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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
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Dermatologist
Dermato-oncologist
General practitioner
Haematologist
Haemato-oncologist
Oncology
Other healthcare professional
I am not a healthcare professional
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.
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?
%
of those that completed this challenge agree with you
to review the next clue
A thickened plaque observed on Mr Mills’ skin after several months.
Permission for use of clinical image obtained.
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?
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.
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?
%
of those that completed this challenge agree with you
to review the next clue
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?
%
of those that completed this challenge agree with you
to review the next clue
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?
%
of those that completed this challenge agree with you
to review the next clue
View & download this case summary for your records (PDF)
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 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?
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