Rapid Communication/Hot Topic Dermatology 2014;229:271–274 DOI: 10.1159/000363319
Received: March 4, 2014 Accepted after revision: April 30, 2014 Published online: October 11, 2014
Hypopigmented Mycosis Fungoides versus Mycosis Fungoides with Concomitant Hypopigmented Lesions: Same Disease or Different Variants of Mycosis Fungoides? Fabricio C. Furlan Bruna A. Pereira Mirian N. Sotto José Antonio Sanches Department of Dermatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil
Abstract Background: Hypopigmented mycosis fungoides (HMF) is a rare subtype of mycosis fungoides (MF). We compared patients with exclusive hypopigmented lesions with a group of MF patients with concomitant different lesions. Methods: 20 patients with HMF only and 14 patients with hypopigmented lesions concomitant with other types of lesions (mixed MF, MMF) were selected. Clinical-epidemiological analysis as well as histological and immunohistochemical studies were performed. Results: HMF and MMF preserve some similarities, like predilection for dark-skinned persons and slow progression, but they also present differences: the exclusive variant is associated with early onset and a clear CD8+ immunophenotype, whereas MMF patients tend to present a predominance of CD4+ cell infiltrates. Histological analysis revealed similar findings; relapsing courses were common. Conclusion: Whether patients are suffering from exclusive HMF or MMF, the presence of hypopigmented lesions can be considered a marker of good prognosis in MF, since both groups presented similar data, such as staging and disease duration. © 2014 S. Karger AG, Basel
© 2014 S. Karger AG, Basel 1018–8665/14/2293–0271$39.50/0 E-Mail [email protected] www.karger.com/drm
Introduction
Mycosis fungoides (MF) is the most common primary cutaneous T cell lymphoma [1, 2]. Hypopigmented MF (HMF) is a clinical variant, first described by Ryan et al. [3] in 1973. Although HMF is not an MF subtype recognized by the World Health Organization (WHO) or the European Organisation for Research and Treatment of Cancer (EORTC) classification (2005), it can present with some peculiar characteristics different from the classical form. There are no criteria, clearly defined in the literature, to define a typical case of HMF; patients with other morphological subtypes of MF lesions and concomitant hypopigmented lesions are usually diagnosed with HMF [4, 5]. The aim of the present study was to analyze and compare a group of MF patients who presented with only hypopigmented lesions and a group of MF patients who presented with other morphological variants besides hypopigmented lesions. Methods Patients were selected between February 2009 and October 2011 from the Cutaneous Lymphoma Unity of the Department of Dermatology, Hospital das Clínicas, University of São Paulo Med-
Fabricio Cecanho Furlan Rua Quatro, no. 36 Rio Claro, SP 13500-030(Brazil) E-Mail facefu @ usp.br
Downloaded by: 70.45.19.143 - 5/12/2015 4:02:27 PM
Key Words Cutaneous T cell lymphoma · Hypopigmentation · Mycosis fungoides · Th1/Th2 cells
b
Color version available online
a
a
b
c
d
Color version available online
Fig. 1. Exclusive HMF as scattered large patches on the trunk (a) and small- to medium-sized patches on the lower limbs (b).
Fig. 2. Morphological variants concomi-
tant with hypopigmented lesions among patients in the MMF group: erythematous (a), poikilodermatous (b), hyperkeratotic (c) and hyperpigmented (d).
272
Dermatology 2014;229:271–274 DOI: 10.1159/000363319
Results
During a 32-month period, from a total of 227 patients evaluated, 20 patients presented only hypopigmented patches (HMF group; fig. 1) and 14 patients presented hypopigmented patches concomitant with other morphological variants (MMF group; fig. 2). HMF and MMF patients’ data are summarized in table 1. Concomitant lesion subtypes identified in MMF patients were predominantly erythematous (5 cases) or poikilodermatous (5 cases), besides hyperpigmented (3 cases), purpuric (2 cases) and hyperkeratotic lesions (1 case); 2 patients showed Furlan/Pereira/Sotto/Sanches
Downloaded by: 70.45.19.143 - 5/12/2015 4:02:27 PM
ical School. Written informed consent was obtained from all subjects and the study was approved by the local Ethics Committee. Pathological studies were performed on hematoxylin and eosin slides obtained from biopsies of hypopigmented lesions in exclusive HMF patients and hypopigmented and non-hypopigmented lesions in mixed MF (MMF) patients. Immunohistochemical studies were performed in all cases as described previously [6]. The panel of antibodies used included CD3 (Dako, Denmark; dilution 1: 400), CD4 (Spring Bioscience, USA; dilution 1: 100) and CD8 (Dako, Denmark; dilution 1:600). Statistical analysis was performed using the Wilcoxon t test for quantitative data and χ2 test for qualitative data. A p value 10% of the body surface area without involvement of lymph nodes or viscera) [1]. Almost all of our reported cases had stage I disease; the only exceptions were 2 patients, 1 from each group, classified as stage IIA (T2N1M0). These patients had the longest illness duration (50 and 41 years, respectively). Median disease duration was 12 years in the MMF group and 8.5 years in the HMF group. Despite the better prognosis reported for HMF patients compared to classical MF, patient deaths have been related with this cutaneous lymphoma [5, 7]. Furthermore, evolution from an exclusive hypopigmented form to a mixed form is possible, as we observed in a patient classified as HMF who presented with erythematous patches after the enrollment period. Histological analysis confirmed previously described characteristics of HMF; however, we were not able to establish any histopathological criteria to differentiate HMF and classical MF lesions. Therefore, features routinely observed in classical MF should be considered in a hypopigmented lesion for a histological diagnosis of HMF.
Comparison of HMF and MF with Concomitant Hypopigmented Lesions
Dermatology 2014;229:271–274 DOI: 10.1159/000363319
Age, years Median Range Age at onset, years Median Range Age at diagnosis, years Median Range Disease duration, years Median Range Ethnicity Caucasian Mixed race Black Asian Sex Male Female Anatomical site of lesions Trunk Arms Legs Stage T1N0M0B0 – IA T2N0M0B0 – IB T2N1M0B0 – IIA
HMF
MMF
37.5 17 – 65
48.0 23 – 71
Received: March 4, 2014 Accepted after revision: April 30, 2014 Published online: October 11, 2014
Hypopigmented Mycosis Fungoides versus Mycosis Fungoides with Concomitant Hypopigmented Lesions: Same Disease or Different Variants of Mycosis Fungoides? Fabricio C. Furlan Bruna A. Pereira Mirian N. Sotto José Antonio Sanches Department of Dermatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil
Abstract Background: Hypopigmented mycosis fungoides (HMF) is a rare subtype of mycosis fungoides (MF). We compared patients with exclusive hypopigmented lesions with a group of MF patients with concomitant different lesions. Methods: 20 patients with HMF only and 14 patients with hypopigmented lesions concomitant with other types of lesions (mixed MF, MMF) were selected. Clinical-epidemiological analysis as well as histological and immunohistochemical studies were performed. Results: HMF and MMF preserve some similarities, like predilection for dark-skinned persons and slow progression, but they also present differences: the exclusive variant is associated with early onset and a clear CD8+ immunophenotype, whereas MMF patients tend to present a predominance of CD4+ cell infiltrates. Histological analysis revealed similar findings; relapsing courses were common. Conclusion: Whether patients are suffering from exclusive HMF or MMF, the presence of hypopigmented lesions can be considered a marker of good prognosis in MF, since both groups presented similar data, such as staging and disease duration. © 2014 S. Karger AG, Basel
© 2014 S. Karger AG, Basel 1018–8665/14/2293–0271$39.50/0 E-Mail [email protected] www.karger.com/drm
Introduction
Mycosis fungoides (MF) is the most common primary cutaneous T cell lymphoma [1, 2]. Hypopigmented MF (HMF) is a clinical variant, first described by Ryan et al. [3] in 1973. Although HMF is not an MF subtype recognized by the World Health Organization (WHO) or the European Organisation for Research and Treatment of Cancer (EORTC) classification (2005), it can present with some peculiar characteristics different from the classical form. There are no criteria, clearly defined in the literature, to define a typical case of HMF; patients with other morphological subtypes of MF lesions and concomitant hypopigmented lesions are usually diagnosed with HMF [4, 5]. The aim of the present study was to analyze and compare a group of MF patients who presented with only hypopigmented lesions and a group of MF patients who presented with other morphological variants besides hypopigmented lesions. Methods Patients were selected between February 2009 and October 2011 from the Cutaneous Lymphoma Unity of the Department of Dermatology, Hospital das Clínicas, University of São Paulo Med-
Fabricio Cecanho Furlan Rua Quatro, no. 36 Rio Claro, SP 13500-030(Brazil) E-Mail facefu @ usp.br
Downloaded by: 70.45.19.143 - 5/12/2015 4:02:27 PM
Key Words Cutaneous T cell lymphoma · Hypopigmentation · Mycosis fungoides · Th1/Th2 cells
b
Color version available online
a
a
b
c
d
Color version available online
Fig. 1. Exclusive HMF as scattered large patches on the trunk (a) and small- to medium-sized patches on the lower limbs (b).
Fig. 2. Morphological variants concomi-
tant with hypopigmented lesions among patients in the MMF group: erythematous (a), poikilodermatous (b), hyperkeratotic (c) and hyperpigmented (d).
272
Dermatology 2014;229:271–274 DOI: 10.1159/000363319
Results
During a 32-month period, from a total of 227 patients evaluated, 20 patients presented only hypopigmented patches (HMF group; fig. 1) and 14 patients presented hypopigmented patches concomitant with other morphological variants (MMF group; fig. 2). HMF and MMF patients’ data are summarized in table 1. Concomitant lesion subtypes identified in MMF patients were predominantly erythematous (5 cases) or poikilodermatous (5 cases), besides hyperpigmented (3 cases), purpuric (2 cases) and hyperkeratotic lesions (1 case); 2 patients showed Furlan/Pereira/Sotto/Sanches
Downloaded by: 70.45.19.143 - 5/12/2015 4:02:27 PM
ical School. Written informed consent was obtained from all subjects and the study was approved by the local Ethics Committee. Pathological studies were performed on hematoxylin and eosin slides obtained from biopsies of hypopigmented lesions in exclusive HMF patients and hypopigmented and non-hypopigmented lesions in mixed MF (MMF) patients. Immunohistochemical studies were performed in all cases as described previously [6]. The panel of antibodies used included CD3 (Dako, Denmark; dilution 1: 400), CD4 (Spring Bioscience, USA; dilution 1: 100) and CD8 (Dako, Denmark; dilution 1:600). Statistical analysis was performed using the Wilcoxon t test for quantitative data and χ2 test for qualitative data. A p value 10% of the body surface area without involvement of lymph nodes or viscera) [1]. Almost all of our reported cases had stage I disease; the only exceptions were 2 patients, 1 from each group, classified as stage IIA (T2N1M0). These patients had the longest illness duration (50 and 41 years, respectively). Median disease duration was 12 years in the MMF group and 8.5 years in the HMF group. Despite the better prognosis reported for HMF patients compared to classical MF, patient deaths have been related with this cutaneous lymphoma [5, 7]. Furthermore, evolution from an exclusive hypopigmented form to a mixed form is possible, as we observed in a patient classified as HMF who presented with erythematous patches after the enrollment period. Histological analysis confirmed previously described characteristics of HMF; however, we were not able to establish any histopathological criteria to differentiate HMF and classical MF lesions. Therefore, features routinely observed in classical MF should be considered in a hypopigmented lesion for a histological diagnosis of HMF.
Comparison of HMF and MF with Concomitant Hypopigmented Lesions
Dermatology 2014;229:271–274 DOI: 10.1159/000363319
Age, years Median Range Age at onset, years Median Range Age at diagnosis, years Median Range Disease duration, years Median Range Ethnicity Caucasian Mixed race Black Asian Sex Male Female Anatomical site of lesions Trunk Arms Legs Stage T1N0M0B0 – IA T2N0M0B0 – IB T2N1M0B0 – IIA
HMF
MMF
37.5 17 – 65
48.0 23 – 71
