Patient M., 23 years old, consulted a dermatologist with complaints of rashes on the face, which had bothered since childhood. On objective examination, skin lesions were widespread. A visual examination revealed spots of hypopigmentation, angiofibromas of the face, shagreen fate of the skin, periungual fibromas. She was diagnosed with tuberous sclerosis. Further examination revealed a neoplasm in the brain and right kidney, damage to the lungs, tubular bones, lymphadenopathy. The patient continues to be monitored by a neurologist and therapist. Based on the results of CT scan of the chest organs, an oncologist's consultation was scheduled to conduct an oncology search. Thus, with skin manifestations characteristic of this disease, it is necessary to conduct a comprehensive examination to identify concomitant pathology and early diagnosis of complications.

Purpose.To present a clinical case of Bloch Sulzberger syndrome. Material and methods. The examinations were performed to diagnose the disease: а visual examination of the skin, cytological analysis of the gallbladder fluid, general and biochemical blood tests, genetic research. Results.During a visual examination of the skin, a differential diagnosis was made with infectious dermatitis, toxic-allergic dermatitis, epidermolysis bullosa and linear IgA-dependent dermatosis in children. Crucial in the diagnosis belonged to a genetic study, after which a deletion of exons 410 of the IKBKG gene was detected, which confirmed Bloch Sulzberger syndrome. Conclusion.Newborns with vesicle-bullous rashes entering the neonatal pathology department and observed by neonatologists require a thorough examination, a mandatory consultation of a dermatologist inorder to determine further management tactics.