Kaya Avni, Yuca Sevil Ari, Karaman Kamuran, Erten Remzi, Doğan Murat, Bektas Mehmet Selcuk, Ustyol Lokman
Department of Pediatrics, Yüzüncü Yil University, Van, Turkey.
Indian J Dermatol. 2013 Mar;58(2):160. doi: 10.4103/0019-5154.108085.
A 9-day-old male baby was hospitalized after his birth due to some swells under the skin. The hard consistency nodules observed under the skin all over the body of the patient were of different size, and presented lesions, among which the biggest was 1 × 1 cm. No lesions were observed on the fingers. By superficial ultrasonography, multiple isoechoic hypoechoic lesions were observed among the muscle plan. In thoracolumbar magnetic resonance imaging, multiple massif lesions retaining peripheral contrast (the biggest was 1.7 × 1.4 cm large) had been observed under the skin muscle plans, between the muscles of the extremities. The biopsy was positive for smooth muscle actin, but negative for desmin, S100, and CD34. These findings were diagnosed as infantile digital fibromatosis (IDF) (inclusion body fibromatosis). The case was presented with an objective to illustrate and remind that IDF can be observed in babies without finger involvement.
一名9天大的男婴出生后因皮肤下出现一些肿胀而住院。在患者全身皮肤下观察到质地坚硬的结节,大小不一,并呈现病变,其中最大的为1×1厘米。手指上未观察到病变。通过浅表超声检查,在肌肉层中观察到多个等回声低回声病变。在胸腰椎磁共振成像中,在皮肤肌肉层下、四肢肌肉之间观察到多个保留周边对比剂的块状病变(最大为1.7×1.4厘米)。活检结果显示平滑肌肌动蛋白呈阳性,但结蛋白、S100和CD34呈阴性。这些发现被诊断为婴儿指纤维瘤病(IDF)(包涵体纤维瘤病)。本病例旨在说明并提醒,IDF可在无手指受累的婴儿中观察到。