June 2019 Case Studies – Page 2 – Derm In-Review
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June 2019 Case Studies

By August 25, 2019 No Comments


The correct answer is B. Primary cutaneous marginal zone lymphoma

Primary cutaneous marginal zone lymphoma (PCMZL), a subtype of primary cutaneous B cell lymphoma (PCBL), is an indolent lymphoma originating from mucosal associated lymphoid tissue (MALT). Most patients present in their fifth or sixth decade of life and it is thought to be more prevalent in men. The exact etiology is still unknown, though it is thought to be associated with Borrelia burgdorferior other infectious etiologies.1

Clinically, patients often present with nonspecific lesions, such as erythematous to violaceous papules plaques or nodules primarily on the extremities and trunk. One study showed that at the time of diagnosis, 84% of skin lesions consisted of nodules, 26% plaques, 19% papules, and only 4% had extracutaneous involvement.1

As PCMZL is a rare disease, there are no large clinical trials regarding treatment. Luckily, the prognosis for PCMZL is excellent, with a 10 year survival rate of approximately 93%, though cutaneous relapses are common. Extracutaneous involvement or spread is rare. 1 

Therefore, for most patients with localized disease, local radiation therapy or surgical excision is typically recommended and can be curative. For patients with asymptomatic multifocal disease, monitoring is a reasonable initial approach. If treatment is necessary, topical steroids, nitrogen mustard, interferon therapy, and rituximab have all been tried with varied outcomes.2

  1. Servitje O, Muniesa C, Benavente Y, et al. Primary cutaneous marginal zone B-cell lymphoma: Response to treatment and disease-free survival in a series of 137 patients. Journal of the American Academy of Dermatology. 2013;69(3):357-365. doi:10.1016/j.jaad.2013.04.047.
  2. Senff NJ, Noordijk EM, Kim YH, 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(5):1600-1609. doi:10.1182/blood-2008-04-152850.

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Author Krista Reznik

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