Kim Sang-Tae, Jeon Young-Seung, Sim Hyung-Jun, Kim Sung-Hee, Kim Yun-Kyew, Suh Kee-Suck, Park Jeong-Hoon, Park Sung-Wook
Department of Dermatology, Kosin University College of Medicine, Busan, South Korea.
J Am Acad Dermatol. 2006 Mar;54(3):466-71. doi: 10.1016/j.jaad.2005.11.1051.
Mycosis fungoides palmaris et plantaris (MFPP), characterized by hyperkeratotic patches or plaques confined to the palms and soles, is rare and easy to misdiagnose because of the clinical similarity to psoriasis, cutaneous inflammatory dermatoses, and dermatophytic infections. The literature about MFPP mostly consists of case reports with short-term follow-up.
Our purpose was to evaluate the clinicopathologic features, T-cell receptor (TCR) gene rearrangement findings, and prognosis of MFPP.
This retrospective study has been reviewed in the clinicopathologic, TCR gamma gene rearrangement findings and follow-up study of 12 patients with MFPP.
The duration of diseases ranged from 9 months to 25 years with a mean duration of 5.3 years. Clinically, hyperkeratotic patches and plaques were observed in all cases, with 6 cases having developed on the palms and soles and 6 cases on the palms only. In TNM classifications, all cases were confined to T1N0M0 (stage IA) showing an early stage of mycosis fungoides (MF). Histopathologic findings revealed marked hyperkeratosis, parakeratosis with plasma, epidermotropism, convoluted lymphocytes, haloed lymphocytes, dense infiltrate of lymphocytes in all 12 cases (100%), Pautrier's microabscess in 9 cases (75%), a wiry bundle of collagen in 11 cases (91.7%) and basilar epidermotropism in 3 cases (25%). TCR gamma gene rearrangement was performed except for one case and monoclonality was detected in 10 of 11 cases. In the comparison group with cutaneous inflammatory dermatoses, all cases showed polyclonality. Treatment was done with Re-PUVA (acitretin and PUVA), ultraviolet A1, as well as systemic acitretin and methotrexate. Most patients showed a good response. In the follow-up study of 9 cases for a mean period of 47.6 months, only one patient's skin lesions were extended to the trunk and face, but the other patients had no sign of extracutaneous involvement.
These results were obtained from patients with MFPP in Korea. A cooperative study with other ethnic groups will be helpful.
If a patient has recalcitrant palmoplantar dermatosis, MFPP should be suspected and histopathologic studies with TCR gene rearrangement should be done for early diagnosis of MFPP.
掌跖蕈样肉芽肿（MFPP）的特征为局限于手掌和足底的角化过度斑块或斑片，较为罕见，且由于其临床表现与银屑病、皮肤炎性皮肤病及皮肤癣菌感染相似，容易误诊。关于MFPP的文献大多为短期随访的病例报告。
我们的目的是评估MFPP的临床病理特征、T细胞受体（TCR）基因重排结果及预后。
本回顾性研究对12例MFPP患者进行了临床病理、TCRγ基因重排结果及随访研究。
病程为9个月至25年，平均病程5.3年。临床上，所有病例均观察到角化过度斑块和斑片，其中6例发生于手掌和足底，6例仅发生于手掌。在TNM分类中，所有病例均局限于T1N0M0（IA期），显示为蕈样肉芽肿（MF）早期。组织病理学结果显示，所有12例（100%）均有明显角化过度、伴有浆细胞的不全角化、表皮内嗜淋巴细胞现象、脑回状淋巴细胞、晕状淋巴细胞、淋巴细胞密集浸润，9例（75%）有Pautrier微脓肿，11例（91.7%）有纤细的胶原束，3例（25%）有基底细胞表皮内嗜淋巴细胞现象。除1例患者外均进行了TCRγ基因重排，11例中有10例检测到单克隆性。在皮肤炎性皮肤病的对照组中，所有病例均显示多克隆性。采用Re-PUVA（阿维A和PUVA）、紫外线A1以及阿维A和甲氨蝶呤进行全身治疗。大多数患者反应良好。在对9例患者平均47.6个月的随访研究中，仅1例患者的皮肤病变扩展至躯干和面部，但其他患者无皮肤外受累迹象。
这些结果来自韩国的MFPP患者。与其他种族群体开展合作研究将有所帮助。
如果患者有难治性掌跖皮肤病，应怀疑MFPP，并应进行组织病理学研究及TCR基因重排以早期诊断MFPP。
