The pathology from a 4-mm punch biopsy of a hypopigmented macule on the patient’s thigh revealed “cerebriform” lymphocytes at the dermal-epidermal junction characteristic of mycosis fungoides (MF), a type of cutaneous T-cell lymphoma (CTCL).
CTCL is a rare disease with 1000 new cases per year in the United States, comprising about 0.5% of all nonHodgkin’s lymphoma cases. MF is the most common form of CTCL. It is more common in African Americans than white individuals with an incidence ratio of 6:1. It is more common in men than women at a ratio of 2:1.
CTCL is a malignant lymphoma of helper T-cells that usually remains confined to skin and lymph nodes. MF is named for the mushroom-like skin tumors seen in severe cases. Metastasis to the liver, spleen, lungs, gastrointestinal tract, bone marrow, and central nervous system may occur.
The most common initial presentation involves scaly patches or plaques with a persistent rash that is often pruritic and usually erythematous. Patches may evolve to generalized, infiltrated plaques or to ulcerated, exophytic tumors.
Hypo- or hyperpigmented lesions, petechiae, poikiloderma (skin atrophy with telangiectasia), and alopecia (with or without mucinosis) are other findings. Lesions typically develop on non-sun-exposed areas initially, such as the trunk below the waistline, flanks, breasts, inner thighs and arms (FIGURES 1 and 2), and the periaxillary areas.
The patient was referred to a hematologist/oncologist. She was started on narrowband ultraviolet B treatment.
Adapted from:
Mahan RD, Usatine RP. Hurricane Katrina evacuee develops a persistent rash. J Fam Pract. 2007;56:454-457.