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Erythroderma as an indicator of hypereosinophilic syndrome

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eMediNexus    19 November 2021

The commonest type of cutaneous T-cell lymphoma (CTCL) is Mycosis fungoides (MF).1,2 MF, an archetype of CTCLs, is a peripheral epidermotropic non-Hodgkin lymphoma of low-quality malignancy that is primarily present in the skin and shows clinical progression through the tumor, patch, plaque, and erythrodermic stages, as well as poor endurance in these progressive stages. Various developments have been made in cellular and immunologic studies by the International Society for Cutaneous Lymphomas/European Organization of Research and Treatment of Cancer (ISCL/EORTC) to reclassify the disease since 1979.3 MF has many clinical deviations with diverse and atypical presentations, and therefore has received the title of second great mimicker after syphilis. Clinical suspicion of MF permits for early analysis and treatment with improved patient survival.

A 32-year-old female came with complaint of numerous itchy lesions throughout the body, which were originally flat but had slowly increased over the last six months. A few of the lesions had been battered with a smelly discharge. Excoriation marks were there throughout the body. A clinical diagnosis of MF was made, and numerous skin biopsies were taken from different sites. On examination, the scanner view exposed a thick diffuse lymphocytic infiltrate in the upper and mid dermis with no particular adnexotropism. Higher intensification demonstrates that these atypical lymphocytes were primarily organized in an interstitial pattern. It is essential to distinguish MF from numeral of other diseases such as Sézary syndrome, adult T-cell leukaemia, psoriasis, drug reactions, atopic dermatitis and other forms of erythrodermas. A comprehensive clinical examination and appropriate investigations comprising IHC will develop the chances of accurate and early diagnosis. Early analysis and management recover the outcome. Consistent follow up visit is essential to observe the advancement of Sézary syndrome.

Suggested Reading

  1. Jerajani HR and Ambade G (2008) Lymphoproliferative disorders, edValia RG IADVL Textbook of Dermatology 3rd edn (Mumbai: Bhalani Publishing House) pp 1539-77.
  2. Weinstock MA and Horm JW (1988) Mycosis in the United States increasing incidence and descriptive dermatology JAMA 260 42-6.
  3. Hans-Uwe Simon et al (2010) Refining the definition of hypereosinophilic syndrome J Allergy ClinImmunol 126(1) 45-9.
  4. Nashan D et al (2007) Mycosis fungoides: a dermatological masquerader Br J Dermatol 156 1-10.

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