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.
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.
Int J Dermatol. 2018 Mar;57(3):306-312. doi: 10.1111/ijd.13855. Epub 2018 Jan 10.
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.
This is a cross-sectional retrospective observational study, with exploratory analysis. The outcome variables were disease progression and related death.
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.
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.
色素减退型蕈样肉芽肿(hMF)是蕈样肉芽肿的一种罕见亚型。本研究的目的是确定我们患者群体的临床流行病学特征,并提供有关治疗反应和预后的更多信息。
这是一项横断面回顾性观察性研究,具有探索性分析。结局变量为疾病进展和相关死亡。
从 102 例诊断为 MF 的患者中选择了 20 例 hMF 患者。男女比例无差异(10 名女性和 10 名男性)。诊断时的平均年龄为 43.85 岁,大多数患者的皮肤颜色为混合色或黑色。皮损出现到诊断的平均时间为 66.75 个月。患者在 IA 期(50%)和 IB 期(50%)的分布均匀。光化学疗法(补骨脂素和紫外线 A)是主要的治疗方式。平均随访时间为 7.25 年。10%的患者观察到疾病进展。1 例患者因疾病死亡。
我们样本中发现的色素减退型 MF 患者的临床和流行病学特征与文献描述相符,除了诊断时的年龄高于预期。尽管诊断延迟时间较长,但也符合文献报道;然而,在本样本中,我们有 2 例疾病进展,1 例患者死亡,尽管进行了治疗,这一点非常重要,因为色素减退型 MF 通常与良好的预后相关。