A 70-year-old man presents with skin lesions located on his back, left thigh, and scalp. These arose simultaneously approximately 4 weeks previously. He is in good health and is a nonsmoker. He takes omeprazole for gastric reflux. He denies fatigue, weight loss, or swollen glands. Examination reveals several dusky red indurated plaques.
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Primary cutaneous B-cell lymphoma (PCBCL) is a rare lymphoproliferative disorder that first manifests in the skin in contradistinction to nodal B-cell lymphomas that begin within lymph nodes.1 The incidence rate increases with age and it occurs with greater prevalence in males.2
Diagnosis is based on immunophenotyping of biopsy specimens, with staging evaluation to rule out internal disease.3 The etiology is unknown, although a significant percentage of cases in Europe have been associated with Borrelia burgdorferi transmitted by ticks.4
In general, PCBCL has a favorable prognosis. Many cases are indolent in nature, with a 5-year survival approaching 90%. Factors related to an adverse prognosis include elevated lactate dehydrogenase levels, the presence of more than 2 lesions, and nodularity.5 Individual lesions respond well to radiation therapy or surgical excision. Rituximab is the drug of choice for more advanced disease.6
Amy Hendrix, CRNP, is a nurse practitioner with Reading Dermatology Associates in Reading, Pennsylvania. Stephen Schleicher, MD, is an associate professor of medicine at the Commonwealth Medical College in Scranton, Pennsylvania, and an adjunct assistant professor of dermatology at the Perelman School of Medicine at the University of Pennsylvania in Philadelphia.
- Swerdlow SH, Campo E, Harris NL, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon, France: World Health Organization; 2008.
- Smith BD, Smith GL, Cooper DL, Wilson LD. The cutaneous B-cell lymphoma prognostic index: a novel prognostic index derived from a population-based registry. J Clin Oncol. 2005;23:3390-3395.
- Kim YH, Willemze R, Pimpinelli N, et al. TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110:479-484.
- Cerroni L, Zöchling N, Pütz B, Kerl H. Infection by Borrelia burgdorferi and cutaneous B-cell lymphoma. J Cutan Pathol. 1997;24:457-461.
- Wilcox RA. Cutaneous B-cell lymphomas: 2013 update on diagnosis, risk-stratification, and management. Am J Hematol. 2013;88:73-76.
- Senff NJ, Noordijk EM, Kim Y, et al. European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas. Blood. 2008;112:1600-1609.