Camisa C, Helm T N, Sexton C, Tuthill R
Department of Dermatology, Cleveland Clinic Foundation.
J Am Acad Dermatol. 1993 Nov;29(5 Pt 1):696-700. doi: 10.1016/0190-9622(93)70233-j.
Regressing atypical histiocytosis is a recently described disease characterized by recurrent nodules or ulcers. The cutaneous lesions appear abruptly and then regress only to return in a manner reminiscent of lymphomatoid papulosis. Immunophenotypic analysis has revealed that most cases are a form of anaplastic large-cell Ki-1-positive (CD30+) lymphoma.
We describe two patients with Ki-1-positive anaplastic large-cell lymphoma that had clinical and pathologic features of regressing atypical histiocytosis and mimicked benign dermatoses (pyoderma gangrenosum and morphea), causing a delay in confirming the true diagnosis. A third case that was readily recognized as a lymphoma is also presented.
The clinical and histopathologic findings were recorded. In addition, T-cell receptor gene rearrangement and immunophenotyping were determined in the index case.
The index patient and second patient were diagnosed as having Ki-1-positive anaplastic large-cell lymphoma by immunophenotyping and underwent cyclophosphamide, doxorubicin, prednisone, and vincristine (CHOP) chemotherapy with complete remission. The patient detected by chart review died of her disease without receiving antineoplastic therapy; disseminated lymphoma was diagnosed at autopsy. Studies on paraffin-embedded tissue were consistent with Ki-1-positive anaplastic large-cell lymphoma.
Regressing atypical histiocytosis may clinically resemble some benign dermatoses. Recent evaluation of these cases has shown that many represent a form of Ki-1-positive anaplastic large-cell lymphoma. Multiple skin biopsy specimens with immunophenotyping and gene rearrangement studies are required to arrive at the diagnosis.
退行性非典型组织细胞增多症是一种最近描述的疾病，其特征为复发性结节或溃疡。皮肤损害突然出现，然后消退，之后又复发，其方式类似于淋巴瘤样丘疹病。免疫表型分析显示，大多数病例是间变性大细胞Ki-1阳性（CD30+）淋巴瘤的一种形式。
我们描述了2例Ki-1阳性间变性大细胞淋巴瘤患者，其具有退行性非典型组织细胞增多症的临床和病理特征，并酷似良性皮肤病（坏疽性脓皮病和硬斑病），导致确诊延迟。还报告了1例易于诊断为淋巴瘤的病例。
记录临床和组织病理学检查结果。此外，对首例患者进行了T细胞受体基因重排和免疫表型分析。
首例患者和第2例患者经免疫表型分析诊断为Ki-1阳性间变性大细胞淋巴瘤，并接受了环磷酰胺、阿霉素、泼尼松和长春新碱（CHOP）化疗，完全缓解。通过查阅病历发现的患者未接受抗肿瘤治疗，死于该病；尸检诊断为播散性淋巴瘤。石蜡包埋组织的研究结果与Ki-1阳性间变性大细胞淋巴瘤相符。
退行性非典型组织细胞增多症在临床上可能类似于某些良性皮肤病。对这些病例的最新评估表明，许多病例代表Ki-1阳性间变性大细胞淋巴瘤的一种形式。需要多次皮肤活检标本进行免疫表型分析和基因重排研究才能确诊。
