Lymphomatoid Papulosis Clinical Presentation
- Author: John A Zic, MD; Chief Editor: William D James, MD more...
Most patients with lymphomatoid papulosis (LyP) describe the gradual onset of an asymptomatic to mildly pruritic papular eruption. Papules appear in crops and resolve spontaneously within 2-8 weeks. Waxing and waning of the crops of papules may continue for decades.
Unless accompanied by systemic lymphoma, most patients have no constitutional symptoms.
Unless accompanied by systemic lymphoma, physical findings are limited to the skin and, very rarely, the oral cavity.[11, 12]
Each erythematous papule evolves into a red-brown, often hemorrhagic, papulovesicular or papulopustular lesion over days to weeks, as demonstrated in the images below.
Some lesions develop a necrotic eschar before healing spontaneously. Occasionally, noduloulcerative lesions may be present, as in the image below.
Each papule heals within 2-8 weeks, leaving a hypopigmented or hyperpigmented, depressed, oval, and varioliform scar.
Large nodules and plaques may take months to resolve. Carefully evaluate solitary ulcerated nodules, plaques, or masses for CD30+ ALCL (see image below), MF, or rarely, HD.
The skin distribution of lesions, characteristically, is on the trunk and extremities, although the palms and/or soles, face, scalp, oral mucosa, and anogenital area also may be involved.
Evolving lesions have been described under dermoscopy. The initial papular lesion showed a vascular pattern of tortuous vessels radiating from the center. A white structureless area was seen around the vessels. More mature lesions, hyperkeratotic papules, looked similar except the vascular pattern in the center of the lesion was obscured. As the lesions progressed to necrotic ulcerations, the vascular pattern was only seen at the periphery, while the center of the lesions was a structure of brownish-gray areas. The final, or cicatricial phase, was similar except no vessel pattern was seen.
The etiology of lymphomatoid papulosis is unknown. Debate persists over whether (1) lymphomatoid papulosis is a benign chronic disorder of activated T cells responding to external or internal stimuli or (2) lymphomatoid papulosis is an indolent T-cell malignancy localized to skin and held in check by the host immune system.
A few investigators have discovered viruslike particles in lymphomatoid papulosis lesions examined under electron microscopy.
Macaulay WL. Lymphomatoid papulosis. A continuing self-healing eruption, clinically benign--histologically malignant. Arch Dermatol. 1968 Jan. 97(1):23-30. [Medline].
Jaffe ES. The 2008 WHO classification of lymphomas: implications for clinical practice and translational research. Hematology Am Soc Hematol Educ Program. 2009. 523-31. [Medline].
Kempf W, Sander CA. Classification of cutaneous lymphomas - an update. Histopathology. 2010 Jan. 56 (1):57-70. [Medline].
Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES. The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. Blood. 2011 May 12. 117 (19):5019-32. [Medline].
Slater DN. The new World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas: a practical marriage of two giants. Br J Dermatol. 2005 Nov. 153(5):874-80. [Medline].
Willemze R, Meijer CJ. Classification of cutaneous T-cell lymphoma: from Alibert to WHO-EORTC. J Cutan Pathol. 2006 Feb. 33 Suppl 1:18-26. [Medline].
Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005 May 15. 105(10):3768-85. [Medline].
de Souza A, El-Azhary RA, Camilleri MJ, Wada DA, Appert DL, Gibson LE. In search of prognostic indicators for lymphomatoid papulosis: A retrospective study of 123 patients. J Am Acad Dermatol. 2011 Oct 7. [Medline].
Rassidakis GZ, Thomaides A, Atwell C, et al. JunB expression is a common feature of CD30+ lymphomas and lymphomatoid papulosis. Mod Pathol. 2005 Oct. 18(10):1365-70. [Medline].
Clarke LE, Bayerl MG, Bruggeman RD, et al. Death receptor apoptosis signaling mediated by FADD in CD30-positive lymphoproliferative disorders involving the skin. Am J Surg Pathol. 2005 Apr. 29(4):452-9. [Medline].
Pujol RM, Muret MP, Bergua P, Bordes R, Alomar A. Oral involvement in lymphomatoid papulosis. Report of two cases and review of the literature. Dermatology. 2005. 210(1):53-7. [Medline].
de-Misa RF, Garcia M, Dorta S, et al. Solitary oral ulceration as the first appearance of lymphomatoid papulosis: a diagnostic challenge. Clin Exp Dermatol. 2009 May 21. [Medline].
Wang HH, Lach L, Kadin ME. Epidemiology of lymphomatoid papulosis. Cancer. 1992 Dec 15. 70(12):2951-7. [Medline].
Moura FN, Thomas L, Balme B, Dalle S. Dermoscopy of lymphomatoid papulosis. Arch Dermatol. 2009 Aug. 145(8):966-7. [Medline].
Kim YC, Yang WI, Lee MG, et al. Epstein-Barr virus in CD30 anaplastic large cell lymphoma involving the skin and lymphomatoid papulosis in South Korea. Int J Dermatol. 2006 Nov. 45(11):1312-6. [Medline].
Vonderheid EC, Kadin ME, Gocke CD. Lymphomatoid papulosis followed by pityriasis lichenoides: a common pathogenesis?. Am J Dermatopathol. 2011 Dec. 33(8):835-40. [Medline].
Zheng Y, Jia J, Tian Q, Dong X, Wang X, Ying Z, et al. Lymphomatoid papulosis misdiagnosed as pityriasis lichenoides et varioliformis acuta: Two case reports and a literature review. Exp Ther Med. 2014 Dec. 8(6):1927-1933. [Medline]. [Full Text].
Leo AM, Ermolovich T. Lymphomatoid papulosis while on efalizumab. J Am Acad Dermatol. 2009 Sep. 61(3):540-1. [Medline].
Talsania N, O'Toole EA. Severe hypersensitivity reaction to minocycline in association with lymphomatoid papulosis. Clin Exp Dermatol. 2009 Oct. 34(7):e397-8. [Medline].
KIM SK, KIM YC. Lymphomatoid papulosis after allogenic stem cell transplantation. Eur J Dermatol. 2009 Sep-Oct. 19(5):520-1. [Medline].
Kempf W, Pfaltz K, Vermeer MH, Cozzio A, Ortiz-Romero PL, Bagot M, 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. Blood. 2011 Oct 13. 118(15):4024-35. [Medline]. [Full Text].
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 Apr. 140(4):441-7. [Medline].
Willemze R, Beljaards RC. Spectrum of primary cutaneous CD30 (Ki-1)-positive lymphoproliferative disorders. A proposal for classification and guidelines for management and treatment. J Am Acad Dermatol. 1993 Jun. 28(6):973-80. [Medline].
Liu HL, Hoppe RT, Kohler S, Harvell JD, Reddy S, Kim YH. CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma. J Am Acad Dermatol. 2003 Dec. 49(6):1049-58. [Medline].
McQuitty E, Curry JL, Tetzlaff MT, Prieto VG, Duvic M, Torres-Cabala C. The differential diagnosis of CD8-positive ("type D") lymphomatoid papulosis. J Cutan Pathol. 2014 Feb. 41(2):88-100. [Medline].
Kempf W, Kazakov DV, Schärer L, Rütten A, Mentzel T, Paredes BE, et al. Angioinvasive lymphomatoid papulosis: a new variant simulating aggressive lymphomas. Am J Surg Pathol. 2013 Jan. 37(1):1-13. [Medline].
Karai LJ, Kadin ME, Hsi ED, Sluzevich JC, Ketterling RP, Knudson RA, et al. Chromosomal rearrangements of 6p25.3 define a new subtype of lymphomatoid papulosis. Am J Surg Pathol. 2013 Aug. 37(8):1173-81. [Medline].
Bergstrom JS, Jaworsky C. Topical methotrexate for lymphomatoid papulosis. J Am Acad Dermatol. 2003 Nov. 49(5):937-9. [Medline].
Vonderheid EC, Sajjadian A, Kadin ME. Methotrexate is effective therapy for lymphomatoid papulosis and other primary cutaneous CD30-positive lymphoproliferative disorders. J Am Acad Dermatol. 1996 Mar. 34(3):470-81. [Medline].
Jain N, Gutte R, Jadhav P, Khopkar U. Granulomatous and eccrinotropic lymphomatoid papulosis. Indian J Dermatol Venereol Leprol. 2012 Jan. 78(1):82-4. [Medline].
Yip L, Darling S, Orchard D. Lymphomatoid papulosis in children: experience of five cases and the treatment efficacy of methotrexate. Australas J Dermatol. 2011 Nov. 52(4):279-83. [Medline].
Newland KM, McCormack CJ, Twigger R, Buelens O, Hughes CF, Lade S, et al. The efficacy of methotrexate for lymphomatoid papulosis. J Am Acad Dermatol. 2015 Jun. 72 (6):1088-90. [Medline].
Bruijn MS, Horváth B, van Voorst Vader PC, Willemze R, Vermeer MH. Recommendations for treatment of lymphomatoid papulosis with methotrexate: a report from the Dutch Cutaneous Lymphoma Group. Br J Dermatol. 2015 May 22. [Medline].
Hoetzenecker W, Guenova E, Hoetzenecker K, Yazdi A, Röcken M, Berneburg M. Successful treatment of recalcitrant lymphomatoid papulosis in a child with PUVA-bath photochemotherapy. Eur J Dermatol. 2009 Nov-Dec. 19(6):646-7. [Medline].
Hughes PS. Treatment of lymphomatoid papulosis with imiquimod 5% cream. J Am Acad Dermatol. 2006 Mar. 54(3):546-7. [Medline].
Kontos AP, Kerr HA, Malick F, Fivenson DP, Lim HW, Wong HK. 308-nm excimer laser for the treatment of lymphomatoid papulosis and stage IA mycosis fungoides. Photodermatol Photoimmunol Photomed. 2006 Jun. 22(3):168-71. [Medline].
Rodrigues M, McCormack C, Yap LM, et al. Successful treatment of lymphomatoid papulosis with photodynamic therapy. Australas J Dermatol. 2009 May. 50(2):129-32. [Medline].
Beljaards RC, Willemze R. The prognosis of patients with lymphomatoid papulosis associated with malignant lymphomas. Br J Dermatol. 1992 Jun. 126(6):596-602. [Medline].
Kempf W, Levi E, Kamarashev J, et al. Fascin expression in CD30-positive cutaneous lymphoproliferative disorders. J Cutan Pathol. 2002 May. 29(5):295-300. [Medline].