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A challenging case needs your expertise

Can you help?

Treatment-refractory skin conditions can be a frustrating reality for both patients and healthcare professionals. Some rarer diseases may mimic benign skin conditions that you could see in your daily practice.1–5

We need your expertise to help diagnose our clinical case.

BUT FIRST

Please confirm that you are a healthcare professional and select your specialism using the options provided:

Sorry. If you are not a registered healthcare professional, you can’t take our challenge. For more information please visit the Kyowa Kirin International website.

Dermatologist

Dermato-oncologist

General practitioner

Haematologist

Haemato-oncologist

Oncology

Other healthcare professional

I am not a healthcare professional

Meet Mr K. Mills

Mr Mills is a 62-year-old taxi driver.

He initially presented to a doctor 8 years ago with itchy lesions primarily on his arms, consistent with atopic dermatitis (eczema) and was initiated on a trial of emollients.1–4

Mr Mills presents today with a worsening of his condition…3

to review the diagnostic clues

A typical lesion observed in Mr Mills with irregular, scaly, erythematous patches.

Permission for use of clinical image obtained.

Clue 1: Presenting complaint

Despite emollients, Mr Mills reported pruritus and a gradual worsening and increase in number of his skin lesions.1,2,6

A mild steroid was commenced and did clear some of the early lesions, but over time these lesions reappeared and new, thicker lesions (irregular, scaly, erythematous patches) developed in untreated areas like the one on the left.2,3,6

At this stage do you still think Mr Mills has eczema?

Yes
No

%

of those that completed this challenge agree with you

Assessing skin dermatoses isn’t an exact science; it can be hard to know whether or not to investigate further, especially with limited evidence. But we have additional information for you…

to review the next clue

A thickened plaque observed on Mr Mills’ skin after several months.

Permission for use of clinical image obtained.

Clue 2: Plaque characteristics

Over the years, the most pronounced of Mr Mills’ lesions become more raised, appearing as well-demarcated annular plaques with a scaly surface. Other lesions are flat and erythematous.2,7,8

Mr Mills volunteered that the condition always seems less severe for a period of time following his annual holidays to Greece.9

Which diagnosis would you suspect based on this additional information?

For more atypical presentations, a skin biopsy and further investigations might be helpful for the differential diagnoses.1,2

Eczema?

Psoriasis?

Lichen planus?

Tinea corporis?

Other(s)?

%

of those that completed this challenge agree with you

to review the next clue

‘Bathing suit distribution’

Permission for use of clinical image obtained.

Clue 3: Trunk involvement

Upon recent full body examination, Mr Mills now has mixed patch and plaque lesions affecting his trunk and buttocks, in what might be described as a ‘bathing suit distribution’, beyond the original lesions that mainly affected his arms.10,11

Does this change your thinking and/or the working of diagnosis if you have one in mind?

Yes
No

%

of those that completed this challenge agree with you

The ‘bathing suit’ distribution is an interesting characteristic of this condition,10,11 but there is also further important information to consider

to review the next clue

Clue 4: Lymph nodes

Concerned that this disease process is more serious and may not simply affect the skin, you examine Mr Mills’ lymph nodes. On palpation, you detect peripheral lymphadenopathy.2

Does this change your thinking at this point?

Yes
No

%

of those that completed this challenge agree with you

Peripheral lymph node involvement points to an uncomfortable possibility here12

to review the next clue

Clue 5: Other information

Mr Mills’ case was discussed at a multidisciplinary team meeting and someone believes that they may recognise the pattern of symptoms, especially owing to the way in which they have progressed. They mentioned that it can affect others body compartments in addition to skin and lymph nodes, including blood and viscera.2,11

Would you now consider rarer, potentially more serious, conditions in a patient with this presentation?

Yes
No

%

of those that completed this challenge agree with you

It’s time to diagnose the uncomfortable possibility

to review the next clue

mr mills case study pdf download

View & download this case summary for your records (PDF)

Let’s review Mr Mills’ details

Basic information

  • Male

  • 62 years old

Presentation

  • Itchy lesions, primarily on his arms, similar to atopic dermatitis (eczema)

Additional information from consultation

  • Unsatisfactory/incomplete response to corticosteroids6

  • Lesions on sun-protected sites1,2

  • Some level of improvement to lesions when exposed to sunlight9

  • Peripheral lymph node involvement1,2

Mr Mills‘ diagnosis

Mr Mills has a rare, serious and potentially life-threatening disease called mycosis fungoides (MF), a cutaneous T-cell lymphoma (CTCL) that can imitate many dermatological disorders.1-4,12

CTCLs are a heterogeneous group of extranodal non-Hodgkin’s lymphomas that are characterised by a cutaneous infiltration of malignant monoclonal T lymphocytes.1,13,14 MF accounts for around 60% of all CTCLs.15

Average time-to-diagnosis is around 4 years but can take decades in some instances.16 At early stage (patches and plaques), the disease is usually indolent, but 25% of patients progress to advanced stage in which the median life expectancy is only around 3 years.17,18

Sézary syndrome (SS) is another subtype of CTCL and it accounts for around 5% of cases.15,19 Patients with SS have erythroderma, lymphadenopathy, and blood involvement with clonally-related neoplastic T cells.15

Are there any patients that you have seen recently who have been refractory to conventional treatment for a long time and/or who seem to continue to have progressive disease, albeit slowly?

Yes
No

MF and SS hide in plain sight

Help diagnose these
elusive blood cancers

Thank you for taking part in our digital case study.

A definitive diagnosis of MF can be difficult due to highly variable presentations and the sometimes, non-specific nature of histological findings.1,3

While the case outlined in this tool fits a potential profile of an MF patient, a definitive diagnosis is based on a combination of clinical characteristics, histopathology, immunopathology, and molecular biology workups.1

If a patient presents with symptoms similar to those illustrated here, and MF or SS are suspected, we highly recommend referral to a CTCL expert centre, where formal diagnosis and investigation can inform accurate disease staging, and hence appropriate treatment.1

If you would like more information about CTCL please contact your local Kyowa Kirin representative or visit www.international.kyowa-kirin.com

References:

  1. Harvey NT et al. J Hematopathol 2015;8:209–223.

  2. Kelati A et al. Int J Women Dermatol 2017;3:100–106.

  3. Wobser M et al. World J Dermatol 2015;4(4):135–144.

  4. Reddy K & Bhawan J. J Cutan Pathol 2007;34(7):519–525.

  5. Diwan AH et al. Am J Clin Pathol 2005;123(4):510–515.

  6. Pimpinelli N et al. J Am Acad Dermatol 2005;53(6):1053–1063.

  7. Reddy K & Bhawan J. J Cutan Pathol 2007;34:519–525.

  8. Haun PL & Scarisbrick J. Fast Facts: Diagnosing Cutaneous T-Cell Lymphoma. Health Press, 2016.

  9. Vieyra-Garcia PA & Woolf P. Front Med (Lausanne) 2018.5:232.

  10. Scarisbrick J et al. Br J Dermatol 2014 170:1226–1236.

  11. Hughes CFM et al. Australas J Dermatol 2016;57(3):182–191.

  12. Agar NS et al. J Clin Oncol 2010;28:4730–4739.

  13. Pulitzer M. Clin Lab Med 2017;37(3):527–546.

  14. Bagherani N & Smoller BR. F1000Research 2016, 5(F1000 Faculty Rev):1882 (doi: 10.12688/f1000research.8829.1)

  15. Willemze R et al. Blood 2019;133(16):1703–1714.

  16. Wilcox RA et al. Am J Hematol 2016; 91:182–191. (doi:10.1002/ajh.24233)

  17. Scarisbrick J et al. Br J Dermatol 2019;181:350–357.

  18. Scarisbrick J. EMJ Hematol 2018;6(1):92–100.

  19. Trautinger F et al. Eur J Cancer 2017;77:57–74.

  20. Spicknall KE. Semin Cutan Med Surg 2018;37:18–23.

Job code: KKI/INT/MOG/0249

Date of preparation: September 2020