Background Steven-Johnson Syndrome (SJS) is an immune mediated hypersensitivity complex, most often triggered by medications (Foster, 2011). It is characterized by a prodrome of malaise and fever, followed by rapid onset of erythematous or purpuric macules and plaques. The skin lesions progress to epidermal necrosis and sloughing. Mucosal membranes are affected in 92 to 100 percent of patients, usually at two or more distinct sites (High & Nirken, 2012). The syndrome was first described in 1922, when the American pediatricians Albert Mason Stevens and Frank Chambliss Johnson reported the cases of 2 boys aged 7 and 8 years with "an extraordinary, generalized eruption with continued fever, inflamed buccal mucosa, and severe purulent conjunctivitis". Both cases had been misdiagnosed by primary care physicians as hemorrhagic measles (Foster, 2011). Erythema multiform (EM), originally described by von Hebra in 1866, was part of the differential diagnosis in both cases but was excluded because of the "character of skin lesions, the lack of subjective symptoms, the prolonged high fever, and the terminal heavy crusting." Despite the presence of leukopenia in both cases, Stevens and Johnson in their initial report suspected an infectious disease of unknown etiology as the cause (Hazin, Ibrahimi, Hazin & Kimyai-Asadi, 2008). In 1950, Thomas divided EM into 2 categories: erythema multiform minor and erythema multiform major. Since 1983, erythema multiform major and Stevens-Johnson syndrome had been considered synonymous (Foster, 2011). In the 1990s, however, Bastuji and Roujeau each proposed that erythema multiform major and Stevens-Johnson syndrome are 2 distinct disorders (Roujeau, 1997). They suggested that the diagnosis of erythema ... ... middle of paper ... ...of the mucous membranes, vaginal synechiae, and nail dystrophy, and diffuse hair loss (Habif, 2004). References Foster, C. S. (2011, September 23). Steven-Johnson syndrome. Retrieved from http://www.emedicine.medscape.com/article/1197450 Hazin, R., Ibrahimi, O. A., Hazin, M. I., & Kimyai-Asadi, A. (2008). Steven-Johnson syndrome: Pathogenesis, diagnosis, and management . Annals of Medicine, 40(2), 129-138. High, W.A., Nirken, M.H. (2012). Steven-Johnson syndrome and toxic epidermal necrolysis: Management, prognosis, and long-term sequelae. Retrieved from http://www.uptodate.com/contents/steven-johnson-syndrome-and-toxic-epidermal-necrolysis McCance, K. L., Huether, S. E., Brashers, V. L., & Rote, N. S. (2010). Pathophysiology: The biological basis for disease in adults and children. (6th ed., pp. 1644-1645). Maryland Heights, Virginia: Mosby.
To begin we will look at the integumentary system and its entire multitude of functions. The main components of the integumentary system are the skin, hair, nails, glands and nerves. For the purpose of this paper we will focus mainly on the levels of the skin and their functions. While the integumentary
Arch Dermatol. 2007;143(1):124–125. Puchenkova, S. G. (1996). "
There are approximately six types of EDS that have been distinguished, but other types exist that are very uncommon. Classical, Hypermobile, Vascular, Kyphoscoliosis, Arthrochalasia, and Dermatosparaxis. Classical and Hypermobile make up over 90% of all reported cases of EDS. With the Classical type of EDS a person would have hyperextensible (stretchy) skin with widened atrophic scars and joint hypermobility. The skin is smooth and velvety with tissue fragility and easy brusability.
Barone, Eugene J., Judson C. Jones, and Joann E. Schaefer. "Hidradenitis Suppurativa." Skin Disorders. Philadelphia: Lippincott Williams & Wilkins, 2000. 21-25. Print.
that cause dark red blotches on the skin, usually on the face). Laboratory tests are another consideration of diagnosis. Tests consist of anti-nuclear anti-body (ANA) counts and anti-topoisomerase (an enzyme that reduces super-coiling in DNA by breaking and rejoining one or both strands of DNA). High ANA’s and low anti-topoisomerases are found in patients with Raynaud syndrome. (Desai, 2003) “Patients with circulating autoantibodies, antinuclear antibodies, and anti-Scl 70 antibodies are at (an) increased risk of developing a connective tissue disease. Systemic sclerosis is the connective tissue disease most frequently associated with Raynaud’s phenomenon.” (Bowling, 2003) This syndrome is described as primary Raynaud phenomenon (PRP) if is not associated with another disorder and as secondary Raynaud phenomenon (SRP) if it occurs in association with another disorder.
In 1931 the first case of Wegener’s Disease was discovered by Heinz Klinger, a German medical student (although it was not known as Wegener’s Disease at that time). It was not until several years later in 1936, that three more cases were discovered. A German pathologist, Friedrich Wegener described and found this disorder as a distinct form of Vasculitis, a rare blood vessel inflammation that since the 1950’s has been called Wegener’s Disease Granulomatosis. (http://www.hopkinsvasculitis.org/types-vasculitis/wegeners-granulomatosis/2016)
Huether, S.E. & McCance, K.L. (2008). Understanding pathophysiology (4th ed.). St. Philadelphia, PA: Mosby Elsevier
Cohen, S. (2013, January). Student Health 101 @ Ashford University. Retrieved April 1, 2014, from http://readsh101.com/ashfordu.html?id=ec8bd17d
Bratton, R. L., Whiteside, J. W., Hovan, M. J., Engle, R. L., Edwards, F. D. (2008). Diagnosis
U.S. National Library of Medicine, 26 Sept. 2011. Web. The Web. The Web. 19 Nov. 2013.
Measles is a highly contagious disease. It is caused by an RNA virus that changes constantly. Measles symptoms usually include a bad cough, sneezing, runny nose, red eyes, sensitivity to light, and a very high fever. Red patches with white grain like centers appear along the gum line in the mouth two to four days after the first symptoms show. These patches are called Koplik spots because Henry Koplick first noticed them in 1896. The Spots are important to diagnose measles. A characteristic red rash in measles is red spots starting at the hairline and going down to the face, body, and limbs.
Uhland, Vicky. “The Picture of Health.” Momentum 6.3 (2013): 42-45. Academic Search Premier. Web. 20 Mar. 2014.
Ed. David Zieve. U.S. National Library of Medicine, 26 Feb. 2014. Web. The Web.
of 1852 and 1853 the symptoms are thought to have become more serious, and in
Rubella also commonly referred to as “German Measles” was previously believed to be a variation of measles until 1814 when it was first correctly indicated as a separate disease in German medical literature. Although the the rubella rash presents similar to the rash associated with measles, rubella is less severe and infectious. Rubella is distinguished by a red rash that first presents on the face and spreads to the trunk, arms, and legs and disappears in the same progression. The rash looks similar to many other viral rashes and must be confirmed by blood tests for the presence of rubella antibodies.