Jones 1

Question 1

Features of Pemphigus

Answer 1

-Autoimmune disease that affects the skin and mucous membranes -Autoantibodies are directly against the epidermal cell surface glycoproteins, desmoglein 3 and desmoglein 1 -Desmogleins are components of desmosomes that hold epithelial cells together

Question 2

Manifestations of Pemphigus

Answer 2

-Initial manifestations often involve the oral mucosa -In 50% of cases, the oral lesions precede the development of skin lesions -Average age of diagnosis = 50; F = M -Positive Nikolsky sign

Question 3

Histopathologic features of Pemphigus

Answer 3

-Intraepithelial separation that occurs just above the basal cell layer of the epithelium -Acantholysis and Tzanck cells -DIF: IgG or IgM and C3 in intercellular areas in most cases -IIF: circulating antibodies in 80-90% of cases

Question 4

Treatment and prognosis of Pemphigus

Answer 4

-Systemic corticosteroids in combination with other immunosuppressive drugs -Success of treatment can be monitored by measuring titers of circulating autoantibodies -Mortality rate: 5-10%

Question 5

Features of Mucous Membrane Pemphigoid

Answer 5

-Also known as cicatrical pemphigoid -Chronic, blistering, mucocutaneous autoimmune disease -Autoantibodies are produced against one or more components of the basement membrane -Twice as common as pemphigus -Average age of 50 to 60 years; F > M (2:1)

Question 6

Manifestations of Mucous Membrane Pemphigoid

Answer 6

-May involve the oral, conjunctival, nasal, esophageal, laryngeal, and vaginal mucosa as well as the skin -Desquamative gingivitis -Erosive lichen planus and pemphigus

Question 7

Diseases associated with Mucous Membrane Pemphigoid

Answer 7

-25% of patients with oral lesions develop ocular disease -Symblepharon: adhesion between the bulbar and palpebral conjunctivae -Entropion: Eyelids turn inward -Trichiasis: Eyelashes rub against the cornea and globe -Eyes become dry and cornea produces keratin which leads to blindness

Question 8

Histopathologic features of Mucous Membrane Pemphigoid

Answer 8

-Split between the surface epithelium and the underlying connective tissue -DIF: linear band of IgG and C3 at the basement membrane zone - 90% of cases -IIF: only positive in 5-25% of cases; lack of circulating autoantibodies

Question 9

Treatment and prognosis of Mucous Membrane Pemphigoid

Answer 9

-Patients should be referred to an ophthalmologist -Topical or systemic corticosteroids -Tetracycline and niacinamide