Infantile Digital Fibromatosis (Inclusion Body Fibromatosis) Observed in a Baby Without Finger Involvement
A 9-day-old male baby was hospitalized after his birth due to some swells under the skin. There was no specific event in his prenatal history. According to the information obtained, the patient was born by spontaneous vaginal birth at home as the third child of a 26-year-old mother during her 38th week of pregnancy. According to the information gathered from the family, it was determined that the mother and the father of the patient were second-degree parents (their parents are cousins). In physical examination, the general condition of the patient was good, and he presented open awareness. His spontaneous movements were normal. His suction, searching, catching and moro reflexes were operational. The weight of the patient was 2950 gr, the height was 49 cm, and the head circumference was 34 cm. The frontal fontanel was 2 × 2 and at a normal cambering level. The hard consistency nodules observed under the skin all over the body of the patient were of different sizes, and presented lesions, among which the largest was 1 × 1 cm []. There were no lesions on the fingers.\nIn the laboratory examinations results, the hemoglobin value was 19.8 g/dL, the erythrocyte count was 5.98 × 106/mm3, the leukocyte count was 12,120/mm3, and the thrombocyte count was 321,000/mm3. The cholesterol and triglyceride levels were normal. Liver functional tests, renal functional tests, and thyroid hormones tests were between normal ranges. The C-reactive protein was 26 mg/dL, and the sedimentation was 2 mm/h. The brain magnetic resonance imaging (MRI), thorax tomography, bone survey, abdominal ultrasonography (USG), and urinary USG were normal. By superficial USG, multiple isoechoic hypoechoic lesions were observed among the muscle plan. The largest lesion was 13 × 10 mm. In thoracolumbar MRI, multiple massif lesions retaining peripheral contrast (the biggest was 1.7 × 1.4 cm large) has been observed under the skin muscle plans in thoracolumbar regions, between the extremities muscles, in the regions of section, at hyperintense and hypointense intravenous contrast substance injection at T2-weighted image and T1-weighted image [].\nHistopathology included varying amounts of extracellular collagen and uniform spindle cells. Cell bundles were spread into adjacent tissues. Lesional cells had central elongated nuclei and vaguely fibrillar cytoplasm. The diagnostic feature was the presence of intracytoplasmic, eosinophilic spherical inclusions []. The lesional cells were not nuclear atypia and mitoses. In histochemical staining, these inclusions stain the brightly purple-red rounded with mason-trichrome staining. No staining with PAS was observed. As a result of the immunohistochemical staining of the lesion biopsy, smooth muscle actin was positive; desmin, S100, and CD34 were negative. These findings were diagnosed via IDF. The patient was followed and was discharged assuming his participation to controls.