Physicians Academy
Established in December 2006 by the Kashmir Academy Of Private Physicians
Table Of Contents
Editorial :
 Bacterial Infections in Patients with Leukemia
Article 1 :
 What after a Suicide Attempt?
Article 2 :
 Neglected Child with severe Bronchopneumonia
Picture of The Month :
 Picture of The Month November 2017
Drug Update :
 VELCADE (Bortezomib)
Abstracts from Other Journals :
 Abstracts from Other Journals-November 2017

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Sep 2017 ( Volume -11 Number -9)

Picture of The Month
Picture of The Month September 2017
Qazi Ashraf, MS; DNB (Surgical oncology); MNAMS; MESSO; FSSO
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SEPTEMBER 2017

Picture of The Month

Qazi Ashraf, MS; DNB (Surgical oncology); MNAMS; MESSO; FSSO 

 

A 50 years old male had this swelling on his face growing slowly over a period of 5 months duration. He had been taking antibiotics and local medications but without any improvement till the whole thing eroded and was fungating. What is the diagnosis?

Send your answer to us on Rapid Response.

Author Information: Dr. Qazi Ashraf, MD; MS; DNB (MCh); MNAMS; MESSO; FSSO(USA) is Senior Consultant, Surgical Oncology in Noora Hospital, Srinagar, Kashmir, India. Email: aashob@gmail.com

 

Answer of last month’s POTM August 2017

This teenager visited my clinic for pyrexia of a week’s duration which turned out to be Enteric fever. However his face revealed this. What is the diagnosis and further workup?

The correct diagnosis is:

Port Wine Stain (Nevus Flammeus)

A port-wine stain (nevus flammeus), is a discoloration of the human skin caused by a vascular anomaly. They are so named for their coloration, which is similar in color to port wine, a fortified red wine from Portugal. Studies have recorded an incidence of about 3–5 cases per 1,000 newborn babies. This is almost always a birthmark; in rare cases it can develop in early childhood. These stains ordinarily persist throughout life. The area of skin affected grows in proportion to general growth.

Port-wine stains occur most often on the face but can appear anywhere on the body, particularly on the neck and upper trunk. Early stains are usually flat and pink in appearance. As the child matures, the color may deepen to a dark red or purplish color. In adulthood, thickening of the lesion or the development of small lumps may occur.

Port-wine stains may be part of a syndrome such as Sturge-Weber syndrome or Klippel-Trenaunay-Weber syndrome. Sturge–Weber syndrome, also called encephalotrigeminal angiomatosis, is rare (1 in 50,000 newborns) and is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation and ipsilateral leptomeningeal angioma (cerebral malformations and tumors).

Port-wine stains were shown to be caused by a somatic activating c.548GA mutation in the GNAQ gene. An association with RASA1 has also been described.

Depending on the location of the birthmark and other associated symptoms, a physician may choose to order a measurement of intraocular pressure or X-ray of the skull. A MRI scan of the brain may be performed (under anesthesia) on infants who have a port-wine stain in the head area in order to check for signs of Sturge-Weber syndrome. Treatment includes surgery, radiation, Laser and tattooing and anticonvulsants in case of seizures.

References:

1. Faurschou, A.; Olesen, AB; Leonardi-Bee, J.; Haedersdal, M. Lasers or light sources for treating port-wine stains. 9 November 2011. Cochrane database of systematic reviews (Online) (11): CD007152. PMID 22071834 doi:10.1002/14651858.CD007152.pub2

2. Ryan E, Warren L. Birthmarks--identification and management. Australian Family Physician. May 2012. 41(5):274–277. PMID 22558616

3. James W, Berger T, Elston D. Andrews' Diseases of the Skin: Clinical Dermatology. 10th ed. 2005. Saunders. Page 582–3. ISBN 0-7216-2921-0

4. Shirley MD, Tang Hao, Gallione CJ, et al. Sturge–Weber Syndrome and Port-Wine Stains Caused by Somatic Mutation in. NEJM. 8 May 2013. 368(21):1971–9. PMID 23656586. doi:10.1056/NEJMoa1213507

5. Eerola I, Boon LM, Mulliken JB, et al. Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations. Am. J. Hum. Genet. December 2003. 73(6):1240–9 PMID 14639529 doi:10.1086/379793

Author Information: Dr. Sarosh A. Khan is Senior Consultant, Internal Medicine and Director, Naseem Medical Center, Baghe-Mehtab, Srinagar, Kashmir, India. Pin: 190019. He is the Editor in Chief of Physicians Academy. Affiliations: Governing Council Member of American College of Physicians (India Chapter) from 2014-2017. Email: drsarosh@gmail.com


Dear Readers: Would you like to contribute to this page? Send us your contribution (photograph and 1-3 paragraphs about it) by email to drsarosh@gmail.com

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