The female child, was her mother's third fetus and second birth, with a gestational age of 38+2 weeks, and was delivered by cesarean section due to dystocia. Her parents were a non-consanguineous healthy couple, with no specific skin, oral, or perineal diseases. Her birth weight was 2980g, and her Apgar score was 6 (A+P+G+A+R=1+1+1+1+2) after 1 min. After wiping the fetal fat, it was found that her skin was slightly damaged, which did not attract enough attention. In the following two days, the aforementioned skin damage progressed to scattered coin-sized desquamation and yellow blisters. Among them, the two upper limbs were the weight, and several skin damages were observed together (See Figure 1). Scattered yellow blisters was not easily broken, and the blister fluid is turbid and purulent. There was no family history with the same features. The patient did not have fever, screaming, irritability, etc. Vital signs showed a blood pressure of 85/50 mmHg, a pulse rate of 120 beats/minute, a respiratory rate of 32 breaths/minute, and a body temperature of 38.2 C. Her body weight, height, and head circumference were all. Her laboratory results such as complete blood cell count of WBC (white blood cell) 28.0x109/L, mainly neutrophils, accompanied by increased eosinophils. RBC (red blood cell count) 4.08x1012/L, PLT (platelet) 433x109/L, liver panel: ALT (alanine aminotransferase) 19 U/L, AST (aspartate aminotransferase) 34 U/L, kidney panel: creatinine 47 mumol/L) were within normal. Her limits and respiratory etiology test results (including tuberculosis) were negative. No abnormalities were detected in the TORCH (T: Toxoplasma gondii; R: Rubella virus; C: Cytomegalovirus; H: Herpes simplex virus; O: Other pathogens), immunization series and autoimmune series examinations. No bacteria or fungi were detected in blister smears, cultures, or blood cultures. The blister culture was sterile. Convulsions occurred on the third day after birth. Head magnetic resonance imaging (MRI) showed multiple blood lesions in the brain (Figure 2). Combined with the patient's medical history, the diagnosis was considered IP. To further clarify and seek sufficient evidence, the patient was examined by a wide-angle digital retinal imaging system, suggesting that fundus fluorescein angiography showed fundus vascular loop-like changes, consistent with the fundus vascular manifestations of pigmented incontinence (Figure 3). In the meantime, the patient underwent skin biopsy, which showed that more eosinophils were aggregated in the epidermis, a small number of dyskeratocytes were scattered in the surrounding epidermis, and a small number of eosinophils and lymphocytes were infiltrated in the superficial dermis (Figure 4). To further clarify the genetic status of the patient's family, the parents and older sister of the patient underwent blood genetic testing. The results suggested that the patient had a heterozygous loss in exons 4-10 of the IKBKG gene, but the parents and older sister were normal (Figure 5).