Gustavo M Amorim1, Joao P Niemeyer-Corbellini2, Danielle C Quintella3, Tullia Cuzzi3, Marcia Ramos-E-Silva4. 1. Sector of Dermatology and Post Graduation Program in Medical Clinics, HUCFF-UFRJ and School of Medicine, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil. 2. Sector of Dermatology and Post-Graduation Course in Dermatology, University Hospital and School of Medicine, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil. 3. Department of Pathology, University Hospital and School of Medicine, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil. 4. Sector of Dermatology, Post-Graduation Course in Dermatology and Post Graduation Program in Medical Clinics, University Hospital and School of Medicine, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
Abstract
BACKGROUND: Hypopigmented mycosis fungoides (hMF) is a rare subtype of mycosis fungoides. The aim of this study was to identify the clinical-epidemiological profile of our patient group and also to provide additional information about treatment responses and prognosis. METHODS: This is a cross-sectional retrospective observational study, with exploratory analysis. The outcome variables were disease progression and related death. RESULTS: Twenty patients with hMF were selected from a group of 102 patients diagnosed with MF. There was no gender difference (10 females and 10 males). Mean age at diagnosis was 43.85 years, and most patients had mixed or black skin color. The mean time between the onset of the lesions and the diagnosis was 66.75 months. Patients were equally distributed in stages IA (50%) and IB (50%). Photochemotherapy (psoralen and ultraviolet A) was the predominant therapeutic modality. The mean follow-up time was 7.25 years. In 10%, disease progression was observed. Death related to the disease occurred in one patient. CONCLUSIONS: The clinical and epidemiological profile of patients with hypopigmented MF found in our sample is in agreement with what is described in the literature, with the exception of the age at diagnosis, higher than expected. Diagnostic delay time, despite long, is also consistent with the medical literature; however, in this sample, we had two cases of disease progression, with death of one patient, despite the treatment, which is extremely important since hypopigmented MF is usually associated with good prognosis.
BACKGROUND:Hypopigmented mycosis fungoides (hMF) is a rare subtype of mycosis fungoides. The aim of this study was to identify the clinical-epidemiological profile of our patient group and also to provide additional information about treatment responses and prognosis. METHODS: This is a cross-sectional retrospective observational study, with exploratory analysis. The outcome variables were disease progression and related death. RESULTS: Twenty patients with hMF were selected from a group of 102 patients diagnosed with MF. There was no gender difference (10 females and 10 males). Mean age at diagnosis was 43.85 years, and most patients had mixed or black skin color. The mean time between the onset of the lesions and the diagnosis was 66.75 months. Patients were equally distributed in stages IA (50%) and IB (50%). Photochemotherapy (psoralen and ultraviolet A) was the predominant therapeutic modality. The mean follow-up time was 7.25 years. In 10%, disease progression was observed. Death related to the disease occurred in one patient. CONCLUSIONS: The clinical and epidemiological profile of patients with hypopigmented MF found in our sample is in agreement with what is described in the literature, with the exception of the age at diagnosis, higher than expected. Diagnostic delay time, despite long, is also consistent with the medical literature; however, in this sample, we had two cases of disease progression, with death of one patient, despite the treatment, which is extremely important since hypopigmented MF is usually associated with good prognosis.