Toro J R, Sander C A, LeBoit P E
Department of Dermatology, University of California, San Francisco 94143-0506, USA.
Am J Dermatopathol. 1997 Apr;19(2):108-18. doi: 10.1097/00000372-199704000-00003.
Mycosis fungoides (MF) can present with purpuric lesions, and rare patients who seemed to have persistent pigmented purpuric dermatitis (PPPD) have developed MF. We recently encountered two patients referred to our cutaneous lymphoma clinic who had PPPD rather than MF and two others who appeared to have both conditions, leading us to explore the histologic similarities of these diseases. We examined specimens from 56 patients with PPPD to determine the frequency of MF-like histologic configurations, namely, the psoriasiform lichenoid, psoriasiform spongiotic lichenoid, and atrophic lichenoid patterns. We also noted the degree of spongiosis, epidermotropism, papillary dermal fibrosis, lymphocytic atypia, and epidermal hyperplasia, the number of extravasated erythrocytes and siderophages, and the distribution of lymphocytic infiltrate within the epidermis. In 29 of 56 patients, there were patterns typically seen in MF. PPPD can feature lymphocytes aligned along the epidermal side of the dermoepidermal junction, with few necrotic keratinocytes, as can MF. Papillary dermal edema occurred frequently in PPPD but not in MF, while lymphocytes in MF but not PPPD had markedly atypical nuclei and had ascended into the upper spinous layer. Given these similarities, we tested for clonality of the T-cell population using a polymerase chain reaction assay for gamma-chain rearrangements. Clonal populations were present in three of three and one of two specimens from patients with both PPPD and MF, but also in 8 of 12 specimens typical of lichenoid patterns of PPPD. These findings raise the possibility that the lichenoid variants of PPPD are biologically related to MF.
蕈样肉芽肿（MF）可表现为紫癜性损害，少数看似患有持久性色素性紫癜性皮炎（PPPD）的患者会发展为MF。我们最近遇到了两名转诊至我们皮肤淋巴瘤诊所的患者，他们患有PPPD而非MF，还有另外两名患者似乎同时患有这两种疾病，这促使我们探究这些疾病的组织学相似性。我们检查了56例PPPD患者的标本，以确定MF样组织学构型的频率，即银屑病样苔藓样、银屑病样海绵状苔藓样和萎缩性苔藓样模式。我们还记录了海绵形成程度、亲表皮现象、乳头真皮纤维化、淋巴细胞异型性和表皮增生情况，外渗红细胞和含铁血黄素细胞的数量，以及淋巴细胞浸润在表皮内的分布。在56例患者中的29例，存在MF中常见的模式。PPPD可表现为淋巴细胞沿真皮表皮交界处的表皮侧排列，坏死角质形成细胞较少，MF也如此。PPPD中乳头真皮水肿频繁出现，但MF中没有，而MF中的淋巴细胞而非PPPD中的淋巴细胞具有明显异型性核，并已上升至棘层上层。鉴于这些相似性，我们使用γ链重排的聚合酶链反应检测法检测T细胞群体的克隆性。在同时患有PPPD和MF的患者的三个标本中的三个以及两个标本中的一个中存在克隆群体，但在12个典型PPPD苔藓样模式标本中的8个中也存在克隆群体。这些发现增加了PPPD的苔藓样变体与MF在生物学上相关的可能性。
