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Review Article
- Lymphomatoid papulosis: a practical review for pathologists
- Mario L. Marques-Piubelli, Carlos A. Torres-Cabala, Roberto N. Miranda
- J Pathol Transl Med. 2026;60(4):388-397. Published online July 15, 2026
- DOI: https://doi.org/10.4132/jptm.2026.06.09
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Abstract
PDF - Lymphomatoid papulosis (LyP) is a primary cutaneous CD30+ lymphoproliferative disorder characterized by a chronic and self-healing recurrent cluster of erythematous papules or nodules on the skin of the trunk and/or extremities. The disease has an indolent clinical course with spontaneous regression or waxing and waning clinical evolution. The histopathologic spectrum of LyP is vast and may show few to numerous atypical cells immersed in a mild to intense inflammatory background. The backbone for the diagnosis is the positivity for CD30, which is one of the criteria to define this group of lymphoproliferative disorders. The association of these different histological and immunophenotypical findings is used to subclassify this disease in different subtypes from A to E, associated with DUSP22/IRF4 rearrangement, and other rare forms. Although this differentiation is important to raise awareness of different differential diagnosis, it does not impact the prognosis or change the treatment, which is usually centered in symptom relief and faster regression. In this review, we aim to summarize the most updated information of the clinical, histopathological, and molecular characteristics of LyP and provide a practical assessment for the diagnostic features that could help with the main differential diagnosis.
Original Articles
- Clinico-Pathologic Evaluation of 18 Cases of Lymphomatoid Papulosis.
- Sug Kyoung Ko, Hye Sook Kim, Kee Suck Suh, Sang Tae Kim, Man Ha Huh
- Korean J Pathol. 1996;30(6):505-514.
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Abstract
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- Lymphomatoid papulosis is an enigmatic disease entity which is clinically benign and histologically malignant. Although sporadic cases have been reported, we could not find any comprehensive report on the combined clinical and histologic features of lymphomatoid papulosis in the literature. Perhaps the most controversial aspect of lymphomatoid papulosis is its pathogenesis and categorization as a benign versus a malignant entity. To date, there are no reports on p53 and bcl-2 protein expression in lymphomatoid papulosis. We analysed the clinico-pathological findings of 18 cases with lymphomatoid papulosis during the 10 year period from 1984 to 1995 and examined the prevalence of immunoreactivity for CD30(DAKO, Ber-H2), p53(DAKO, DO-7), and bcl-2(DAKO, 124) using an immunohistochemical(ABC) method. The results obtained are summarized as follows. 1) Age distribution ranged from 20 to 65, with a mean age of 45 years and a sex distribution which showed a male predominence(8:1). The lesions were located on the trunk and extremities(8cases), extremities (7cases), and trunk(3 cases). The morphology of the lesions were papules or plaques(12 cases), and nodules(6 cases). 2) Histopathologic types were classified into 3 types: type A(4 cases), type B(8 cases) and mixed type (6 cases). 3) Positive immunoreactivity for CD30 was seen in 17%(3 of 18cases): type A(2 of 3) and mixed type(1 of 3). 4) The positive immunoreactivity for p53 and bcl-2 was observed in 29%(5 of 18) and 11%(2 of 18), respectively. 5) Cases showing positive immunoreactivity for P53 were type A(1 of 5), type B(1 of 5), and mixed type(3 of 5). 6) Cases showing positive immunoreactivity for bcl-2 were mixed type(2 of 2). One case developed into Ki-1 lymphoma. These results support the idea that lymphomatoid papulosis and Ki-1 lymphoma represent a continuum. The role of p53 gene mutation and bcl-2 activation in the development of lymphomatoid papulosis is currently unknown. But, our results suggest that p53 gene mutation and bcl-2 activation are not a critical step in the development of lymphomatoid papulosis. Further studies are needed to elucidate the role of p53 gene mutation and bcl-2 activation in the development and progression of lymphomatoid papulosis.
- A Pathologic Study of Lymphoproliferative Disorders of the Skin.
- Yee Jeong Kim, Kwang Gil Lee, Soo Il Chun, Yun Woong Ko
- Korean J Pathol. 1991;25(6):551-562.
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Abstract
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- Forty eight skin biopsies obtained from 24 patients were reviewed, and clinical, histological and immunohistochemical findings were analyzed. Results obtained are as follows: 1) Skin manifestation was plaque, erythroderma, scale and hyperpigmentation in mycosis fungoides, and subcutaneous nodule, mass and ulcerated patch in cutaneous lymphoma. The skin of lymphomatoid papulosis revealed hemorrhagic ulcerated and erythematous papules which healed spontaneously. 2) Histologically, mycosis fungoides showed epidermotropism in most cases. Pautrier's micro-abscesses were present in one-fourth of the cases. Malignant lymphoma was different in histology from mycosis fungoides. As compared with mycosis fungoides, it showed less frequent epidermotropism, more compact and diffuse infiltration of atypical lymphocytes, more often association with ulcer and necrosis, and more frequent mitotic figures. Lymphomatoid papulosis showed striking hemorrhage and edema of the papillary dermis. 3) Based on the results of immunohistochemical study, mycosis fungoides and lymphomatoid papulosis were considered as a T cell proliferative disorder of the skin. According to these findings, lymphoproliferative disorders of the skin occurred predominantly in the elderly and males. Clinical and histopathologic findings overlapped and were similar each other. It was difficult to make a definite diagnosis in early lesions, and a sequential follow up biopsy was required. It is concluded that strict criteria such as marked atypia and clustering of atypical cells are necessary for a histologic diagnosis of malignant lymphoproliferative disorder of the skin.
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