Nevus Flammeus Research Articles

Ultrastructure and organization of the cutaneous microvasculature in normal and pathologic states.

The cutaneous microvasculature is organized into upper and lower horizontal plexuses with the dermal capillary loops arising from the upper plexus. The arteriolar and venular sides of the microvasculature can be identified by the ultrastructure of the mural basement membrane material. Collecting venules present in the lower dermis contain valves. Periadventitial cells (veil cells) are present around all microvessels. Their size and number appear to correlate with the quantity of mural basement membrane material found in cutaneous vessels in diabetes, actinic damage, and chronological aging. The contractile cells of the vascular wall surround the endothelial cell tube in a manner suggesting specific functions. The smooth muscle cells in the arteriolar segment form a sleeve, whereas each pericyte in the postcapillary venular simultaneously makes many contacts with several underlying endothelial cells. The common telangiectases can be explained by abnormalities in this organization and ultrastructure rather than by neovascularization or random anastomoses. The macular telangiectases seen in scleroderma, generalized essential telangiectasia, and nevus flammeus are produced by dilatation of the postcapillary venules of the upper horizontal plexus. Cherry angiomas are produced by spherical and tubular dilatations of capillary loops in dermal papillae with tortuous cross-connections between individual loops. Angiokeratomas of Fabry and Fordyce have the ultrastructure of collecting venules that contain valves, and appear to represent the ectopic development or placement of small valve-containing collecting veins. The cutaneous lesions of hereditary hemorrhagic telangiectasia represent arteriovenous communications.

Cutis marmorata telangiectatica congenita: Report of 22 cases

Twenty-two cases of cutis marmorata telangiectatica congenita were evaluated during an 8-year period. All but two patients were examined in the first year of life; 14 of the 22 (64%) were female infants. Four patients had focal cutaneous atrophy associated with the reticulated vascular pattern, and eight had ulcerations of involved skin. Six (27%) had additional anomalies. Of these, three patients had a nevus flammeus, and one had congenital generalized fibromatosis and hemiatrophy. Two of the infants had glaucoma; one also had a facial nevus flammeus and the other had cutis marmorata telangiectatica congenita of the face. A congenital pigmented nevus and a localized venous malformation constituted the remaining associated defects. This disease is an uncommon cutaneous vascular anomaly that is most often solitary but occasionally may be associated with other developmental defects.

Klippel Trenaunay Weber Syndrome with Spinal Arteriovenous Malformation

A 28-year-old female with Klippel-Trenaunay-Weber syndrome associated with an arteriovenous malformation (AVM) of the spinal cord is reported. She was admitted to our hospital with a 4-month history of steadily progressive weakness and dysesthesia of the legs. A nevus flammeus, varices, hypertrophy and elongation of the left leg had been present since her infancy. These symptoms progressed and she became unable to walk and pass water by herself. Myelography disclosed a spinal AVM extending from Th11 to L2. Angiography confirmed the presence of stretched and tortuous vessels as well as an arteriovenous shunt in the left leg. Magnetic resonance imaging showed a high-intensity area extending from Th10 to L2. Following laminectomy from Th10 to L2 and resection of the AVM, her motor and bladder functions worsened. However, 6 months later, her motor function improved to the preoperative state and the bladder dysfunction disappeared. The coexistence of Klippel-Trenaunay-Weber syndrome with spinal AVM, considered to be rare, is discussed, and the pertinent literature is reviewed. The case presented here is the first to have been surgically treated in Japan.

Nuchal nevus flammeus as a skin marker of prognosis in alopecia areata.

In this work, the incidence of nuchal nevus flammeus was studied in 205 patients suffering from various forms of alopecia areata, as well as in a group of 555 volunteers without alopecia areata examined in our outpatient clinic. The incidence of nuchal nevus flammeus in the totalis-universalis form of alopecia areata was 58.2% (examined patients, n = 79), in ophiasis-extensive forms 22.8% (examined patients, n = 70) and in simple forms of alopecia areata 3.6% (examined patients, n = 56). In the group of 555 volunteers without alopecia areata the incidence of nuchal nevus flammeus was 4.5%. Our results show that nuchal nevus flammeus could be a valuable skin marker indicating a more severe course of alopecia areata.

The Sturge-Weber syndrome.

The Sturge-Weber syndrome is characterized by angiomas of the leptomeninges overlying the cerebral cortex in association with a facial nevus flammeus. Although frequently included with other neurocutaneous genodermatoses, the syndrome is almost always sporadic in occurrence. The most common associated neurologic abnormality is seizures, which are controlled in more than 50% of patients by the administration of anticonvulsants. Laser therapy is the most promising therapeutic option for cosmetic management of the facial nevus flammeus.

(10) Naevus flammeus and naevus anaemicus

Journal Article (10) Naevus flammeus and naevus anaemicus Get access S.W. Lanigan, S.W. Lanigan The General Infirmary at Leeds Search for other works by this author on: Oxford Academic Google Scholar J.A. Cotterill J.A. Cotterill The General Infirmary at Leeds Search for other works by this author on: Oxford Academic Google Scholar British Journal of Dermatology, Volume 117, Issue s32, 1 June 1987, Pages 58–59, https://doi.org/10.1111/j.1365-2133.1987.tb12051.x Published: 01 June 1987

AMYOPLASIA: AUTOSOMAL RECESSIVE INHERITANCE IN TWO FAMILIES

Amyoplasia has been previously described as a sporadic congenital form of skeletal muscle dysplasia resulting in arthrogryposis. We presently follow two families in which amyoplasia is not sporadic but is probably inherited as an autosomal recessive trait. In the first family, a 31 year old gravida 5 parity 4 Hispanic mother delivered a female child via cesarean delivery at term. Salient features included arthrogryposis, decreased muscle bulk, decreased movements, bilaterally dislocated hips, moderate hirsutism, nevus flammeus of the forehead and a high arched palate. No central or peripheral nervous system abnormalities were demonstrable and muscle biopsy confirmed the diagnosis of amyoplasia. Family history revealed that the parents were first cousins and a sibling of the current patient died at four months of age with similar features. The sibling also failed to demonstrate any central or peripheral nervous system abnormalities and, on review, had a muscle biopsy consistent with the diagnosis of amyoplasia. The second family was referred for counseling after a four year old daughter with decreased muscle mass, arthrogryposis, minimal spontaneous movements, small mouth, long tapered fingers and toes and a nevus flammeus of the forehead was diagnosed as having amyoplasia. Family history revealed that the Hispanic parents were first cousins, once removed. Conclusion: Amyoplasia is a syndrome of heterogenous etiologies and, in ser lected populations, may be inherited as an autosomal recessive trait.

Wiedemann-Beckwith syndrome: presentation of clinical and cytogenetic data on 22 new cases and review of the literature.

The main features of Wiedemann-Beckwith syndrome (WBS) include macroglossia, abdominal wall defects, visceromegaly, gigantism, hypoglycemia, ear creases, nevus flammeus, and mid-face hypoplasia. Twenty-two cases of WBS were examined clinically and cytogenetically, and compared to 226 previously reported cases. Aspects of the clinical evaluations are discussed. All individuals examined were chromosomally normal with no evidence of 11p abnormality as has been reported recently. The relevance of a possible relationship between clinical findings, chromosome abnormalities, and genes present on 11p is discussed. Transmission of this condition is most consistent with autosomal dominant inheritance with incomplete penetrance.

Squamous Cell Carcinoma in Hemangioma of Lip

A deeply invasive squamous cell carcinoma of the right vermilion border of the lip superposed on a nevus flammeus (port-wine stain) was excised by the Mohs micrographic surgery, fixed-tissue technique. The course was uneventful and the final cosmesis was good.

Unilateral dermatomal superficial telangiectasia

Summary The appearance of telangiectatic vessels in a ‘naevoid’ distribution is usually associated with the new-born period as in the naevus flammeus. Their development later in life has been described by several authors and emphasized by Wilkin (1977). We describe three further cases and discuss the clinical relevance of the lesions.

Phakomatosis Pigmentovascularis Type IVa

Phakomatosis pigmentovascularis was first reported in 1947. We describe a 1-year-old Japanese girl who, since birth, has had three nevoid skin disorders: nevus flammeus, nevus spilus, and aberrant mongolian spots. No systemic disease of any kind has been present. We believe that this phakomatosis, which we have named phakomatosis pigmentovascularis type IVa, does not reflect the merely coincidental coexistence of three kinds of nevi.

The association of extracranial and intracranial vascular malformations in children.

This review is an attempt to relate the types of angiomas and other cutaneous vascular lesions appearing in the face with abnormalities in the formation of cerebral vessels including arteries at their origin, veins and venous sinuses, and with other less constant alterations that affect other organs, particularly the heart and cerebellum. After angiographic study of a series of patients exhibiting diverse types of vascular nevi (capillary and cavernous hemangiomas, nevus flammeus, and lymphangiomas), a correlation was established for each type of dermal nevus with a corresponding group of intracranial vascular anomalies. An explanation of these malformations based on the embryological development of the principal cerebral vessels during the 7th and 8th weeks of gestation is offered. Involvement of the facial and scalp dermatome innervated by the first sensory trigeminal branch correlates most consistently with the presence of abnormal cerebral vessels. The capillary hemangioma occurs preferentially in girls and frequently is associated with developmental anomalies of major cerebral arteries characterised by the absence of some adult vessels and the persistence of others that should have disappeared during embryogenesis such as the fetal trigeminal artery. The complete angiographic exploration of the cerebral vessels from their origin in the aortic arch to the phase of intracranial venous evacuation may reveal unexpected important findings, and is recommended for children with major facial vascular nevi, even if they are neurologically asymptomatic.

Ultrastructure and Three-Dimensional Reconstruction of Several Macular and Papular Telangiectases

Eight types of telangiectases were studied by light and electron microscopy and by 3-dimensional reconstruction from photomicrographs. Five were macular: mat telangiectasia of scleroderma, generalized essential telangiectasia, nevus flammeus, and 2 macular types not previously described. Three were papular: cherry angioma, angiokeratoma (Fabry), and angiokeratoma (Fordyce). The macular telangiectases were produced by dilatation of postcapillary venules of the upper horizontal plexus. There was no evidence of neovascularization or vascular malformation. The walls of the dilated venules were thickened by the peripheral deposition of basement membrane-like material admixed with reticulin fibers. The ultrastructure and configuration of the papular telangiectases were different. The cherry angioma was produced by spherical and tubular dilatations of capillary loops in dermal papillae. Each abnormally dilated loop was connected to the neighboring loop or loops by tortuous vascular channels. The vessels in the upper horizontal plexus were not involved. Ultrastructurally, the cherry angiomas were composed of both venous capillaries and postcapillary venules whose walls were thickened in a manner identical to that observed in the macular telangiectases. The angiokeratomas of Fabry and Fordyce were also produced by vascular abnormalities predominantly involving the dermal papillae. Ultrastructurally these vessels were similar to the small collecting veins which are normally found at the dermal-subcutaneous interface. Thus, the papular telangiectases also arose by alterations of the existing microvasculature rather than by proliferation of new vessels with random anastomoses. Reconstruction of the upper horizontal plexus from normal skin showed an undulating network of arterioles and their accompanying postcapillary venules. A 3-layered plexus arranged as venules, arterioles, and venules was not found.

The Sturge-Weber Syndrome

The Sturge-Weber syndrome is an acquired disorder in which vascular malformations are found in the skin, meninges, and eyes.¹Patients usually have a unilateral nevus flammeus, ipsilateral meningeal angiomatosis, cerebral calcification, and epilepsy. Less frequently, mental retardation, hemiplegia, and congenital glaucoma are found.^1-3A patient who has this syndrome, but with some unusual features, is described. <h3>Report of a Case</h3> The patient, a 53-year-old man, was born with extensive cutaneous vascular nevi. Initially, the nevi were macular, but, with time, cavernous changes developed in some areas. The left eye was involved and was blind from birth. Focal epilepsy commenced in early childhood but was controlled with anticonvulsants. Atrophy and weakness of the right side of the body became apparent as the patient grew older. Despite these handicaps, he was able to lead a fairly normal life. He did not have any children. There was no family history of

Late results following treatment of nevus flammeus and facial burns with split-thickness skin grafts, with special reference to postoperative color changes

Late results following treatment of nevus flammeus and facial burns with split-thickness skin grafts, with special reference to postoperative color changes

Orbitofacial Angiomatosis

Two patients with orbital vascular malformation ipsilateral facial nevus flammeus were studied. The literature contains one similar case that interestingly shares with our cases the lack of leptomeningeal angiomatosis. Orbitofacial angiomatosis seems to be a distinct entity from meningofacial angiomatosis because of its true vascular malformation of the orbit.

Endodontic Treatment Involving Intraoral Nevus Flammeus

<h2>Summary</h2> A maxillary central incisor with a necrotic pulp and acute periapical periodontitis was treated. The tooth was located in angiomatous tissue, which may have caused hemorrhagic and restorative problems. The endodontic treatment was completed without any complications. For patients with nevus flammeus of the gingiva who have nonreversible pulpal pathosis, endodontic treatment is an excellent treatment selection.

Late results following treatment of naevus flammeus and facial burns with split thickness skin grafts, with special reference to post-operative colour changes

Two cases of extensive split thickness skin transplant to the face are presented. It was noted that the usual brownish pigmentation of grafted skin did not occur in these cases. Continued exposure in the open or to sunlight during work has been implicated as a causative factor.

Adrenal Calcification in Beckwith-Wiedemann Syndrome

The features of the Beckwith-Wiedemann syndrome are characterized by exomphalos, macroglossia, macrosomia, visceromegaly, ear lobe anomaly, nevus flammeus, hemihypertrophy, and other abnormalities. Adrenal calcifications can be seen in a variety of pediatric conditions. More than 171 children with the Beckwith-Wiedemann syndrome have been described in the pediatric literature, but none of these patients have been reported as having adrenal calcification.1The present communication describes two girls with the Beckwith-Wiedemann syndrome who were demonstrated to have adrenal calcifications at ages 7 and 4 years, respectively. Report of Cases.—Case1.—This 7-day-old girl was hospitalized because of macrosomia (4.8 kg), macroglossia, and a history of poor feedings. She had been born at term, following a traction delivery. On admission macrosomia, macroglossia, peculiar facies, relative microcephaly, navel hernia, and visceromegaly were striking. Serum bilirubin level on the seventh day of life was 18.8 mg/100 ml. A diagnosis of cretinism was suspected

Dr. Jacobs Replies

Drs. Gorman and Snead bring up a purely semantic question: "What is the Sturge-Weber syndrome?" According to McKusick,1 Sturge-Weber syndrome, sometimes called the fourth phacomatosis, is characterized by nevus flammeus of the face and angioma of the meninges. Warkany2 states that it "consists of a facial port wine nevus, convulsions and other neurological manifestations." In fact, the latter author, in discussing reported cases, rules out as Sturge-Weber syndrome a case with the CNS changes but no skin involvement.