Complications
Ulceration of cutaneous plaques and tumors is frequently seen in patients with advanced disease. This may increase the risk of cutaneous and/or systemic infections.
Patients frequently develop cutaneous infections, primarily involving Staphylococcus aureus. This is related to impaired skin barrier function from cutaneous disease and immunosuppression, particularly in advanced disease.
Signs include impetiginization of lesions, with weeping and honey-colored crusts or cutaneous pustules.
Skin swabs for microbiology will confirm infection and antibiotic sensitivities.
Treatment is with topical antiseptics/antibiotics or systemic antibiotic therapy.
Other bacterial, viral, and atypical infections may be seen.
Patients with advanced stages of CTCL have an increased risk of developing systemic infections. This may be due to disease-related immunosuppression, impaired skin barrier function due to cutaneous disease, or treatment-related immunosuppression.
Patients should be monitored clinically during the disease course for symptoms and signs of systemic infection. Atypical infections may be seen. Treatment is directed by clinical condition and the results of appropriate cultures. Septicemia is one of the major causes of death in patients with advanced disease.
CTCL patients have an increased risk of second malignancies, independent of treatment, compared with the general population. These include lymphoproliferative malignancy, solid organ malignancy, and cutaneous malignancy. Patients should be followed up regularly, and screened for symptoms and signs that may indicate second malignancies. Investigation and treatment will depend on the precise diagnosis made.
Many skin-directed therapies for CTCL are carcinogenic, and are associated with an increased risk of both non-melanoma and melanoma skin cancer. This complication is particularly relevant in patients treated with phototherapy (PUVA), and is dose-related, with the highest risk in those patients receiving >200 exposures. Cutaneous malignancy has also been reported with topical mechlorethamine, and as a late complication of radiation therapy.
Maintenance phototherapy should be avoided where possible, and patients should be screened for skin cancer during follow-up. Any changing cutaneous nodule or ulcer should be considered for biopsy if there is clinical suspicion of malignancy.
Older patients with erythrodermic skin disease and/or Sézary syndrome may develop high-output cardiac failure, dehydration and renal impairment, impairment of thermoregulation, and impaired barrier function of the skin.
Involvement of the central nervous system (CNS) is a potential complication of CTCL and is associated with poor prognosis.[120][121]
Patients with mycosis fungoides (MF) which progresses to CNS involvement may exhibit neurologic deficit; symptoms include general systemic abnormality and vestibular, cognitive, and ocular changes. There is no consensus on the treatment of CNS progression in CTCL: the National Comprehensive Cancer Network recommends temozolomide to treat patients with CTCL with CNS involvement despite prior treatment.[32] One study of four patients with MF with CNS progression who received temozolomide with either low-dose irradiation as initial treatment, or following disease progression with methotrexate-based chemotherapy and CNS irradiation, demonstrated that temozolomide following low-dose CNS irradiation appears to be well tolerated and effective in MF patients with CNS progression and is a less toxic alternative to methotrexate chemotherapy as second-line treatment.[121]
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