Lerner L H, Wiss K, Gellis S, Barnhill R
Department of Pathology, Massachusetts General Hospital, Boston 02114, USA.
J Am Acad Dermatol. 1996 Aug;35(2 Pt 2):330-3. doi: 10.1016/s0190-9622(96)90662-3.
Congenital leukemia and leukemoid reactions may be indistinguishable on clinical and histologic grounds and are highly associated with trisomy 21. This report characterizes a specific vesiculopustular skin eruption in an infant with Down syndrome and a congenital leukemoid reaction. On the first day of life an unusual vesiculopustular eruption developed, starting in areas of cutaneous trauma. A biopsy revealed immature myeloid cells in an epidermal spongiotic vesiculopustule and in a perivascular distribution, suggestive of leukemia cutis. As the peripheral blood smear normalized, the eruption cleared. Myelodysplasia subsequently developed and evolved into acute myelogenous leukemia. This is the first detailed report of a specific skin infiltrate caused by the immature cells of a leukemoid reaction. Skin infiltration by immature myeloid cells during a congenital leukemoid reaction may portend an aggressive course of the myeloproliferative disorder.
先天性白血病和类白血病反应在临床和组织学上可能难以区分，且与21三体高度相关。本报告描述了一名患有唐氏综合征和先天性类白血病反应的婴儿出现的一种特定的水疱脓疱性皮肤疹。出生第一天，一种不寻常的水疱脓疱疹开始出现，起始于皮肤创伤部位。活检显示在表皮海绵状水疱脓疱及血管周围分布中有未成熟髓样细胞，提示皮肤白血病。随着外周血涂片恢复正常，皮疹消退。随后发生骨髓发育异常并演变为急性髓系白血病。这是关于类白血病反应的未成熟细胞引起的特定皮肤浸润的首次详细报告。先天性类白血病反应期间未成熟髓样细胞的皮肤浸润可能预示着骨髓增殖性疾病的侵袭性病程。
