Erythrodermic Mycosis Fungoides with Hypereosinophilic Syndrome: A Rare Presentation |
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Erythrodermic Mycosis Fungoides with Hypereosinophilic Syndrome: A Rare Presentation
Dr Savita Chaudhary, Dr Cherry Bansal, DrUpasna Ranga, Dr KK Singh,  16 October 2020
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Mycosis fungoides (MF) is the most common variant of primary cutaneous T-cell lymphoma (CTCL). It isgenerally associated with an indolent clinical course and characterized by well-defined clinicopathological features.

Although rare, CTCLs constitute 65% of all cutaneous lymphoid malignancies, of which 50% are patients withMF. The erythrodermic variants of MF, a malignancy of mature, skin homing and clonal T lymphocytes, usuallypresent in mid to late adulthood. Association with hypereosinophilia is important in prognosis. We report a case oferythrodermic MF with hypereosinophilic syndrome in a 22-year-old female presenting with gradually progressiveintractable erythroderma with intensely pruritic multiple papules, plaques, and nodules involving more than 90%of body surface area. Diagnosis was confirmed by histopathological examination and immunophenotyping frommultiple skin biopsies.

Introduction

Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma (CTCL).1,2The skin is the second most common site of extranodal lymphomas after the gastrointestinaltract. MF, a prototype of CTCLs, is a peripheral epidermotropic non-Hodgkin lymphoma oflow-grade malignancy that is initially present in the skin and shows clinical progression throughthe patch, plaque, tumor and erythrodermic stages, as well as poor survival in these progressivestages. Various advances have been made in cellular and immunologic studies by theInternational Society for Cutaneous Lymphomas/European Organization of Research andTreatment of Cancer (ISCL/EORTC) to reclassify the disease accordingly since 1979.3 MF hasmany clinical variants with varied and atypical presentations, and thus has earned the title ofsecond great mimicker after syphilis. Clinical suspicion of MF allows for early diagnosis andtreatment with better patient survival.

Case Report

A 22-year-old female presented in the Dermatology Outpatient Department of Era’s LucknowMedical College and Hospital, complaining of multiple itchy lesions all over the body, which were initially flat but had gradually increased in size and number over the past six months. A fewof the lesions had been eroded with a foul-smelling discharge. The patient had receivedtreatment from a local medical practitioner in the form of oral antibiotics and anti-inflammatorydrugs according to medicine strips she showed us, but no detailed records were available, andshe did not have any relief from this treatment. On dermatological examination, multiple plaquesand nodules were distributed all over the body, including the scalp, face, chest, abdomen, andbilateral upper and lower limbs, of size ranging from 1 × 2 to 6 × 7 cm. A few lesions had well-defined margins, while others had ill-defined margins. The color of the lesions varied fromnormal skin color to erythematous. Excoriation marks were present all over the body. A clinicaldiagnosis of MF was made, and multiple skin biopsies were taken from different sites. Onhistopathological examination, the scanner view revealed a dense diffuse lymphocytic infiltrate inthe upper and mid dermis with no particular adnexotropism. Neither was there on any areas ofpale blue-staining mucin pools. Higher magnification revealed that these atypical lymphocyteswere mainly arranged in an interstitial pattern. On immunohistochemistry (IHC), lymphocyticinfiltrate was strongly positive for T-cell marker CD4 but not for CD8. B-cell marker CD20 didnot show positive expression in epidermotropic cells.

Contrast Enhanced Axial Computed Tomographic (CECT) images showed bilateral axillary andinguinal lymph nodes enlarged (white arrows). The dimensions of axillary lymph nodes were;Left: 25 × 17.1 mm, Right: 40.6 × 16 mm and of inguinal lymph nodes were Left: 33.7 × 15 mm,Right: 40.6 × 18.1 mm.

A hematological examination revealed the following values: hemoglobin: 10.9 g/dL (referencerange 14.0-17.5 g/dl); total leukocyte count: 13,500 cells/μL (reference range 4,500-11,000cells/μL); neutrophils: 65% (reference range 40-75%); lymphocytes: 18% (reference range 20-45%); eosinophils: 16% (reference range 01-06%); monocytes: 01% (reference range 01-08%);and absolute eosinophil count (AEC): 2,150/μL (reference range 40-400/μL). Bone marrowaspiration was not conclusive. Liver and kidney function tests were within normal limits. Noorganomegaly was evident. CT-guided aspiration cytology from enlarged axillary and inguinallymph nodes showed reactive lymphoid hyperplasia. Peripheral blood smears did not reveal anySézary cells. According to updated ISCL/EORTC staging classification, she was diagnosed withthe erythrodermic variant of MF, stage IIIA (T4N1M0B0): T4, erythroderma; N1, clinicallyabnormal peripheral lymph node; M0, no visceral organ involvement; and B0, absence ofsignificant blood involvement. Based on the history, examination, histopathology and IHC,diagnosis of erythrodermic MF was made.3 Narrow-band ultraviolet B (UVB) therapy and low-dose methotrexate resulted in improvement of the lesions and a decrease in AEC; regular follow-up visits were conducted for eight months to monitor progression to Sézary syndrome.

Discussion

Alibert first described the classic plaque form of MF in 1806.4 He termed it MF because of theresemblance of the lesions to ‘mushrooms’. MF, a rare CTCL of mature skin homing clonalmalignant T lymphocytes, has an annual incidence of 0.36/100,000 person–years in the UnitedStates.5 It usually presents in mid to late adulthood (in the fifth–sixth decades of life) with a maleto female ratio of 2:1. Blacks have twice the incidence compared with caucasians, as suggested bysome studies. Most of the cases are diagnosed in the fifth and sixth decades of the person’s life(55-60 years of age).4,6,7 The erythrodermic variant was originally described in 1892.

Erythroderma occurs as a progression from the plaque or patch stage of MF (secondary SS), or itmay arise de novo. It differs from Sézary syndrome by the lack of an elevated number ofcirculating Sézary cells and often is termed pre-Sézary erythroderma, indicating that some of thecases eventually progress to Sézary syndrome.8 Our patient had eosinophilia in peripheral blood,and the AEC was 2,150/μL. Since 1975, the following criteria suggested by Chusid et al9 havebeen used for defining hypereosinophilic syndrome (HES)—(a) blood eosinophilia ≥1,500/mm3 for longer than six months (or death before six months associated with signs andsymptoms of hypereosinophilic disease), (b) lack of evidence for parasitic, allergic, or other known causes of eosinophilia and (c) presumptive signs of organ involvement, such as heartfailure, gastrointestinal dysfunction, central nervous system abnormalities, fever, or weight lossthat warrant special attention with regards to the potential development of disease complications.

However, in 2010, Hans- Uwe Simon et al10 proposed some changes in the definition to ensureoptimal clinical evaluation and therapeutic decision and suggested that all patients with bloodeosinophilia ≥ 1,500/mm without a discernible secondary cause (e.g., allergic diseases, drughypersensitivity, parasitic, helminthic infestations, HIV infection and nonhematologicmalignancies) should be considered to have HES or a disorder that overlaps in definition withHES, regardless of the number and nature of affected organs or potential pathogenicmechanisms. In our case, AEC was 2,150/μL, without any signs of eosinophil-mediated organdamage or dysfunction at the time of presentation. There were no signs or symptoms of asthmaor allergic rhinitis, which can lead to eosinophilia. Hence, we labelled our case ‘erythrodermicMF with HES’. Systemic methotrexate and narrow band UVB treatment resulted inimprovement of the lesions, and AEC was decreased to 320/μL.

Conclusion

Our patient was presented at an early age with erythroderma and hypereosinophilia withoutsystemic involvement. It is important to differentiate MF from a number of other diseases suchas Sézary syndrome, adult T-cell leukaemia, psoriasis, drug reactions, atopic dermatitis and otherforms of erythrodermas.11 A detailed history, thorough clinical examination and relevantinvestigations including IHC will improve the chances of correct and early diagnosis. Earlydiagnosis and treatment improve the outcome. Regular follow up visits are required to monitorthe progression of Sézary syndrome.

References

  1. Willemze R et al (1997) EORTC classification for primary cutaneous lymphomas: aproposal from the Cutaneous Lymphoma Study Group of the European Organizationfor Research and Treatment of Cancer Blood 90 354.
  2. Fink-Puches R et al (2002) Primary cutaneous lymphomas: applicability of currentclassification schemes (European Organization for Research and Treatment of Cancer,World Health Organization) based on clinicopathologic features observed in a largegroup of patients Blood 99 800.
  3. Olsen E et al (2007) Revisions to the staging and classification of mycosis fungoides andSézary syndrome: a proposal of the International Society for Cutaneous Lymphomas(ISCL) and the cutaneous lymphoma task force of the European Organization ofResearch and Treatment of Cancer (EORTC) Blood 110 1713-22.
  4. Jerajani HR and Ambade G (2008) Lymphoproliferative disorders, edValia RG IADVLTextbook of Dermatology 3rd edn (Mumbai: Bhalani Publishing House) pp 1539-77.
  5. Weinstock MA and Horm JW (1988) Mycosis in the United States increasing incidenceand descriptive dermatology JAMA 260 42-6.
  6. Vonderheid EC, Bernengo MG and Burg G (2002) Update on cutaneous T celllymphoma: report of international society of cutaneous lymphomas J Am AcadDermatol46 95-105.
  7. Weinstock MA and Gardstei B (1999) Twenty year trend in the reported incidence ofmycosis fungoides and associated mortality Am J Public Health 89 1240-4.
  8. Beuchner SA and Winkelmann RK (1983) Pre-Sézary erythroderma evolving to Sézarysyndrome a report of seven cases Arch Dermatol 119 285-91.
  9. Chusid MJ et al (1975) Thehypereosinophilic syndrome: analysis of fourteen cases withreview of the literature Medicine (Baltimore) 54 1-27.
  10. Hans-Uwe Simon et al (2010) Refining the definition of hypereosinophilic syndrome JAllergy ClinImmunol 126(1) 45-9.
  11. Nashan D et al (2007) Mycosis fungoides: a dermatological masquerader Br J Dermatol156 1-10 .
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