Download A Case Report of Unusual Location of Medial Telangiectatic Nevus

JCBPS; Section B; August 2016 – October 2016, Vol. 6, No. 4; 1193-1196,
E- ISSN: 2249 –1929
Journal of Chemical, Biological and Physical Sciences
An International Peer Review E-3 Journal of Sciences
Available online at www.jcbsc.org
Section B: Biological Sciences
CODEN (USA): JCBPAT
Research Article
A Case Report of Unusual Location of Medial
Telangiectatic Nevus
Ayesha Sameera1, E. Sridhar Reddy1, Jesudass Govada2, Rajesh Kumar Patel3,
A. Sudarshan Kumar4
1
2.
. MNR Dental College & Hospital, Sangareddy , Telangana,India.
Rajiv Gandhi Institute of Medical Sciences, Kadapa , Andhra Pradesh, India.
3
. Sandor Animal Biogenics Pvt Ltd, Hyderabad, Telangana, India.
4
. GSL Dental College and Hospital, Rajahmundry, Andhra Pradesh, India.
Received: 22 August 2016; Revised: 30 August 2016; Accepted: 03 September 2016
Abstract: Medial telangiectatic nevus (MTN) or Angel’s kiss is a vascular stain
located on the midline body most commonly on the glabellar region. It is an
autosomal dominant inheritance with only 0.3 % to 1 % prevalence of new born. In a
present case, a 21 year old male having pink stains on the forehead and also on
philtrum of lip which is a rare location for medial telangiectatic nevus. The patient
exhibited pale pink stains on glabellar region close to right eyebrow and on the
philtrum near the right ala of the nose of size 2 cm sand roughly triangular in shape.
The vascular stains are present since birth which have been slightly increased in size
with growing age and color has become paler pink. Family and medical history is
non-contributory. There was no associated pain and compared to adjacent skin no
undue abnormality was present. There was no visible pulsation. On radiographic
examination, posteroanterior (PA) view did not show any important alterations. No
molecular genetics was performed to know whether the condition is associated with
some mutations in the genes responsible for MTN. The article presents a case report
of MTN in an adult patient.
Key words: Vascular stain, Autosomal dominant, Telangiectatic Nevus, Angel’s kiss.
INTRODUCTION
A Telangiectatic nevus a common skin condition of neonates, characterized by flat, deep-pink
localized areas of capillary dilation that occurs predominantly on the back of the neck, lower occiput,
1193
J. Chem. Bio. Phy. Sci. Sec. B, August 2016 – October 2016; Vol.6, No.4; 1193-1196
A case …
Ayesha Sameera et al.
upper eyelids, upper lip, and bridge of the nose. The areas disappear permanently by about 2 years
of age. Vascular stains (telangiectatic naevi or nevi) are malformed dilated blood vessels in the skin
(vascular or capillary malformations). Previously referred to as vascular birthmarks, vascular
anomalies are now classified based on a system developed1 and classified them as vascular tumors
and vascular malformations. Vascular malformations are irregular vascular networks defined by their
particular blood vessel type. Common names for these birthmarks include angel kisses and stork bites.
In most cases, no treatment is necessary since the overwhelming majority disappear with no
treatment, if do not, most are in a location that is covered by hair. Telangiectatic nevi are not a threat
to overall health but rarely affect appearance. Capillary malformation is made to differentiate different
types of stains which are classified as Telangiectatic naevi (Nevus flammeus, Nevus roseus, and Cutis
marmorata telangiectatica congenita) and Nevus anemicus. However, vascular lesions that do not
represent naevi are Salmon patch (stork-bite, angel’s kiss) and Telangiectases of Rendu-Osler disease.
Lateral lesions, commonly referred to as port-wine stains, have a more protracted course2. Capillary
malformations that are present in birth and continue in adulthood are of two types; Medial
telangiectatic nevus (MTN) and lateral telangiectatic nevus (LTN). These are seen in 0.3% to 1% 3 of
the new born. Capillary vascular malformations are now known due to genetic abnormalities.
Investigators have found a mutation in the GNAQ gene in the vast majority of patients with port-wine
stains and Surge-Weber syndrome4. Port-wine stains were shown to be caused by mutation
(c.548G→A) mutation in the GNAQ gene. An association with RASA1 has also been described5.
CASE REPORT
A 21 year old male patient reported with complaint of dental problem to one of the Dental College &
Hospitals in Andhra Pradesh. It was observed that the patient had pink discoloration on his forehead
and above the philtrum of lip. He gave a history that these stain where present since birth and they
have further slightly increased in size since birth and color has become pale pink. Family and medical
history was non-contributory. On physical examination pale pink stains where noticed on glabellar
region close to right eyebrow and on the philtrum near the right ala of the nose of size 2 cm sand
roughly triangular in shape (fig. 1 & 2). There was no associated pain and compared to adjacent skin
no undue abnormality was present. There was no visible pulsation. On radiographic examination,
posteroanterior (PA) view did not show any important alterations (fig.3). However there was slight
rise in temperature and on auscultation bruits were felt. No further investigation like biopsy or CT or
MRI was done. Based on these findings the patient was diagnosed as Angle’s Kiss (AK).
Fig. 1: Angel’s kiss on glabella close to eyebrow
1194
Fig.2: Angel’s kiss on philtrum near all of nose.
J. Chem. Bio. Phy. Sci. Sec. B, August 2016 – October 2016; Vol.6, No.4; 1193-1196
A case …
Ayesha Sameera et al.
Fig.3: PA –No contributory findings in x-ray of posteroanterior (PA) view
DISCUSSION
Pathogenesis of isolated capillary malformations (CM) is unknown. A genome wide linkage analysis
has identified a locus on chromosome 5q associated with familial disease5. A rare autosomal dominant
inherited disease consisting of a combination of CM and arteriovenous malformations (AVM) is
associated with a loss-of-function mutation in RASA1 gene6. This gene provides instructions for
making a protein known as p120-RasGAP, which is involved in transmitting chemical signals from
outside the cell to the nucleus. The role of the p120-RasGAP protein is not fully understood, although
it appears to be essential for the normal development of the vascular system. This has spurred further
research into the cause of the more common sporadic form of CM. Angel’s kiss is generally
autosomal dominant with linkage on 5q 13-227, but in this case report no familial history was present
and also this is a unique case where in the AK is present on the philtrum which is not the usual site.
However, earlier report indicates six families in which a few members, in three generations, were
affected with medial telangiectatic nevus (salmon patch, stork bite, angel's kiss) on the forehead,
glabella, upper eyelids, upper lip, nose, and nuchal and occipital areas are presented8.
MTN that continue beyond the first five years of life are called persistent MTN and those that become
smaller and lighter are called residual MTN9. MTN that persist are rare and also accounts for the
uniqueness of this case. MTN are also associated with multiple hamartomas, intracranial
malformations, Beckwith-Wiedemann syndrome and craniofacial synostosis. Histological these are
composed of dilated or increased capillary like vessels. Diagnosis is usually made by physical
examination alone. Cases where the findings are inconsistent, for example, pain or spontaneous
bleeding, an MRI may be performed. Treatment of vascular anomalies is usually complex and
involves multiple disciplines and therapeutic options. AK usually does not possess any problem
except when unsightly and hampers the esthetics then treatment modality becomes prime important.
In our case AK does not require any treatment as it is well accepted by the patient.
1195
J. Chem. Bio. Phy. Sci. Sec. B, August 2016 – October 2016; Vol.6, No.4; 1193-1196
A case …
Ayesha Sameera et al.
REFERENCES
1. J.B. Mulliken and J. Glowacki, Hemangiomas and vascular malformations in infants and
children: a classification based on endothelial characteristics. Plastic and Reconstructive
Surgery, 1982, 69(3), 412–422.
2. R. Happle, What is a capillary malformation. J Am Acad Dermatol., 2008, 59, 1077-1079.
3. A.H. Hartley and J.E. Rasmussen J E, Hemangiomas and spitz nevi. Pediatr. Rev. 1990, 11,
262-267.
4. K.A. Pasky, Familial multiple lateral telangiectatic nevi (port wine stain or nevi flammei).
Clinical Genetics, 1992, 41, 197-201.
5. I. Eerola, L.M. Boon, S. Watanabe, H. Grynberg, J.B. Mulliken and M. Vikkula, Locus for
susceptibility for familial capillary malformation (“port-wine stain”) maps to 5q, European
Journal of Human Genetics, 2002,10 (6), 375–380.
6. L.M. Boon, J.B. Mulliken and M. Vikkula, RASA1: variable phenotype with capillary and
arteriovenous malformations. Current Opinion in Genetics and Development, 2005, 15 (3),
265–269.
7. K.M. Cordoro, L.S. Speetzen, M.A. Koerper, I.J. Frieden, Physiologic changes in vascular
birthmarks during early infancy: Mechanism and clinical implications, J Am Acad
Dermatol., 2009, 60,669-675.
8. K.A. Pasyk, S.R. Wlodarczyk, M.M. Jakobczak, M. Kurek, D.J. Aughton, Familial medial
telangiectatic nevus: variant of nevus flammeus-port-wine stain. Plast Reconstr Surg., 1993,
91(6), 1032-1041.
9. M. C. Garzon, J.T. Huang, O. Enjolras, I.J. Frieden, Vascular malformations. J Am Acad
Dermatol., 2007, 56, 353-370.
Corresponding author: Rajesh Kumar Patel,
Sandor Animal Biogenics Pvt Ltd, Hyderabad, Telangana, India.
1196
J. Chem. Bio. Phy. Sci. Sec. B, August 2016 – October 2016; Vol.6, No.4; 1193-1196