The patient is an 86-year-old man with a 4-decade history of skin rashes. The condition was first noted on his thigh and was diagnosed as eczema. Over time, the rash spread to his arms and trunk and is occasionally itchy. Examination revealed widespread hyperpigmentation. The skin was xerotic with some areas manifesting slight induration. Shoddy lymphadenopathy was noted of his neck and axillae.
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Mycosis fungoides (MF) is a subtype of cutaneous T cell lymphoma. It initially presents in the skin but may involve the nodes, blood, and viscera.1 MF is uncommon, with an incidence of about 500 cases in the United States annually. It typically affects middle-aged and older adults, but there have been cases affecting young adults and children. The etiology of the disease is unclear.2 The course of the disease is typically indolent and manifests as patches, plaques, and tumors.3 Initially, the disease may present with scaling patches in a limited distribution. As the disease progresses, the lesions may evolve into plaques with a more generalized distribution.2 Tumors characterize end-stage disease.
Patients with limited skin involvement are usually treated with skin-directed therapies such as potent topical steroids, nitrogen mustard, ultraviolet B or PUVA light treatment, or electron beam radiation. Patients with refractory or advanced disease can be treated with oral or systemic immunomodulatory agents. A potentially curative strategy for advanced or resistant disease includes allogeneic stem cell transplantation.4
John Pappas, BS, is a medical student at the Geisinger Commonwealth School of Medicine in Scranton, PA. 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.
- 1. Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers. J Am Acad Dermatol. 2014; 70:205.e1-e16; quiz 221-222.
- 2. Hoppe RT, Wood GS, Abel EA. Mycosis fungoides and the Sézary syndrome: Pathology, staging, and treatment. Curr Probl Cancer. 1990;14:293-371.
- 3. Furue M, Kadono T: New aspects of the clinicopathological features and treatment of mycosis fungoides and Sézary syndrome. J Dermatol. 2015;42:941-944.
- 4. Foss FM, Girardi M. Mycosis fungoides and Sezary syndrome. Hematol Oncol Clin North Am. 2017;31:297-315.