I recently read an article by Roger and Lake (2022) called, Mycosis fungoides in skin of color published in the Journal of the Dermatology Nurses' Association (JDNA). The authors highlight the diagnostic and treatment disparities in skin of color (SOC) patients with mycosis fungoides. It's a topic not common in nurse practitioner school, and so I thought it was fitting to discuss on this platform which targets the education, training, and professional development of Aspiring Dermatology Nurse Practitioners with an interest in treating SOC. In this blog article, I define mycosis fungoides, describe the prevalence and incidence rates, clinical presentation, diagnosis, treatment, and prognosis of mycosis fungoides in SOC. I discuss the implications of the Roger and Lake et al. study for Aspiring Dermatology Nurse Practitioners and how we can improve public awareness of skin cancer when it comes to SOC patients.
The Study
Taylor and Eden (2022) wrote the article, Mycosis Fungoides in Skin of Color, to increase awareness about MF in SOC patients in an effort to improve early detection, and morbidity and mortality rates. One reason this study is significant is because diagnosing MF in SOC patients has proven to be challenging.
Mycosis Fungoides
Cutaneous T-cell lymphomas (CTCLs) refer to a group of non-Hodgkin lymphomas that primarily effect the skin. Mycosis fungoides (MF) is the most common form (50% of all cases) of CTCL's. You may hear MF and CTCL inappropriately used interchangeably. However, while all cases of MF classify as a CTCL, the opposite is not true as there is more than one type of CTCL. As a family of disease, CTCLs are a group of rare cancers that effect white blood cells (a component of the immune system that helps fight infection and disease). Ten percent of the time, MF will progress to the lymph nodes and internal organs.
MF patch stage. Image source: The Full Spectrum of Dermatology: A Diverse and Inclusive Atlas.
Skin of Color
On average, 3,000 people are diagnosed annually (incidence rate of 0.3-0.6 per 100,000) with MF, while as many as 30,000 people live with MF during any given year (prevalence rate) between the United States and Canada. MF has a 2:1 male to female ratio with men being diagnosed more often than women, and usually around age 51-52 in Black patients. Early-onset (before age 40) is seen more often in Black and Hispanic women. Unfortunately, we see the same degree of disparity when it comes to misdiagnosis, late diagnosis, advanced disease at time of diagnosis, and poor outcomes in skin of color (SOC) patients diagnosed with MF that we see in SOC patients diagnosed with breast cancer and melanoma.
MF Variants. MF has varied presentations and undergoes multiple changes throughout the disease process complicating appropriate diagnosis and timely treatment. The subtypes of MF include classic, hypopigmented, folliculotropic, and verrucous. Diagnosis is further complicated by the fact MF is known to mimic inflammatory and infectious diseases like eczema and psoriasis, and is often misdiagnosed in the beginning as atopic dermatitis (AD) and tinea. In the pictures accompanying this article you can see MF easily resembles a drug eruption, allergic contact dermatitis, plaques and pustular psoriasis. SOC patients often present with the hypopigmented variant associated with pruritic patches on nonexposed areas (parts of the body covered by a bathing suit) like the hips and buttocks. This unusual presentation reflects the rate of misdiagnosis especially given the paucity of SOC education and training in academic nursing and medical school education, particularly when it comes to darker skin tones. Considering its rarity, I wonder how many people have the opportunity to diagnosis and treat MF during nursing and medical school training.
Folliculotropic MF presents as follicular plugging, hyperpigmented or violaceous perfollicular halos and perifollicular scale. Verrucous MF presents as large multicolored amorphous structures with yellow gray ridges and comedo-like openings. The pathogenesis of MF is not completely understood, however there is some evidence that suggests the presence of chronic inflammation, malignant hemopathies, or an immunosuppressive microenvironment may create an inviting atmosphere for cancer growth (Roger and Lake, 2022).
Image source: DermNetZ.org
MF in SOC Snapshot
-2:1 male to female ratio
-Men often diagnosed around age 51-52
-Onset at younger age
-Early onset (younger than age 40) seen in Black and Hispanic women
-Hypopigmented variant more common in SOC, easier to treat, and has better prognosis
-High eosinophil blood count
-May require early aggressive treatment for remission
-Needs enhance/advanced surveillance, close monitoring, and regular follow-ups
Diagnosis
Diagnosis is based on clinical presentation and can be enhanced with dermoscopy which can aid in determining which lesions to biopsy. Dermoscopy generally reveals pseudonetworks (absent in hypopigmented variant), geometric white lines, and white rosettes. An accurate and timely diagnosis is prolonged by the number of biopsies, visits, and immunohistochemical analyses required to identify the disease. The International Society for Cutaneous Lymphomas developed a 7-point scoring system to diagnosis MF based on the clinical, histopathological, and immunopathological presentation. A score of four or more is diagnostic for MF (Roger and Lake, 2022).
Treatment
The treatment is dependent on MF subtype, stage and symptoms. Treatment can also be categorized as skin-directed and systemic therapies. You'll recognize these dermatology mainstays of treatment including, topical steroids, phototherapy and retinoids, particularly in the early stages (Roger and Lake, 2022; Taylor and Kelly, 2016). Most Aspiring Dermatology Nurse Practitioners are likely unfamiliar with nitrogen mustard (a powder) which is an alkylating agent that can be administered topically or intravenously to slow or stop cancer progression. The topical formulation has been used to treat MF since the 1950s and is highly efficacious (76-80% response rate) when treating those with limited patches and plaques, as compared to a generalized presentation (35-68%) (Ngan, 2005). It cannot be applied to the face or genitals (mucous membranes) and is not pregnancy safe. Side effects include contact dermatitis, darkening of the skin, and the rare chance of anaphylaxis as an adverse effect (Ngan, 2005).
During later stages in the disease process, systemic treatment can include biologics, chemotherapy, stem cell transplants, and retinoids. Immunotherapies have yielded better health outcomes when administered as the initial treatment for advanced disease. Antimicrobials compliment systemic therapy very well as cutaneous infections are common in the setting of a compromised skin barrier.
Phototherapy and corticosteroids have been extremely helpful in decreasing the progression of hypopigmented MF, however some reoccurrence is still common. Hyperpigmented MF is more challenging to treat, especially with phototherapy because of the lack of erythema as a measure of phototoxicity and the risk of worsening pigmentation. Topical nitrogen mustard is an option for patients with hyperpigmented MF who don't experience remission following phototherapy, but worsening hyperpigmentation is a potential side effect.
Image source: The Full Spectrum of Dermatology: A Diverse and Inclusive Atlas and DermNetZ.org
Why You’ve Never Heard of Mycosis Fungoides
Most of us have likely never heard of or are not very familiar with MF because it is a rare condition. Prior to dermatology, the most exposure one might receive is to the class of CTCLs, but that is as far as exposure goes in didactic and clinical training. When a list of important dermatologic conditions are listed, it’s rare to see diagnoses that primarily impact SOC patients, even when education targets dermatology specialists. There has been an increasing number of conferences, webinars, and research dedicated to SOC skin, hair, and nail disorders. But, what remains absent is the standardization of SOC clinical strategy in every single presentation. I would love to see more people take the initiative to include SOC when it comes to discussing incidence, prevalence, morbidity and mortality rates; presenting illness, assessment, diagnostics, management pearls, cultural considerations, and how to increase inclusivity to improve health literacy and health outcomes.
Aspiring Dermatology Nurse Practitioners
To Aspiring Dermatology Nurse Practitioners, I encourage you to ask how someone talks to, diagnosis, treats, and evaluates SOC patients anytime you attend a meeting, webinar, class, conference, or consult with another provider. To improve public awareness when it comes to SOC, we need to consider using imagery to get buy-in as many SOC patients need to see someone who looks like them to relate to a topic. Imagery will also help support the verbal conversation as people only remember about 30% of what we tell them, yet 70% of what we show them through nonverbal communication and pictures. The most popular social media sites today are successful because we are a visual global society drawn more to images than to text. Graphics and pictures of SOC patients can be used in skin cancer marketing campaigns, on social media platforms, and on practice websites.
If you have a personal story of MF, I would love to hear about it. If you would like for me to elaborate on treatment options for MF or another rare, but debilitating or fatal disease in SOC, let me know here.
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