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Indian Journal of Dermatology, Venereology, and Leprology, Vol. 76, No. 5, September-October, 2010, pp. 592 Net letter Angel's kiss in three generations Zafer Turkoglu1, Burce Can1, Mukaddes Kavala1, Ozlem Turkoglu2 1 Department of Dermatology, Goztepe Training and Research Hospital, Istanbul, Turkey Correspondence Address: Code Number: dv10186 DOI: 10.4103/0378-6323.69098 Sir, Vascular stains are the most common capillary malformations (CMs) encountered in newborns. Similar stains called ′′salmon patch,′′ ′′angel′s kiss,′′ ′′stork bite′′ occur in up to 40-50% of newborns but fade progressively during infancy and so termed as ′′fading macular stains.′′ Angel′s kiss (AK) represents a subset of CMs that occupies a midline location of the glabellar region. Some macular stains particularly those on the nape of neck (stork bite) and rarely on the glabellar region (angel′s kiss) persist into adulthood without significant darkening and then called as medial telangiectatic nevus. Despite the high frequency CMs in the general population, only few families that show familial segregation of CMs as an autosomal dominant trait have been reported. [1],[2],[3],[4] We describe three generations including daughter, mother and grandmother with persisting AK located on the glabellar region. A 22-year-old girl (proband) presented with a vascular lesion on her forehead. She reported that she had this red discoloration without any change in color and size since birth. There was 3Χ5 cm reddish butterfly shaped macule on the central part of her forehead and glabella [Figure - 1]a. When questioned, the patient reported that in her family her mother and grandmother also had pinkish discoloration becoming paler with age on their forehead since birth. Her mother and grandmother were later examined. Her mother was 45-year-old and had a V-shaped 2Χ3 cm pinkish macule on her glabella becoming more evident during crying and after emotional reflections [Figure - 1]b. Her grandmother was 70-year-old and had a 1.5Χ3 cm paler pinkish macule on her glabellar area [Figure - 1]c. Physical examination of them were normal and family history was not supportive for other vascular malformations and internal disorders. Radiological examinations including cranial computerized tomography and abdomen ultrasonography for our patient and her mother were normal. Medial telangiectatic nevus (MTN) showed an autosomal dominant mode of transmission in three generations of family [Figure - 2]. CMs presenting at birth and persisting in adulthood are of two types: (1) MTN, which is the most common form and (2) lateral telangiectatic nevus (LTN), so-called port wine stain or nevus flammeus. CMs of birth appears in 50-74% of all newborn infants as irregular patches ranges salmon pink to red in color that is located primarily on the medial part of glabello-frontal( angel′s kiss), and occipito-nuchal regions of head (stork bite) or lumbosacral areas (salmon patch). [5],[6] They are sometimes termed "fading macular stains" rather than true CMs because these lesions typically lighten significantly or disappear with in the first few years. If the capillary lesions of AK or the others do not involute during the first 5 years of life, they are called persistent MTN or if the lesions become much lighter and smaller, in time they are called as residual MTN. In the literature MTN is detected in 40-55% of normal adults particularly those on the nape of the neck. [1],[2],[3],[4] Van Baar et al.[7] supposed that 57.5% of stork bite at the nape persist throught life. However the macular stains of AK persisting in adulthood as MTN have been reported rarely in the literature. MTN are associated with other abnormalities such as umbilical hernias and hyperextensions of the joints as well odontodysplasia, multiple hamartomas and intracranial malformations, meningoencephalocele, meningomyelocele, spinal dysraphism, trisomy 13-15, Beckwith-Wiedemann syndrome, Down syndrome, mega cisterna magna, and craniofacial synostosis. [1],[3],[4] There was no clinical sign of these malformations and diseases in our patient and her family. Histologically CMs are composed of capillary-like vessels that are dilated and/or increased in numbers. They have been suggested to be remnants of unmodified primitive capillary plexus. In contrast, neuronal marking is significantly decreased, suggesting that the lack of innervation may be the dilatation of the cutaneous capillaries. Linkage analysis recently led to the identification of a locus on 5q 13-22. [1],[2],[3],[4] References
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