Cutaneous T-Cell Lymphoma 1_1111 Derm Dx
Cutaneous T-Cell Lymphoma 2_1111 Derm Dx
A 67-year-old man presented with complaints of pain and discomfort associated with multiple spreading skin lesions and enlarging lymph nodes. He also noted a 10-kg weight loss and fatigue. There was no previous medical or surgical history. The patient had no known drug allergies and was not taking any medications. However, he had been exposed to Agent Orange (2,3,7,8-tetrachlorodibenzo-p-dioxin) in 1963 and 1964 while serving in the Vietnam War. Examination revealed grossly enlarged cervical, axillary and inguinal lymph nodes bilaterally. Multiple 2- to 4-cm nodules were found on his chest, abdomen, upper extremities and back. He had a large, ulcerated fungating mass of coalescing nodules on his right groin. What is your diagnosis?Submit your answer, and then read the full explanation below. If you like this activity or have a suggestion, tell us about it in the comment box at the bottom of the page.
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The patient was diagnosed with advanced stage IVB cutaneous T-cell lymphoma (CTCL), also known as “mycosis fungoides.” He was seen by several physicians during the early 1990s for nonspecific dermatitis that did not resolve with the usual treatment.
Multiple skin biopsies were nondiagnostic, yet suspicious for CTCL. In 1994, the patient was told that he might have CTCL and advised to begin psoralen-UVA therapy. The patient refused.
Brain CT was normal. Chest CT revealed axillary lymphadenopathy without mediastinal or lung involvement. Abdominal CT showed multiple large liver metastases, hepatomegaly and extensive periceliac and inguinal lymphadenopathy. Excision of an axillary lymph node suggested diffuse large cell non-Hodgkin’s lymphoma. There was no evidence of bone marrow involvement by lymphoma.
Cutaneous T-cell lymphoma is a monoclonal proliferation of CD4+ T-helper cells.1,2 These malignant cells infiltrate the skin, resulting in a variety of lesions. Early manifestations of CTCL may be confused with eczema, psoriasis or contact dermatitis. Suspected CTCL should be confirmed with skin biopsy.
Pautrier’s microabscess is the most important histologic feature. However, histologic diagnosis requires hyperchromatic, slight to markedly atypical lymphoid cells in the epidermis, either as single, often haloed cells or in a linear configuration at the dermal-epidermal junction.3
The disease progresses from patch to plaque to tumor. Tumor-stage CTCL may then progress to lymph node and visceral involvement. Staging of CTCL incorporates the tumor, node and metastasis system, as well as prognosis.
Progression is related to stage at diagnosis, presence of extracutaneous disease, type and extent of skin involvement, failure to obtain complete remission on initial treatment, associated follicular mucinosis and older age.3
Overall, disease-specific survival rates for patients with CTCL at five and 10 years is 89% and 75%. However, in limited disease, progression within the first 10 years after diagnosis is <10 percent.
Treatment of CTCL for localized disease is topical steroids, psoralen-UVA or UVB, topical nitrogen mustard, or total body electron beam therapy.1,2 Often, methotrexate, isotretinoin and/or interferon alfa-2b are added. For systemic disease, combination chemotherapy is preferred, with or without topical therapy.1,2
Despite treatment with combination therapy, this patient died. Any association between Agent Orange exposure and later development of non-Hodgkin’s lymphoma is still unresolved.4
Rade Nicholas Pejic, MD, practices family medicine at Santa Monica/UCLA Medical Center in California.
1. Elmer KB, George RM. “Cutaneous T-cell lymphoma presenting as benign dermatoses.” Am Fam Physician. 1999;59:2809.
2. Lookingbill DP, Marks JG, eds. “Scaling papules, plaques, and patches. Principles of 3.” Dermatology. 3rd ed. Philadelphia, PA: WB Saunders; 2000:150.
4. van Doorn R, van Haselen CW, van Voorst Vader PC et al. “Mycosis fungoides: disease evolution and prognosis of 309 Dutch patients.” Arch Dermatol. 2000;136:504.
5. Kramarova E, Kogevinas M, Anh CT et al. “Exposure to Agent Orange and occurrence of soft-tissue sarcomas or non-Hodgkin lymphomas: an ongoing study in Vietnam.” Environ Health Perspect. 1998:106(Suppl 2):671.