Shamim Huma, Johnson Emma F, Gibson Lawrence E, Comfere Nneka
Department of Dermatology, Mayo Clinic Rochester, Rochester, Minnesota.
Department of Laboratory Medicine and Pathology, Mayo Clinic Rochester, Rochester, Minnesota.
J Cutan Pathol. 2019 Sep;46(9):645-652. doi: 10.1111/cup.13477. Epub 2019 May 10.
Mycosis fungoides (MF) is characterized by epidermotropic atypical lymphocytes in the absence of spongiosis. However, we describe an unusual presentation of MF with spongiosis, mimicking benign inflammatory dermatoses and highlight the importance of pathologic interpretation for diagnostic confirmation.
A cross-sectional study of consecutive patients diagnosed with MF with spongiosis was conducted. The clinical, histopathologic, immunophenotypic, and molecular genetic features of cases were reviewed.
We identified nine cases of MF (age range 34-82 years; mean 75 years), with an initial diagnosis of dermatitis (6/9), psoriasis (4/9), or other inflammatory dermatoses (2/9). Pruritus, pain, and blisters were common clinical manifestations. The most common areas of involvement were the extremities (8/9). Epidermotropism with spongiosis was a central histopathological feature in all cases.
These cases highlight prominent spongiosis in MF and overlap with common benign inflammatory dermatoses. We present these cases to show the diagnostic pitfalls associated with spongiotic presentations of MF. Dermatitis, psoriasis, and other inflammatory skin conditions not responsive to standard therapy warrant further work-up including biopsy to rule out MF. Multiple skin biopsies and review by a dermatopathologist with expertise in the diagnosis of cutaneous lymphoma is highly recommended.
蕈样肉芽肿（MF）的特征是表皮趋向性非典型淋巴细胞，且无海绵形成。然而，我们描述了一例伴有海绵形成的MF异常表现，其类似良性炎症性皮肤病，并强调了病理诊断对于确诊的重要性。
对连续诊断为伴有海绵形成的MF患者进行横断面研究。回顾了病例的临床、组织病理学、免疫表型和分子遗传学特征。
我们确定了9例MF患者（年龄范围34 - 82岁；平均75岁），最初诊断为皮炎（6/9）、银屑病（4/9）或其他炎症性皮肤病（2/9）。瘙痒、疼痛和水疱是常见的临床表现。最常受累的部位是四肢（8/9）。伴有海绵形成的表皮趋向性是所有病例的核心组织病理学特征。
这些病例突出了MF中显著的海绵形成，且与常见的良性炎症性皮肤病有重叠。我们展示这些病例以表明与MF海绵形成表现相关的诊断陷阱。对标准治疗无反应的皮炎、银屑病和其他炎症性皮肤病需要进一步检查，包括活检以排除MF。强烈建议进行多次皮肤活检，并由在皮肤淋巴瘤诊断方面有专业知识的皮肤病理学家进行复查。
