Chao-Lo Melanie Pauline G, King-Ismael Daisy, Lopez Rolando A
Department of Dermatology, University Santo Tomas, Philippines.
J Dermatol Case Rep. 2008 Oct 11;2(3):31-4. doi: 10.3315/jdcr.2008.1013.
Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare type of non-Hodgkin's lymphoma comprising approximately 0.9-9.0% of all cutaneous lymphomas. PCALCL is characterized by the absence of systemic involvement, spontaneous regression and low recurrence rate especially in localized lesions.We present a 47-year-old female with a 1½-year history of two asymptomatic erythematous indurated plaques on the right arm. Skin punch biopsy revealed dense infiltrates of non-epidermotropic, large, irregularly-shaped lymphocytes with hyperchromatic and pyknotic nuclei. Immunohistochemistry revealed that these atypical cells are anaplastic lymphoma kinase (ALK) positive, CD30+, CD3-, CD20- and epithelial membrane antigen (EMA) negative. Clinical, histopathological and immunohistochemical findings are consistent with PCALCL. Work-ups revealed no systemic involvement. Short course CHOP (Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) chemotherapy resulted in total resolution of skin lesions; however, recurrence was noted 12 months after treatment. She then underwent radiotherapy and achieved complete remission.Because the clinical presentation of PCALCL can be variable, a high index of suspicion is necessary in patients presenting with chronic plaques and nodules unresponsive to topical or oral medications.
原发性皮肤间变性大细胞淋巴瘤（PCALCL）是一种罕见的非霍奇金淋巴瘤，约占所有皮肤淋巴瘤的0.9%-9.0%。PCALCL的特点是无全身受累、可自发消退且复发率低，尤其是局限性病变。我们报告一名47岁女性，右臂有两个无症状的红斑性硬结斑块，病史1年半。皮肤穿刺活检显示非亲表皮性、大的、形状不规则的淋巴细胞密集浸润，核染色质增多且固缩。免疫组化显示这些非典型细胞间变性淋巴瘤激酶（ALK）阳性、CD30+、CD3-、CD20-且上皮膜抗原（EMA）阴性。临床、组织病理学和免疫组化结果符合PCALCL。检查未发现全身受累。短期CHOP（环磷酰胺、阿霉素、长春新碱和泼尼松）化疗使皮肤病变完全消退；然而，治疗12个月后复发。随后她接受了放疗并实现完全缓解。由于PCALCL的临床表现可能多变，对于出现对局部或口服药物无反应的慢性斑块和结节的患者，必须保持高度怀疑。
