Demirkesen Cuyan, Esirgen Galip, Engin Burhan, Songur Abdullah, Oğuz Oya
Department of Pathology, Cerrahpaşa Medical Faculty, Istanbul University, Istanbul, Turkey.
J Cutan Pathol. 2015 Jan;42(1):22-31. doi: 10.1111/cup.12423. Epub 2014 Dec 8.
The recognition of folliculotropic mycosis fungoides (FMF) may pose diagnostic challenges, owing to the variety of histopathological findings.
In this study, our aim is to describe the broad spectrum of the histopathological patterns in a total of 86 biopsies from 38 patients with FMF, together with the clinical features.
The most frequent histopathologic pattern was the folliculocentric/folliculotropic pattern, with or without follicular mucinosis. Keratin-filled cysts and comedones were the second most common pattern in the biopsies. Other less common findings included widening of the hair follicle orifis with keratotic plugging, reminiscent of keratosis pilaris, granuloma formation, eosinophilic or suppurative folliculitis and basaloid folliculolymphoid hyperplasia. Coexisting syringotropism was present in some biopsies. The CD4 : CD8 ratio was at least 4 : 1 or more in most biopsies. Grouped follicular papules and patch/plaque lesions with follicular prominence were the most frequent clinical findings. Folliculocentric lesions such as milia, cysts and acneiform lesions, alopecia, loss of hair or eyebrows were also seen. In 6 out of 38 (15.8%) patients, transformation to large-cell lymphoma was observed during the follow-up.
The awareness and the identification of the various histopathological presentations of FMF by pathologists, as well as by clinicians, are imperative to prevent diagnostic errors.
由于组织病理学表现多样，毛囊性蕈样肉芽肿（FMF）的诊断可能具有挑战性。
在本研究中，我们旨在描述38例FMF患者共86份活检标本的广泛组织病理学模式以及临床特征。
最常见的组织病理学模式是以毛囊为中心/向毛囊性模式，伴或不伴毛囊黏蛋白沉积症。角质填充囊肿和粉刺是活检标本中第二常见的模式。其他较不常见的表现包括毛囊口增宽伴角化栓形成，类似毛发角化病、肉芽肿形成、嗜酸性或化脓性毛囊炎以及基底样毛囊淋巴样增生。部分活检标本中存在并存的汗腺趋向性。大多数活检标本中CD4:CD8比值至少为4:1或更高。成簇的毛囊丘疹以及伴有毛囊突出的斑片/斑块状损害是最常见的临床表现。还可见以毛囊为中心的损害，如粟丘疹、囊肿和痤疮样损害、脱发、头发或眉毛缺失。38例患者中有6例（15.8%）在随访期间观察到转化为大细胞淋巴瘤。
病理学家以及临床医生了解并识别FMF的各种组织病理学表现对于防止诊断错误至关重要。
