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.
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.
Pemphigus vulgaris is a serious but rare autoimmune disorder of the epithelial cells and mucosal lining of the skin. The first cases were recorded by McBride in 1771 and by Whichman in 1979. McBride was to describe the first to casualties as being caused by “bloody ichor” and “putrid ulcers”. (Jordon, 2013) However, Whichman was the one who would originally name the disease as pemphigus. The word “pemphigus” is of Greek origin which means blister or bubble. (Jordon, 2013) As the name suggests, this disease causes painful blisters or bullous erosions of the squamous epithelia and mucous membranes. It can be sub-classified based on the location of the lesions in the epidermis. Pemphigus vulgaris is considered to be one of the deep forms of the disease. The lesions appear deep into the epidermis of the skin right above the basal lamina. (Zeina, 2013)
Skin lesions are often difficult to assess only by visual inspection. Diagnostic testing, a history related to chief complaint and a provider’s experience may all be necessary to reach a definitive diagnosis. Diagnostic testing for presentations similar to this are not common, unless the lesion becomes persistent or reoccurring. In this case, the primary provider had the experience to ascertain the cause. The student could only categorize it as a skin lesion from possible
Philip Mortimer BMJ: British Medical Journal , Vol. 321, No. 7269 (Nov. 4, 2000) , p. 1123
Schepis, Carmelo, Donatella Greco, and Corrado Romano. "Cardiofaciocutaneous (CFC) Syndrome." Australasian Journal of Dermatology 40.2 (1999): 111-13. Print.
A doctor may suspect Epidermolysis Bullosa by the appearance of your skin but many tests must be performed on a patient to fully diagnose the disease. Many te...
From what my patient is showing and complaining of gives me two differential diagnoses of what he actually might be suffering from.
Epidermolysis Bullosa is an umbrella term for a group of skin disorders that involve fragile skin and mucosa that blister for no apparent reason. These skin disorders are passed down genetically (Sarkar, Bansal, Garg, 2011). Epidermolysis is a rare disease but there have been many efforts of research to help aid in the prevention and management of this disease.
The New England Journal of Medicine -- February 1, 1996 -- Vol. 334, No. 5
...d and will be published in JSciMed Central, one of the International peer reviewed dermatologic journals.
There are more than ten inherited disorders within Elhers-Danlos syndrome. Ehlers-Danlos syndrome (EDS) is a “genetic defect in collagen and connective tissue synthesis and structure” (Schwartz, 2013). EDS affects the skin, joints and blood vessels in most types. In EDS the abnormality of the collagen varies dependent on the type of EDS. Six of the main types of Ehlers-Danlos syndrome include; types I and II EDS which are called the classic type, type III hypermobile EDS, type IV vascular EDS, type VI kyphoscoliosis EDS, type VII A and B arthrochalasia EDS, and type VII C dermatosparaxis EDS (Willacy, 2011).
The term, “Ichthyosis”, is a skin disorder generally causing dry, scaly skin. It is affecting around 1 in 250 people and is typically an autosomal dominant inherited disease; although, a rare non-heritable version called “acquired ichthyosis” exists to this day in modern age. The condition is not life-threatening, however, the impact on the patient, if it is a mild case, is commonly regulated to mild itching and the social impression of having skin with an unusual appearance will affect those surrounding the person tremendously. People afflicted with mild cases acquire symptoms which comprises scaly patches on the shins, fine white scales on the forearms and upper arms, and rough palms. These severe cases involve the “build-up” of scales everywhere. Furthermore, when the “build-up” of scales are depraved, the person with a severe case suffers from "prickly itch" when he or she needs to sweat but cannot because of the scales. Various contemporary treatments are available to "exfoliate" the scales. These include lotions that contain alpha-hydroxy acids.
Psoriasis is a chronic skin disorder, easily identified by its symptoms of white, scaly skin and red lesions, though not so easily cured or understood. In psoriasis, skin cells mature faster than the body can shed them, causing a buildup. Although there are many theories as to what the cause of such a disease might be – genetics, stress, or other triggers – no one is quite sure why the disease occurs, or what could be a possible way to fully cure it. In this essay we will explore the symptoms, types, and effects of this condition, and also some of the known treatments.
... Medicine. 3rd ed. Vol.3. Detroit: Gale, 2006.2139-2141. Gale Virtual Reference Library. Web. 3 Apr. 2014.