RESUMO
Langerhans cells, often referred to as the "macrophages of the skin", are dendritic cells that normally reside in the epidermis and papillary dermis. Just like macrophages, they function as antigenpresenting cells that activate naive T cells. Certain mutations such as those involving the BRAF gene can cause unopposed production of Langerhans cells, which is known as Langerhans cell histiocytosis (LCH). LCH triggers an inflammatory immune response that causes systemic manifestations such as fever and fatigue, as well as other manifestations depending on the affected organs. The pathogenesis behind LCH remains poorly understood. It is still unknown whether it is a neoplastic process or a reactive cancer-mimicking illness. Diagnosis of LCH is confirmed by biopsy, and treatment is largely dependent on the extent and severity of the disease. Common treatments include corticosteroids, excision, radiation, and chemotherapy. We present a case of a 1-year-old Saudi male with LCH.
RESUMO
Oral propranolol is commonly used as a first-line treatment for infantile hemangioma. However, its use in PHACE (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, and eye anomalies) syndrome raises concerns that it might exacerbate the patient's risk of stroke. Here, we report the case of a four-month-old premature girl with PHACE syndrome, who presented with a large hemangioma involving the left side of her face, following the V1+V2+V3 distribution, including the upper lip, left ear, and left eye. This condition was successfully treated with propranolol, and no adverse side effects were reported.