Clinical Case of Lymphomatoid Papulosis

Keywords: Lymphomatoid papulosis, Clinical findings, Differential diagnosis, Treatment

Abstract

Lymphomatoid papulosis is a rare skin disease. The incidence averages 1.5 cases per 1 million population. It is the most common in adults around the age of 40. It was previously considered as paraneoplastic dermatosis, but recent studies have confirmed that lymphomatoid papulosis is a primary CD30 + T-lymphoma of the skin. Despite the aggressive morphological features, it is benign. The disease is characterized by a tendency to spontaneous regression. Typically, skin lesions last 3-12 weeks, although in some severe cases they may last longer. Skin lesions may disappear or recur over decades. Patients require observation because a second lymphoproliferative disease develops in 10-40% of patients.

          A case of lymphomatoid papulosis in a patient with diffuse astrocytoma is presented. The patient was treated with systemic retinoids. The results of differential diagnosis and additional research methods, including dermoscopy, are given. Although the use of dermoscopy was not crucial, the results of the research showed microscopic visual differences between the elements of the rash, depending on the stage of the disease. The picture was mostly typical, however, spots formed by the areas of central erythema on the background of light brown areas, with a predominance of the reticular vessels pattern were also noted in addition to the rash elements on the hairy skull. Clarification of this feature requires further study.

          The purpose of our report is to draw the attention of dermatologists and family doctors to cases of rare dermatoses. The rareness of the disease, lack of knowledge on the clinical findings lead to diagnostic errors, “inadequate treatment”.

References

Macaulay WL. Lymphomatoid papulosis. A continuing self-healing eruption, clinically benign--histologically malignant. Arch Dermatol. 1968; 97(1): 23-30. Available from: https://doi.org/10.1001/archderm.97.1.23

Killoran E, Mehta-Shah N, Musiek A. Lymphomatoid papulosis. JAMA Dermatol. 2020; 156(3): 360. Available from: https://doi.org/10.1001/jamadermatol.2019.4513

Wieser I, Wohlmuth C, Nunez CA, Duvic M. Lymphomatoid papulosis in children and adolescents: A systematic review. Am J Clin Dermatol. 2016; 17(4): 319-27. Available from: https://doi.org/10.1007/s40257-016-0192-6

Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood. 2019; 133(16): 1703-14. Available from: https://doi.org/10.1182/blood-2018-11-881268

Griscelli F, Féraud O, Oudrhiri N, Gobbo E, Casal I, Chomel J-C, et al. Malignant germ cell-like tumors, expressing Ki-1 antigen (CD30), are revealed during in vivo differentiation of partially reprogrammed human-induced pluripotent stem cells. Am J Pathol. 2012; 180(5): 2084-96. Available from: https://doi.org/10.1016/j.ajpath.2012.01.011

Werner B, Massone C, Kerl H, Cerroni L. Large CD30-positive cells in benign, atypical lymphoid infiltrates of the skin. J Cutan Pathol. 2008; 35(12): 1100-7. Available from: https://doi.org/10.1111/j.1600-0560.2007.00979.x

Nikolaenko L, Zain J, Rosen ST, Querfeld C. CD30-positive lymphoproliferative disorders. Cancer Treat Res. 2019; 176: 249-68. Available from: https://doi.org/10.1007/978-3-319-99716-2_12

Martinez-Cabriales SA, Walsh S, Sade S, Shear NH. Lymphomatoid papulosis: an update and review. J Eur Acad Dermatol Venereol. 2020; 34(1): 59-73. Available from: https://doi.org/10.1111/jdv.15931

Moura FN, Thomas L, Balme B, Dalle S. Dermoscopy of lymphomatoid papulosis. Arch Dermatol. 2009; 145(8): 966-7. Available from: https://doi.org/10.1001/archdermatol.2009.167

Caccavale S, Vitiello P, Mascolo M, Ciancia G, Argenziano G. Dermoscopy of different stages of lymphomatoid papulosis. J Eur Acad Dermatol Venereol. 2018; 32(5): e198-200. Available from: https://doi.org/10.1111/jdv.14706

Published
2022-10-17
Section
Case Reports