Jambrosic J, From L, Assaad D A, Lipa M, Sibbald R G, Walter J B
Department of Pathology, Women's College Hospital, Toronto, Ontario, Canada.
J Am Acad Dermatol. 1987 Oct;17(4):621-31. doi: 10.1016/s0190-9622(87)70247-3.
The clinical and pathologic appearance of seven patients with lymphomatoid granulomatosis who had skin lesions when first seen is reviewed. Six patients subsequently developed systemic disease. Although the gross morphology of the skin lesions is variable, the pathology is distinctive. An adequate deep biopsy shows the characteristic lymphohistiocytic infiltrate with variable numbers of atypical cells. Angiodestruction is less evident in the skin compared to other organs. The infiltrate surrounds and invades not only vessels but also nerves and epidermal appendages. The skin biopsy specimen can be differentiated from the lymphomatous infiltrates and Wegener's granulomatosis. Two of the patients who developed systemic disease were diagnosed by skin biopsy but clinicians failed to institute therapy, preferring to wait for other organ involvement. In addition, two patients developed lymphoma, one of which was confirmed at autopsy and one on subcutaneous and bone marrow biopsy 5 years after the initial skin diagnosis. Lymphomatoid granulomatosis can be diagnosed by performing a skin biopsy. Appropriate chemotherapy may result in a high percentage of complete remissions and therefore the dermatopathologist can play an important role in the early diagnosis of this potentially fatal disease.
对7例初次就诊时即有皮肤损害的淋巴瘤样肉芽肿病患者的临床和病理表现进行了回顾。6例患者随后发展为全身性疾病。虽然皮肤损害的大体形态各异，但其病理表现具有特征性。充分的深部活检显示出特征性的淋巴组织细胞浸润，并伴有数量不等的非典型细胞。与其他器官相比，血管破坏在皮肤中不那么明显。浸润不仅围绕并侵犯血管，还侵犯神经和表皮附属器。皮肤活检标本可与淋巴瘤浸润及韦格纳肉芽肿相鉴别。2例发展为全身性疾病的患者通过皮肤活检确诊，但临床医生未进行治疗，而是选择等待其他器官受累。此外，2例患者发生了淋巴瘤，其中1例在尸检时确诊，另1例在初次皮肤诊断5年后经皮下及骨髓活检确诊。淋巴瘤样肉芽肿病可通过皮肤活检进行诊断。适当的化疗可能会使高比例的患者完全缓解，因此皮肤病理学家在这种潜在致命疾病的早期诊断中可发挥重要作用。
