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Mycosis fungoides (MF), the most common cutaneous T-cell lymphoma, typically presents in its early stage as inflammatory erythematous patches or plaques, with epidermotropism as the histopathologic hallmark of the disease. Over the past 30 years, numerous atypical types of MF, which deviate from the classic Alibert-Bazin presentation of the disease, have been described. These variants can simulate a wide variety of benign inflammatory skin disorders either clinically, both clinically and histopathologically, or mainly histopathologically. We have summarized the many faces of the disease, which set MF as a "great imitator," with special focus on the differential diagnosis and its benign mimickers.

Background: Primary cutaneous lymphoproliferative disorders in solid organ recipients are rare. Reports of mycosis fungoides (MF) diagnosed in these patients are limited to less than 30 cases, with a heterogeneous disease course. Furthermore, management in terms of type and modulation of the preceding immunosuppressive treatments after MF diagnosis was not uniform. Objective: To report the characteristics of MF, treatment response, disease course, and status of immunosuppressive therapy in patients who are solid organ recipients, or under similar immunosuppression. Methods: The medical files were retrospectively reviewed for all solid organ transplant patients/patients who are under similar immunosuppression for other indications, who were also diagnosed with MF, and were followed at the cutaneous lymphoma outpatient clinics of 2 tertiary medical centers from 1/2010 to 12/2020. Results: The cohort included 11 patients (8 male; median age at organ transplantation/immunosuppressive state, 33 years). Transplanted organs involved the liver (n=5), kidney (n=3), liver and kidney (n=1), or heart (n=1). Patients were treated with mycophenolic acid, tacrolimus, and prednisone (n=5) or tacrolimus (n=5). One patient with nephrotic syndrome received cyclosporine. Median age at diagnosis of MF was 48 years, 45% were diagnosed before the age of 18 years, median time from organ transplantation/immunosuppressive state to diagnosis of MF was 8 years (range 0.5–22 years). Ten patients had early stage, (IA, 5; IB, 5), 50% of whom had early folliculotropic MF (FMF), (in some combined with classic/hypopigmented MF). One patient had advanced MF (IIIA) with folliculotropic erythroderma. Early-stage patients were treated mainly with skin-directed therapies, achieving either partial (50%) or complete response (50%). The erythrodermic patient achieved partial response after being treated with ultraviolet A and narrow-band ultraviolet B with acitretin. Immunosuppressive treatment was modulated in 4/11 patients, (cyclosporine switched to mycophenolic acid, 1; mycophenolic acid stopped and tacrolimus continued, 1; tacrolimus continued at a lower dose, combined with mycophenolic acid and prednisone, 1; tacrolimus stopped, 1). Median time of follow-up was 4 years (range 1–8 years), with no stage progression noted. At the last visit, 6 had no evidence of disease, and 5 had active disease (IA, 4; III, 1). Conclusions: Most solid organ recipient MF patients present with early-stage disease, similar to MF in the general population, with over representation of FMF, and over representation among children. Treatment is challenging due to the notorious higher risk of non-melanoma skin cancer in organ transplant recipients. Furthermore, immunosuppressive therapy modifications to improve MF should be balanced against the risk of solid organ rejection. Response to treatment and course do not seem to differ from MF in the general population, although longer follow-up is needed.

Background Classic Sezary syndrome (SS) is defined by erythroderma, generalized lymphadenopathy, and leukemic blood involvement. Clinical observations suggest that SS begins as a nonerythrodermic disease. Objective To describe the early clinical characteristics of patients with SS. Methods A retrospective, multicenter chart review was performed for 263 confirmed cases of SS diagnosed during 1976-2015. Results Erythroderma was the earliest recorded skin sign of SS in only 25.5% of cases, although most patients (86.3%) eventually developed erythroderma. In patients without erythroderma during their initial visit, the first cutaneous signs of SS were nonspecific dermatitis (49%), atopic dermatitis-like eruption (4.9%), or patches and plaques of mycosis fungoides (10.6%). The mean diagnostic delay was 4.2 years overall, 2.2 years for cases involving erythroderma at the initial presentation, and 5.0 years for cases not involving erythroderma at the initial presentation. Limitations This study is retrospective. Conclusion Erythroderma is uncommon as an initial sign of SS. Early SS should be considered in cases of nonerythrodermic dermatitis that is refractory to conventional treatments. In these cases, examination of the blood by PCR for monoclonal T-cell receptor rearrangement and by flow cytometry to identify an expanded or aberrant T-cell population should be considered.

Background The literature on mycosis fungoides (MF) in children/adolescents is sparse. Objective We sought to evaluate the characteristics of juvenile MF in a large cohort. Methods Data were collected on all patients with MF, aged 18 years or younger at the time of clinicopathologic diagnosis, who attended the Rabin Medical Center Dermatology Department, Petach Tikva, Israel, between 1994 and 2012 and were followed up prospectively. Results There were 50 patients (30 male; mean age 11.4 years at diagnosis); 18 (36%) had Fitzpatrick skin type IV or higher. All were given a diagnosis of early-stage disease (IA-IIA) except 1 (tumor stage, IIB). Eight had classic MF lesions only and 42 had other variants, alone or in combination; these were mainly hypopigmented MF (n = 29) and cases with subtle but clear clinicopathologic features of folliculotropic MF (FMF) (n = 18). Among the various skin-targeted therapies, psoralen plus ultraviolet A (systemic/bath) proved beneficial for FMF. During a follow-up period of 0.25 to 15 years (mean 4.5), 2 patients progressed from stage IA to IB or IIA. Limitations Relatively short follow-up is a limitation. Conclusions This case series shows that FMF is not uncommon in children and adolescents. It is characterized by more superficial clinical features and less heavy perifollicular lymphocytic infiltrates than adult FMF, and responds well to psoralen plus ultraviolet A. The prognosis of childhood FMF remains unclear.

Background Human herpesvirus (HHV) 8, an essential etiologic agent of Kaposi sarcoma, is also associated with several lymphoproliferative disorders. The involvement of HHV 8 in mycosis fungoides (MF) and large plaque parapsoriasis (LPP) is controversial, with contradictory reports from various countries worldwide. Objective We sought to investigate the presence of the HHV 8 genome in skin lesions of LPP and early-stage sporadic, familial, and juvenile MF in patients in Israel. Methods Archival paraffin-embedded and frozen samples from skin biopsies of untreated patients with LPP and early-stage MF performed in 1990 through 2006 were randomly collected from the department of dermatology of a tertiary medical center in central Israel. DNA was extracted, and a TaqMan-based real-time polymerase chain reaction assay specific for the K6 gene region was used to detect the HHV 8 genome. Results A total of 46 biopsies were sampled from 11 patients with LPP and 35 with early-stage MF (17 adults with sporadic MF, 10 children, and 8 patients with familial MF). In all, 44 samples were negative for HHV 8 DNA; two samples from adults with sporadic MF were positive. Limitations The presence of HHV 8 antibodies or virus sequences was not assessed in peripheral blood. Conclusion The results of this study, conducted in a region relatively endemic for HHV 8, support most earlier studies showing a lack of association of HHV 8 infection with LPP and sporadic adult-type MF. To our knowledge, the lack of association of HHV 8 infection with juvenile and familial MF has not been previously reported.