Lymphomatoid papulosis: A rare case report and review of literature
Keywords:
Lymphomatoid papulosis, cutaneous CD30 lymphoproliferative disordersAbstract
Lymphomatoid papulosis (LyP) is a rare cutaneous condition characterized by chronic, recurrent, and self-regressing papulonodular skin eruptions. It belongs to the spectrum of primary cutaneous CD30+ lymphoproliferative disorders, along with primary cutaneous anaplastic large cell lymphoma (primary C-ALCL) with which it shares overlapping clinical and histopathologic features. The occurrence of LyP is extremely rare, with an estimated overall prevalence rate of 1.2 to 1.9 cases per 1 million population. Patients with LyP are at an increased risk of developing cutaneous or nodal lymphoid malignancies such as classic mycosis fungoides, ALCL, and Hodgkin lymphoma. Treatment includes use of topical steroids with or followed by phototherapy (psoralen-UVA light therapy [PUVA]) or oral low-dose methotrexate (MTX). Complications due to long-term treatment may also include a higher incidence of non-melanoma skin cancer (due to PUVA) or hepatic fibrosis (due to MTX). Here, we report a case of 48-year-old female, admitted with the complaints of multiple erythematous painless nonpruritic plaques and subcutaneous nodules over the right shoulder, both upper limbs, lower limbs, back and the abdomen for 4 months and a large ulcerated tumor over the medial aspect of left leg for 3 months. To the best of our knowledge, this is the first case of LyP to be published from Bangladesh.References
Willemze R, Meyer CJ, van Vloten WA. The clinical and histological spectrum of lymphomatoid papulosis. Br J Dermatol. 1982;107:131-44.
Harvell J, Vaseghi M, Natkunam Y, Kohler S, Kim Y. Large atypical cells of lymphomatoid papulosis are CD56 -negative: a study of 18 cases. J Cutan Pathol. 2012;29:88-92.
Paulli M, Berti E, Rosso R, Boveri E, Kindle E, Klersy C et al. CD30/Ki-1-positive lymphoproliferative disorders of the skin - clinicopathologic study from the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Project Group. J Clin Oncol. 1995;13:1343-54.
Steinhoff M, Hummel M, Anagnostopoulos I, Kaudewitz P, Seitz V, Assaf C et al. Single-cell analysis of CD30+ cells in lymphomatoid papulosis demonstrates a common clonal T-cell origin. Blood. 2002;100:578-84.
Kempf W, Levi E, Kamarashev J, Kutzner H, Pfeifer W, Petrogiannis-Haliotis T et al. Fascin expression in CD30-positive cutaneous lymphoproliferative disorders. J Cutan Pathol. 2002;29:295-300.
Volkenandt M, Kerscher M, Sander C, Meurer M, Röcken M. PUVA-bath photochemotherapy resulting in rapid clearance of lymphomatoid papulosis in a child. Arch Dermatol. 1995;131:1094.
Wolf P, Cohen PR, Duvic M. Ambivalent response of lymphomatoid papulosis treated with methoxypsoralen and UVA. J Am Acad Dermatol. 1994;30:1018-20.
Wollina U. Lymphomatoid papulosis treated with extracorporeal photochemotherapy. Oncol Reports. 1998;5:57-9.
Kadin ME, Levi E, Kempf W. Progression of lymphomatoid papulosis to systemic lymphoma associated with escape from growth inhibition by transforming growth factor-beta and CD30 ligand. Ann NY Acad Sci. 2001;941:59-68.
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;34:470-81.
Knobler R, Girardi M. Extracorporeal photochemoimmunotherapy in cutaneous T cell lymphomas. Ann NY Acad Sci. 2001;941:123-38.
Wollina U, Kaatz M. Extracorporeal photochemotherapy in cutaneous T-cell lymphoma. An overview of current status. Int J Immunopathol Pharmacol Section Dermatol. 2002;14:411-8.
Krathen RA, Ward S, Duvic M. Bexarotene is a new treatment option for lymphomatoid papulosis. Dermatology. 2003;206:142-7.
Wollina U, Wurbs C, Schönlebe J. Lymphomatoid papulosis - Report of two cases. An Bras Dermatol. 2005;80:161-4.
Obaidat N, Masri A, Awamleh A. Lymphomatoid papulosis: A case report. J Med J. 2006;40 (2):137-41.
Sina B, Burnett JW. Lymphomatoid papulosis. Case reports and literature review. Arch Dermatol. 1983;119:189-97.