In some cases, vascular lesions occur in conjunction with other systemic diseases and there may be more than one type of vascular lesion, constituting a variety of specific syndromes. We will briefly describe several relatively common syndromes associated with hemangiomas and vascular malformations. I. PHACE syndrome is a neurocutaneous anomaly that occurs in conjunction with a facial hemangioma and one or more of the following lesions: posterior cranial fossa malformation, cerebral arteriovenous malformation, and cardiovascular anomalies, i.e., ocular lesions. the initials of the five types of lesions make up the PHACE syndrome (PHACES). The clinical manifestations are very complex, with more than 50 corresponding systemic lesions reported, such as cerebellar hypoplasia, carotid hypoplasia, ductus arteriosus, optic nerve atrophy, and small jaw malformation. Among them, the distribution area of facial hemangioma is characteristic, often in the form of patches or plaques in specific areas, such as frontotemporal region, maxillary region, temporomandibular region and frontonasal region, and this kind of hemangioma is called Segmental Hemangioma (Chinese translation for the author ), suggesting that hemangioma may be due to developmental defects in certain areas. KasabachCMerritt syndrome (KMS) is a clinical phenomenon in which thrombocytopenia occurs on the basis of a vascular tumor. This vascular tumor was long thought to be a “hemangioma,” but pathologic studies have confirmed that it is actually one of two other vascular tumors – Kaposiform endothelioma (Kaposi’s) and Haemangioendothelioma (Kaposi’s). Haemangioendothelioma (KHE) and Tufted Hemangioma (TA), and the KasabachCMerritt Phenomenon (KMP) is gradually replacing KMS. KHE is an intermediate tumor that can be locally Both KHE and TA develop at birth or in early childhood and often involve the trunk and extremities. The clinical presentation is varied, typically as dark red or purplish patches or masses, with a few incomplete spontaneous regressions. 90% of KMS are secondary to KHE, and the decrease in platelets is associated with their filtration and destruction by the tumor. Treatment is mainly aimed at controlling tumor growth and raising and maintaining platelet count. Localized lesions can be surgically excised, otherwise local compression, platelet transfusion (severe platelet reduction) and pharmacological treatment (corticosteroids, vincristine, etc.) can be used, mostly with good prognosis. Sturge-Weber syndrome (SWS) is an epidemic congenital vascular anomaly, typically manifested by facial wine discoloration and ipsilateral vascular malformations of the soft meninges and choroid. In addition to facial erythema, other possible symptoms include epilepsy, hemiplegia, mental retardation and glaucoma. The distribution of wine discoloration is generally consistent with the innervation of the trigeminal nerve, with erythema in the first branch of innervation (V1 area), i.e., the upper eyelid and frontotemporal area, having the highest incidence of SWS (about 10%). In addition to the cutaneous manifestations, more than 50% of patients with SWS will have seizures. In cases of periocular erythema, approximately 50% develop glaucoma and can present independently of neurological lesions. CT can detect the volume changes of the brain parenchyma and the degree of expansion of the choroid plexus, while MRI can show the extent of the lesion and its relationship to the surrounding tissue. Wine stains can be treated by laser, photochemistry or surgery. For cases without or with neurological or ocular symptoms, it is recommended to follow up and manage them in the appropriate specialty. Klippel-Trenaunay Syndrome (KTS) is a congenital disseminated vascular malformation, but there are also reports of familial onset. It typically presents with wine stains on the limbs, venous malformations or varicose veins, and hypertelorism of bone and soft tissue. The lymphatic component of the skin lesions is also common and is mixed with capillary malformations, forming “map-like” dark red plaques around the knee joint with follicular nodules of varying size that may thicken and break down and bleed. In children with large erythema of the limb, KTS is suspected, and the limb will gradually thicken and lengthen during growth and development. Some children are reluctant to walk or exercise because of pain during activity, resulting in knee stiffness or Achilles tendon contracture. MRI is necessary to clarify the extent and depth of the deep lesion, and there is no effective treatment for KTS. Local sclerotherapy can help relieve pain. In cases of significant joint dysfunction, surgical treatment with active functional exercise is indicated. V. Parkes-Weber syndrome (PWS) PWS has similarities to KTS in that the affected limb is erythematous and hypoplastic, but the deep lesions of PWS are not venous malformations (varicosities) but multiple arteriovenous fistulas. Ultrasound, MRI or angiography can show the lesions better. In childhood, angiograms often show dense areas of dilated arteries or patchy vessels. The lesion often accelerates when it reaches adolescence or pregnancy, or after trauma or inappropriate surgical treatment. Localized tissue may become necrotic due to “blood theft”, and in severe cases, congestive heart failure may occur. If the affected limb becomes obviously dysfunctional or repeatedly infected with ulcers, the limb is often amputated. Maffucci syndrome (MS) usually appears in infancy and is characterized by subcutaneous venous malformations, abnormal cartilage development of the limbs and multiple endogenous chondromas. The diagnosis is based on clinical manifestations. Venous malformations can be seen throughout the body, but most commonly in the distal extremities, presenting as soft blue-purple masses with occasional tenderness. Pathologically, however, venous malformations are mixed with spindle cell hemangioendothelioma. Endogenous chondromas often appear asymmetrically bilaterally, and X-rays show swollen and thinning bony cortex with oval defects and small sandy calcified spots within the venous malformation. The most serious complications of MS syndrome include multiple fractures in childhood, neurological abnormalities due to endogenous chondrosarcoma of the skull, and malignant transformation of endogenous chondrosarcoma (approximately 15% malignant transformation to chondrosarcoma). Venous malformations that are asymptomatic are usually treated nonoperatively, and chondral lesions that are amenable to surgical treatment must be biopsied to rule out malignancy. Blue Rubber Bleb Nevus Syndrome (BRBNS) is mainly manifested as multiple venous malformations of the skin and (visceral) digestive tract. Most of them are disseminated, and some of them may be autosomal dominant. The lesions are usually present at birth or in early childhood. Cutaneous venous malformations are most commonly found on the trunk and upper extremities and can be single or hundreds of them, ranging in size from a few millimeters to several centimeters and appearing purplish, blue or even black. Gastrointestinal venous malformations are easily detected by endoscopy or MRI, vary in size, are often multiple, are located in the submucosa, and can involve the small intestine to the end of the colon, without correlation to the extent of distribution of surface venous malformations. Venous malformations are also seen in other organs, such as the liver, lungs, urethra, brain and muscles. Treatment is mainly to control chronic bleeding from the gastrointestinal tract and, in case of severe bleeding, endoscopic electrocoagulation or surgical excision. Skin lesions may be treated by sclerotherapy or surgery if they affect the appearance.