,” Journal of Virology, Vol. 83, No. 24, 2009, pp. 12925-12933. doi:10.1128/JVI.01352-09

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