Shaffer Matthew P, Walling Hobart W, Stone Mary Seabury
Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA.
J Am Acad Dermatol. 2005 Aug;53(2 Suppl 1):S143-6. doi: 10.1016/j.jaad.2005.01.015.
Blueberry muffin baby is a descriptive term for purpuric lesions reflective of extramedullary hematopoeisis. The clinical lesions most commonly result from intrauterine infections, such as rubella and cytomegalovirus, and less commonly with malignancy and hematologic disorders. Langerhans cell histiocytosis is a clonal proliferation of dendritic histiocytes in the skin. This has very rarely been associated with a blueberry muffin presentation. We report the case of a newborn with typical lesions of cutaneous hematopoiesis and lytic bone lesions related to Langerhans cell histiocytosis. At birth, approximately 40 2 mm to 5 mm purpuric, nonblanching macules were scattered on the trunk, extremities, and soles of our patient. Laboratory studies were unremarkable and cultures were negative. Skin biopsy showed a dermal proliferation of histiocytes staining positive for S100 and Cd1a. Pediatric bone surveys, chest radiographs, and computed tomography scans of the head were normal. Six months later, the skin lesions had resolved, but radiographs revealed lytic bone lesions of the right tibia, right ilium, and left pubic ramus, consistent with skeletal Langerhans cell histiocytosis.
蓝莓松饼样婴儿是一个描述性术语，用于指反映髓外造血的紫癜性损害。临床损害最常见于宫内感染，如风疹和巨细胞病毒感染，较少见于恶性肿瘤和血液系统疾病。朗格汉斯细胞组织细胞增多症是皮肤中树突状组织细胞的克隆性增殖。这与蓝莓松饼样表现的关联非常罕见。我们报告一例新生儿，有与朗格汉斯细胞组织细胞增多症相关的典型皮肤造血损害和溶骨性骨损害。出生时，我们的患者躯干、四肢和足底散在约40个2毫米至5毫米的紫癜性、不褪色斑疹。实验室检查无异常，培养结果为阴性。皮肤活检显示组织细胞在真皮层增殖，S100和Cd1a染色呈阳性。小儿骨骼检查、胸部X线片和头部计算机断层扫描均正常。6个月后，皮肤损害消退，但X线片显示右胫骨、右髂骨和左耻骨支有溶骨性骨损害，符合骨骼朗格汉斯细胞组织细胞增多症。
