Clinical and Immunomorphological Characteristics of Lymphomatoid Papulosis Type E (Literature Review and Case Report)

Lymphomatoid papulosis (LP) is a rare variant of benign lymphoproliferative disease with skin involvement. Based on clinical, morphological, and immunobiological characteristics, WHO hematopoietic and lymphoid tissue tumor classification (2016) differentiates between several LP types: А, В, С, D, Е, and with 6p25.3 rearrangement. The present article reviews the literature on clinical course, pathomorphological, immunological, and biomolecular characteristics of LP in adults and children. For the first time in the domestic literature, it provides a case report of LP, type E, in a 2-year-old child. Differential diagnosis and optimal disease management of LP are also described in detail.

• Timur Teimurazovich Valiev NN Blokhin National Medical Cancer Research Center, 24 Kashirskoye sh., Moscow, Russian Federation, 115478 ; ФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России, Каширское ш., д. 24, Москва, Российская Федерация, 115478
• A.M. Kovrigina National Research Center for Hematology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167 ; ФГБУ «НМИЦ гематологии» Минздрава России, Новый Зыковский пр-д, д. 4, Москва, Российская Федерация, 125167
• T.S. Belysheva NN Blokhin National Medical Cancer Research Center, 24 Kashirskoye sh., Moscow, Russian Federation, 115478 ; ФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России, Каширское ш., д. 24, Москва, Российская Федерация, 115478

1. Gross TG, Termuhlen AM. Pediatric non-Hodgkin lymphoma. Curr Hematol Malig Rep. 2008;3(3):167–73. doi: 10.1007/s11899-008-0024-8. DOI: https://doi.org/10.1007/s11899-008-0024-8
2. Macaulay Lymphomatoid papulosis: A continuing self-healing eruption, clinically benign—histologically malignant. Arch Dermatol. 1968;97(1):23–30. doi: 10.1001/archderm.97.1.23. DOI: https://doi.org/10.1001/archderm.1968.01610070029004
3. Liu HL, Hoppe RT, Kohler S, et al. CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma. J Am Acad Dermatol. 2003;49(6):1049–58. doi: 10.1016/s0190-9622(03)02484-8. DOI: https://doi.org/10.1016/S0190-9622(03)02484-8
4. Wieser I, Oh CW, Talpur R, et al. Lymphomatoid papulosis: treatment response and associated lymphomas in a study of 180 patients. J Am Acad Dermatol. 2016;74(1):59–67. doi: 10.1016/j.jaad.2015.09.013. DOI: https://doi.org/10.1016/j.jaad.2015.09.013
5. Martorell-Calatayud А, Hernandez-Martin А, Colmenero I, et al. Lymphomatoid Papulosis in Children: Report of 9 Cases and Review of the Literature. Actas Dermosifiliogr. 2010;101(8):693–701. DOI: https://doi.org/10.1016/S1578-2190(10)70699-5
6. Sauder MB, O’Malley JT, LeBoeuf NR. CD30+ lymphoproliferative disorders of the skin. Hematol Oncol Clin North Am. 2017;31(2):317–34. doi: 10.1016/j.hoc.2016.11.006. DOI: https://doi.org/10.1016/j.hoc.2016.11.006
7. Duvic M. CD30+ neoplasms of the skin. Curr Hematol Malig Rep. 2011;6(4):245–50. doi: 10.1007/s11899-011-0096-8. DOI: https://doi.org/10.1007/s11899-011-0096-8
8. Валиев Т.Т., Виноградова Е.Ю., Гилязитдинова Е.А. и др. Случай саркомной трансформации лимфоматоидного папулеза. Гематология и трансфузиология. 2006;5:44–6. [Valiev TT, Vinogradova EYu, Gilyazitdinova EA, et al. A case of sarcomatous transformation of lymphomatoid papulosis. Gematologiya i transfuziologiya. 2006;5:44–6. (In Russ)]
9. Nijsten T, Curiel-Lewandrowski C, Kadin ME. Lymphomatoid papulosis in children: a retrospective cohort study of 35 cases. Arch Dermatol. 2004;140(3):306–12. doi: 10.1001/archderm.140.3.306. DOI: https://doi.org/10.1001/archderm.140.3.306
10. LeBoit Lymphomatoid papulosis and cutaneous CD30+ lymphoma. Am J Dermatopathol. 1996;18(3):221–35. doi: 10.1097/00000372-199606000-00001. DOI: https://doi.org/10.1097/00000372-199606000-00001
11. El Shabrawi-Caelen L, Kerl H, Cerroni L. Lymphomatoid papulosis: reappraisal of clinicopathologic presentation and classification into subtypes A, B, and C. Arch Dermatol. 2004;140(4):441–7. doi: 10.1001/archderm.140.4.441. DOI: https://doi.org/10.1001/archderm.140.4.441
12. Kempf W, Kazakov DV, Scharer L, et al. Angioinvasive lymphomatoid papulosis: a new variant simulating aggressive lymphomas. Am J Surg Pathol. 2013;37(1):1–13. doi: 10.1097/PAS.0b013e3182648596. DOI: https://doi.org/10.1097/PAS.0b013e3182648596
13. Sharaf MA, Romanelli P, Kirsner R, Miteva M. Angioinvasive lymphomatoid papulosis: another case of a newly described variant. Am J Dermatopathol. 2014;36(3):75–7. doi: 10.1097/DAD.0b013e3182943394. DOI: https://doi.org/10.1097/DAD.0b013e3182943394
14. Scarisbrick JJ, Evans AV, Woolford AJ, et al. Regional lymphomatoid papulosis: a report of four cases. Br J Dermatol. 1999;141(6):1125–8. doi: 10.1046/j.1365-2133.1999.03218.x. DOI: https://doi.org/10.1046/j.1365-2133.1999.03218.x
15. Ba W, Yin G, Yang J, et al. Lymphomatoid papulosis type E with a CD56+ immunophenotype presented with purpura-like lesions. J Cutan Pathol. 2019;46(7):542–5. doi: 10.1111/cup.13472. DOI: https://doi.org/10.1111/cup.13472
16. Kiavash K, Abner SM, Malone JC. New variant lymphomatoid papulosis type E preceding and coexisting with mycosis fungoides – a case report and review of the literature. J Cutan Pathol. 2015;42(12):1018–23. doi: 10.1111/cup.12606. DOI: https://doi.org/10.1111/cup.12606
17. Fujimura T, Lyu C, Tsuchiyama K, Aiba S. CD30-Positive Angioinvasive Lymphomatoid Papulosis (Type E) Developing from Parapsoriasis en Plaque. Case Rep Oncol. 2018;11(3):850–4. doi: 10.1159/000495689. DOI: https://doi.org/10.1159/000495689
18. Kempf W, Pfaltz K, Vermeer MH, et al. EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. 2011;118(15):4024–35. doi: 10.1182/blood-2011-05-351346. DOI: https://doi.org/10.1182/blood-2011-05-351346
19. Kakizaki A, Fujimura T, Kambayashi Y, et al. Comparison of CD163+ Macrophages and CD206+ Cells in Lesional Skin of CD30+ Lymphoproliferative Disorders of Lymphomatoid Papulosis and Primary Cutaneous Anaplastic Large-cell Lymphoma. Acta Derm Venereol. 2015;95(5):600–2. doi: 10.2340/00015555-2016. DOI: https://doi.org/10.2340/00015555-2016