DOI: http://dx.doi.org/10.18203/issn.2455-4529.IntJResDermatol20204567

A puzzling diagnosis: mycosis fungoides masquerading as pyoderma gangrenosum

Neil K. Jairath, Ruple Jairath, Krislyn Porter, Jon C. Davis

Abstract


Mycosis fungoides (MF) is a form of cutaneous T cell lymphoma that initially affects the epidermis, and is characterized by the clonal proliferation of mature cluster of differentiation (CD4)+T cells. We report a 73-year old male presenting with chin and right axillary skin lesions that had ulceration and granulating areas. Initial biopsy revealed mixed inflammatory cell infiltrate, which suggested an initial diagnosis of pyoderma gangrenosum. Despite treatment, the progressive worsening of the skin lesions prompted multiple repeat biopsies, which eventually revealed a predominance of T cells within the infiltrate. A T cell receptor rearrangement resulted in elevated monoclonal T cell populations, confirming a diagnosis of MF. Subsequent positron emission tomography (PET) scans revealed metastatic involvement of the disease, which ultimately led to the patient’s death. Clinical presentations of MF can mimic several different clinical entities, including pyoderma gangrenosum. This report highlights the importance of a multimodal approach to the diagnosis of unidentified skin lesions.


Keywords


Mycosis fungoides, Pyoderma gangrenosum, Cluster of differentiation, Cutaneous T cell lymphoma

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References


Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, et al. The 2018 update of the WHO-EORTC classification for primary cutane ous lymphomas. Bloo. 2019;133(16):1703-14.

Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Bloo. 2007;110(6):1713-22.

Agar NS, Wedgeworth E, Crichton S, Mitchell TJ, Cox M, Ferreira S, et al. Survival outcomes and prognostic factors in mycosis fungoides/Sézary syndrome: validation of the revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer staging proposal. J Clinic Oncol. 2010;28(31): 4730-9.

Willemze R. Mycosis fungoides variants-clinicopathologic features, differential diagnosis, and treatment. Semin Cutan Med Surg. 2018; 37(1):11-7.

Nashan D, Faulhaber D, Ständer S, Luger TA, Stadler R. Mycosis fungoides: a dermatological masquerader. Br J Dermatol. 2007;156(1):1-10.

Martínez-Escala ME, González BR, Guitart J. Mycosis Fungoides Variants. Surg Pathol Clin. 2014;7(2):169-89.

Zackheim HS, McCalmont TH. Mycosis fungoides: the great imitator. J Am Acad Dermatol. 2002;47(6):914-8.

Ahn CS, ALSayyah A, Sangüeza OP. Mycosis fungoides: an updated review of clinicopathologic variants. Am J Dermatopathol. 2014;36(12):933-48

Cerroni L. Mycosis fungoides-clinical and histopathologic features, differential diagnosis, and treatment. Semin Cutan Med Surg. 2018;37(1):2-10.

Pimpinelli N, Olsen EA, Santucci M, Vonderheid E, Haeffner AC, Stevens S, et al. Defining early mycosis fungoides. J Americ Academ Dermatol. 2005;53(6):1053-63.

Saha M, Jain BB, Chattopadhyay S, Podder I. CD8-positive Mycosis Fungoides Masquerading as Pyoderma Gangrenosum. Indian J Dermatol. 2016; 61(5):580.

Dueck D, Hermes B, Audring H, Kohl PK. [CD8-positive, CD30-negative cutaneous T-cell lymphoma simulating pyoderma gangrenosum]. Hautarzt. 2002;53(2):114-7.

Salameh F, Barzilai A, Baum S, Trau H. Mycosis fungoides and CD30+ cutaneous T-cell lymphoma simulating pyoderma gangrenosum in a patient with ulcerative colitis. J Dermatol Case Rep. 2009; 3(2):30-3.

Cozzani E, Scaparro M, Rongioletti F, Pierri I, Pimpinelli N, Parodi A. Pyoderma gangrenosum-like CD30+ cutaneous T-cell lymphoma in a patient with mycosis fungoides. J Eur Acad Dermatol Venereol. 2015;29(4):819-21.

Ho KK, Browne A, Fitzgibbons J, Carney D, Powell FC. Mycosis fungoides bullosa simulating pyoderma gangrenosum. Br J Dermatol. 2000;142(1):124-7.

Smoller BR, Bishop K, Glusac E, Kim YH, Hendrickson M. Reassessment of histologic parameters in the diagnosis of mycosis fungoides. Am J Surg Pathol. 1995;19(12):1423-30.

Nasu K, Said J, Vonderheid E, Olerud J, Sako D, Kadin M. Immunopathology of cutaneous T-cell lymphomas. Am J Pathol. 1985;119(3):436-47.

Zinzani PL, Ferreri AJ, Cerroni L. Mycosis fungoides. Crit Rev Oncol Hematol. 2008;65(2):172-82.