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April 2016

Cutaneous T-Cell Lymphoma Misdiagnosed as Lipodermatosclerosis

Author Affiliations
  • 1Division of Dermatology, Department of Medicine, University of Louisville School of Medicine, Louisville, Kentucky
  • 2Associate Editor, JAMA Dermatology
JAMA Dermatol. 2016;152(4):487-488. doi:10.1001/jamadermatol.2015.6106

Herein, we describe an obese woman with a 2-year history of a persistent indurated plaque on the right calf misdiagnosed as lipodermatosclerosis, while the true diagnosis was mycosis fungoides (MF). To our knowledge, there have been no other cases of MF mimicking lipodermatosclerosis reported.

Report of Case

A woman in her 60s presented with an indurated, erythematous, nontender plaque on her right leg. There was associated aching pain and numbness, but no peripheral edema was noted, and her ankle was not involved. She had previously been evaluated by an internist, a vascular surgeon, a local dermatologist, and a dermatologist at a tertiary referral center, all of whom diagnosed lipodermatosclerosis based on her clinical examination and suggested topical corticosteroid therapy and compression. The vascular surgeon had referred the patient to an oncologic surgeon for a biopsy, but a biopsy was not performed owing to concern about poor healing. The dermatologic consultation had noted “eczematous” changes on the trunk and thighs that responded partially to topical corticosteroids. Vascular studies had revealed venous insufficiency in both legs but worse on the unaffected leg.

Physical examination at presentation revealed an obese woman with nonspecific, slightly scaly patches on her abdomen and tan petechial patches on her legs. There was a 10-cm indurated, erythematous, nontender plaque on the right posteromedial calf (Figure 1). The surface had a peau d’orange appearance. There was no peripheral edema, and the ankles were uninvolved. The distal portion of the legs appeared to be normal in size, and the skin was not bound down. Her thighs were increased in size due to her obesity, giving the overall appearance that there was distal tapering.

Figure 1.
Clinical Presentation of the Case Patient
Clinical Presentation of the Case Patient

Indurated, erythematous, nontender plaque on initial presentation.

An incisional biopsy of the right distal calf was performed revealing a monomorphic population of atypical, dermal-based lymphocytes that were small to medium in size and did not show significant anaplasia (Figure 2). Staining found CD3+ and CD4+ T cells with a diminished expression of CD7 and no CD8 and CD30 expression. A clonal T-cell receptor gene rearrangement was also identified. A second biopsy of a tumor on the contralateral thigh 3 weeks later revealed MF again. A reactive node was present in the right inguinal chain, but findings of a positron emission tomography–computed tomography scan were otherwise negative.

Figure 2.
Incisional Biopsy Specimen From Right Distal Calf Lesion
Incisional Biopsy Specimen From Right Distal Calf Lesion

Epidermotropic lymphocytes are seen occurring in collections and individually along the dermoepidermal junction. Notice the irregular nuclear contours (hematoxylin-eosin, original magnification ×600).

The patient was diagnosed with stage 2B MF and treated first with local radiation therapy and then with oral bexarotene, 75 mg, twice daily. After 4 months there was a complete response.

Discussion

The occurrence of lipodermatosclerosis mimicking MF has been poorly described in the literature, with only 1 other case reported to our knowledge.1 Several clinical morphological and textural features should be considered when evaluating similar lesions on the legs. Lipodermatosclerosis often presents as a localized, painful, indurated plaque with erythema and hyperpigmentation on the lower legs in a setting of venous insufficiency. The chronic form is said to resemble an “inverted champagne bottle.” The typical presentation of MF is erythematous patches, plaques, or tumors with fine scale that favor sun protected areas.2

Treatments for MF are based on the stage of the disease and include topical corticosteroids, topical nitrogen mustard, total skin electron beam therapy, oral retinoids, subcutaneous interferon alfa, histone deacetylase inhibitors, alemtuzumab, psoralen plus UV-A photochemotherapy (PUVA), and extracorporeal photopheresis.

Lipodermatosclerosis is the result of chronic venous insufficiency. Both septal and lobular panniculitis as well as lipomembranous changes are seen on pathologic analysis. Mainstay treatments include compression, leg elevation, topical and/or intralesional corticosteroids, and anabolic steroids for recalcitrant disease.3

Mycosis fungoides should be included in the differential diagnosis of any cutaneous patch or plaque that does not respond to first- and second-line treatments. A low threshold for obtaining biopsy should be the rule for any patient with a chronic, recalcitrant eruption.

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Article Information

Corresponding Author: W. James Tidwell, MD, Division of Dermatology, Department of Medicine, University of Louisville School of Medicine, 3810 Springhurst Blvd, Louisville, KY 40241 (wjamestidwell@gmail.com).

Published Online: February 24, 2016. doi:10.1001/jamadermatol.2015.6106.

Conflict of Interest Disclosures: None reported.

Disclaimer: Dr Callen is Associate Editor of JAMA Dermatology, but he was not involved in any of the decisions regarding review of the manuscript or its acceptance.

References
1.
Labau  D, Frouin  E, Dereure  O,  et al.  Atypical skin lesions of the lower limbs in patients with chronic venous insufficiency: a case of granulomatous fungoid mycosis [in French].  J Mal Vasc. 2012;37(1):15-18.PubMedGoogle ScholarCrossref
2.
Olsen  EA, Whittaker  S, Kim  YH,  et al; International Society for Cutaneous Lymphomas; United States Cutaneous Lymphoma Consortium; Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer.  Clinical end points and response criteria in mycosis fungoides and Sézary syndrome: a consensus statement of the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer.  J Clin Oncol. 2011;29(18):2598-2607.PubMedGoogle ScholarCrossref
3.
Morton  LM, Phillips  TJ.  Venous eczema and lipodermatosclerosis.  Semin Cutan Med Surg. 2013;32(3):169-176.PubMedGoogle Scholar
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