Management of Mycosis Fungoides in Geriatrics

What Is Mycosis Fungoides?

Mycosis fungoides is a type of non-Hodgkin’s lymphoma affecting the skin. The condition is rare and primarily affects the older age group. The median age group for being diagnosed with mycosis fungoides is 55 to 60 years. The incidence of developing mycosis fungoides increases rapidly with age. The course of the disease is refractory to therapies.

What Causes Mycosis Fungoides?

The condition occurs when T-lymphocytes (white blood cells) become cancerous and travel to the skin. Although the specific cause of the cancerous condition is unknown, factors like genetic mutation, exposure to chemicals and solvents, viral infection, and increased cytokine activities are attributed to developing mycosis fungoides.

What Are the Symptoms of Mycosis Fungoides in Geriatrics?

The symptoms of mycosis fungoides resemble those of similar skin conditions. The disorder clinically presents itself slowly and indolently. Initially, it forms itchy, scaly patches and elevated plaque that gradually transform into tumors, erythroderma, or visceral involvement. The disease appears as a three-stage process, but not all go through every stage. Other presenting symptoms are alopecia (hair loss) and dyspigmentation.

1. Patch Stage

Individuals in the patch stage can develop irregular, poorly defined, and scaling lesions. It can be red, pink, or brown colored. The skin surrounding the lesion becomes thin and wrinkled with spider vein formation. The patches or skin lesions form on areas without adequate sunlight, such as the abdomen, breasts, upper thighs, and buttocks.

2. Plaque Stage

The plaque type is thick skin lesions that are itchy and scaly. The borders of the plaque-type lesions are well-defined and appear red, purple, or brown. It forms on existing patches or unaffected skin.

3. Tumor Stage

Some individuals have their plaques develop into tumors. The tumors appear as irregular large lumps with shiny surfaces. It may become deep red or purple and forms open sores. Few patients tend to develop skin cysts with fever and weight loss.

How Is Mycosis Fungoides Diagnosed in Geriatrics?

The skin patches and plaques formed in mycosis fungoides can be misdiagnosed clinically and histologically as Psoriasis (autoimmune skin disease) or Eczema (skin inflammation). Only a high degree of suspicion and skin or lymph node biopsy can confirm the diagnosis and aid in treating the condition. Dermoscopy can help differentiate it from other skin disorders.

An elderly individual's skin and scalp must be inspected for mycosis fungoides. It is essential not to ignore minor complaints of scalp irritation and hair loss among the older age group. When these symptoms are dismissed as a normal aspect of aging and delayed diagnosis, resulting in severe consequences and increased risk of mortality.

What Is the Treatment for Mycosis Fungoides in Geriatrics?

Treatment for mycosis fungoides depends on the disease stage and the patient’s overall health. However, there are no universal guidelines for curing mycosis fungoides. Based on the National Comprehensive Cancer Network, the treatment guidelines established are:

1. Early-Stage Mycosis Fungoides

Only 5 to 10 percent of patients with early-stage mycosis fungoides progress to advanced stage. The treatment includes skin-directed therapies such as:

1. Topical Corticosteroids: Patches or plaques are treated with topical corticosteroids. It is administered as monotherapy in the initial stage and adjuvant therapy in a later stage. Prolonged use of topical steroids can cause toxicity.

2. Topical Retinoid: Bexarotene is a retinoid (derived from vitamin A) used for treating refractory cutaneous lesions. The medication can be administered in addition to steroids. It is not recommended to use Bexarotene while pregnant. Patients find the treatment to be convenient and well-tolerated. Associated side effects are hypertriglyceridemia and hypothyroidism.

3. Phototherapy: Phototherapy is the first line of treatment for hypopigmented mycosis fungoides. Narrow-band ultraviolet B (NB-UVB) is administered for patch-stage disease in three sessions per week and causes complete remission of the patches but can increase skin cancer risk. Psoralen-ultraviolet A (PUVA) treats the early stage of mycosis fungoides and refractory lesions. It is more effective than NB-UVB. The patch or plaque lesions require two or three sessions per week. However, individuals must be observed for side effects such as increased sensitivity to sunlight or skin cancers.

4. Phototherapy Plus Systemic Therapy: PUVA can be administered with interferons or retinoids to improve the effectiveness in refractory lesions and reduce adverse effects.

5. Topical Chemotherapy: A limited number of lesions and affected sites can be treated with topical chemotherapy. Some individuals may develop local reactions.

6. Radiotherapy: Radiotherapy is a critical mode of treatment for the early and advanced stages of mycosis fungoides.

2. Advanced-Stage Mycosis Fungoides

The systemic treatment is suggested in extensive lymph nodes and skin involvement. Management of advanced-stage mycosis fungoides is complex in older age groups. Systemic treatment for mycosis fungoides includes:

1. Histone Deacetylase Inhibitors: Vorinostat and Romidepsin are the frequently used interferons for managing mycosis fungoides

.2. Interferons: It is an immunomodulatory therapy. However, elderly patients need to have adverse effects carefully handled.

3. Monoclonal Antibody Therapies: Alemtuzumab may be the preferred treatment in specific cases.

4. Biological Response Modifiers: Bortezomib and Alemtuzumab are some examples of biological response modifiers.

5. Chemotherapeutic Agents: Common chemotherapeutic agents are:

• Methotrexate.

• Doxorubicin.

• Gemcitabine.

• Cyclophosphamide.

• Chlorambucil.

• Fludarabine.

6. Low-dose Methotrexate: A low-dose Methotrexate is used in some patients to control the symptoms of the disease and is a second line of treatment for hypopigmented mycosis fungoides. A combination of chemotherapy drugs can help with therapy.

7. Allogenic Hematopoietic Stem Cell Transplantation: Severe cases of mycosis fungoides can benefit from bone marrow transplants.

The treatment for mycosis fungoides is expensive and not readily available. Preventive measures are unavailable, but disease risk and complications could be lowered.

What Is the Prognosis for Mycosis Fungoides in Geriatrics?

The older age group suffering from mycosis fungoides generally has a poor prognosis and disease progression. They require a longer duration of treatment and have frequent relapses. Advanced age is frequently associated with progressed clinical stages and poor outcomes. Treating the disease earlier may help improve patient outcomes.

What Are the Complications Caused by Mycosis Fungoides in Geriatrics?

The complications are:

1. Subcutaneous fat inflammation.

2. Swelling of lymph nodes.

3. Atypical finger or toenail growth and appearance.

4. Skin ulcers.

5. Chronic itchy skin.

6. Scarring alopecia.

Conclusion

Mycosis fungoides is a form of non-Hodgkin’s disease that can result in poor outcomes in older age patients. Early-stage disease can be cured, and patients lead a longer life. In most advanced cases, they are harder to treat, and the symptoms are managed by palliative care.