Rohmer E, Mitcov M, Cribier B, Lipsker D, Lenormand C
Clinique dermatologique, université de Strasbourg et hôpitaux universitaires de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg cedex, France.
Clinique dermatologique, université de Strasbourg et hôpitaux universitaires de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg cedex, France.
Ann Dermatol Venereol. 2020 Jun-Jul;147(6-7):418-428. doi: 10.1016/j.annder.2020.02.007. Epub 2020 Mar 27.
Poikilodermatous mycosis fungoides is a rare and indolent clinical variant of mycosis fungoides (MF). It can be difficult to distinguish from poikilodermatous parapsoriasis, a group of chronical dermatoses that may sometimes progress to MF. We aimed to specify the clinical, histopathological and developmental features of these entities by means of a retrospective study of 12 cases followed in our center.
We identified cases of poikiloderma for which a diagnosis of MF or parapsoriasis was made by the physician. Photographs and histological slides were reviewed, and a final diagnosis of MF was made if the International Society for Cutaneous Lymphoma criteria for the diagnosis of early MF were fulfilled.
Twelve patients were included, 10 of whom met of the MF criteria. 5 patients had large poikilodermatous patches or thin, well-defined plaques ; 3 patients had the same lesions associated with classical MF lesions ; finally, 4 patients had widespread ill-defined erythematous lesions in a net-like pattern, described as parakeratosis variegata, including 3 MF. 2 patients with well-defined lesions (one associated with classical MF lesions) progressed to the tumoral stage whereas none of the patients with parakeratosis variegata presented such progression. A total of 5 patients had a high skin phototype (IV and V). Two patients had squamous cell carcinoma on poikilodermatous lesions.
Our study suggests that poikilodermatous MF covers a heterogeneous clinical spectrum comprising on one hand a presentation of delimited lesions sharing classical MF risk of progression, and on the other, an entity similar to parakeratosis variegata, an entity overlooked in the French nomenclature, which was particularly benign in our small series, raising the question of its affiliation to the MF group. This question merits further investigation in a larger-scale study.
斑片状蕈样肉芽肿是蕈样肉芽肿（MF）一种罕见且进展缓慢的临床变异型。它可能难以与斑片状副银屑病相区分，斑片状副银屑病是一组慢性皮肤病，有时可能进展为MF。我们旨在通过对本中心随访的12例病例进行回顾性研究，明确这些疾病的临床、组织病理学和发展特征。
我们确定了医生诊断为MF或副银屑病的斑状皮肤病变病例。回顾了照片和组织学切片，如果符合皮肤淋巴瘤国际协会早期MF诊断标准，则做出MF的最终诊断。
纳入12例患者，其中10例符合MF标准。5例患者有大片状斑状皮肤损害或薄的、边界清晰的斑块；3例患者有与经典MF损害相关的相同损害；最后，4例患者有广泛的边界不清的网状红斑性损害，称为斑驳性角化不全，其中包括3例MF。2例边界清晰损害的患者（1例与经典MF损害相关）进展至肿瘤期，而斑驳性角化不全的患者均未出现这种进展。共有5例患者皮肤光型较高（IV型和V型）。2例患者在斑状皮肤损害处发生了鳞状细胞癌。
我们的研究表明，斑片状MF涵盖了一个异质性的临床谱，一方面表现为具有经典MF进展风险的局限性损害，另一方面是一种类似于斑驳性角化不全的疾病，这是法国命名法中被忽视的一种疾病，在我们的小样本系列中其特别良性，这就引发了它是否属于MF组的问题。这个问题值得在更大规模的研究中进一步探讨。
