Often the first symptoms of Bullous Pemphigoid
What is severe itching of the skin?
This type of hypersensitivity reaction describes bullous pemphigoid.
What is Type II?
This type of biopsy is obtained for an H&E stain in bullous pemphigoid.
What is a lesional biopsy?
These are the major goals for Tx of bullous pemphigoid
Decrease blister formation and pruritus
Promote healing of blisters and erosions
Improve quality of life
True or False: Patients with bullous pemphigoid will have a positive Nikolsky sign
False: they will have a negative Nikolsky sign
Four risk factors for Bullous Pemphigoid
Older adults
Neruo diseases (Parkinson disease, Stroke, Epilepsy)
Burns or Trauma
UV radiation exposure
Meds(penicillins or dieretics)
Disorders that may trigger it (psoriasis, lichen planus, diabetes, rheumatoid arthritis, ulcerative colitis, and multiple schlerosis
This transmembrane protein adheres basal keratinocytes in stratum basalis to the basement membrane and is targeted in bullous pemphigoid.
What is BP180?
This type of cell composes the blisters seen in bullous pemphigoid and may be seen as elevated on a CBC.
What are eosinophils?
This therapy is associated with lower mortality and risk for severe complications. Challenges with its administration can limit use in some settings.
This blistering skin disorder is associated with celiac disease.
What is dermatitis herpetiformis?
4 symptoms of pemphigus vulgaris
lesions extending deep into the skin
blisters that usually start in mouth or on scalp
Red skin
Painful, open sores,
blisters that may expand into surrounding tissue when pressure is added
Soft and easily broken blisters that release fluid
Name one infection known to trigger bullous pemphigoid.
What are hepatitis B, hepatitis C, Helicobacter pylori, Toxoplasma gondii, and cytomegalovirus?
These two types of blood antibodies will be seen in an ELISA.
What are anti-BP180 and anti-BP230 antibodies?
This is a benefit of adding corticosteroid-sparing therapy to Bullous pemphigoid Tx
What is facilitating corticosteroid tapering and reducing adverse effects
This type of biopsy is obtained for direct immunofluroescense .
What is a perilesional biopsy?
This blistering disorder is characterized by fever, bullae formation and necrosis, + Nikolsky sign, high mortality rate. Mucous membranes typically involved. Targetoid skin lesions may appear
What is Stevens-Johnson syndrome?
A 73-year-old man comes to the clinic because of itchy and sensitive skin, which started acting up a few weeks ago. He believes that he has been having allergic reactions, along his abdomen to a new lotion his wife had recently purchased. He is worried because over-the-counter allergy medications have not been working, and blisters began forming along the lesions. Physical examination reveals multiple, stable blisters and hive-like lesions along the abdomen. Adjacent skin was negative for Nikolsky sign. Which of the following would most likely be seen on a skin biopsy?
A. Direct immunofluorescence is positive for IgG and C3 deposition within the epidermis
B. Acantholytic cells in the upper layers of epidermis
C. Direct immunofluorescence is positive for granular deposition of IgG and C3 along the basement membrane
D. Histological analysis shows subepidermal bullae with dense, eosinophilic infiltrate
E. Indirect immunofluorescence demonstrates the presence of circulating IgG autoantibodies
D.Histological analysis shows subepidermal bullae with dense, eosinophilic infiltrate
Pattern seen on direct immunoflourescence of bullous pemphigoid.
What are intracellular IgG deposits along the basement membrane in a linear pattern?
Doxycycline has bacteriostatic activity through this mechanism of action
What is inhibiting bacterial protein synthesis by binding to the 30s ribosomal subunit
Can act against a broad range of Gram-positive and Gram-negative bacteria
A 45-year-old man presents to the office because of oral pain. The pain has been constant for several weeks and worsens with chewing and swallowing food. He also reports the development of non-pruritic, tender skin sores on his arms and legs, which occurred two weeks following the onset of the oral pain. He has been working as a gardener in California for 20 years. Physical examination shows multiple erosions and flaccid bullae on the extremities and buccal mucosa. When lateral pressure is applied on the bullae, the overlying layer easily slips off, revealing an erythematous base. He was diagnosed with:
Pemphigus vulgaris
A 60-year-old man comes to the physician because of tender blisters on his arm and flank, worsening over the last few months. His medical history includes hypertension and hyperlipidemia. He is taking atorvastatin and hydrochlorothiazide for these conditions. Physical examination shows blisters and flaccid bullae; a few have ruptured, leaving red, sore, denuded areas. Pathologic examination reveals acantholysis and few lymphocytes.
Which of the following findings is most likely in this patient?
A. Eosinophils within bullae.
B. IgA deposits in the dermal papillae.
C. IgG antibody against hemi-desmosomes.
D. Negative Nikolsky sign.
E. Oral mucosal lesions.
E. Oral mucosal lesions.
An 80-year-old woman comes to the emergency department because of widespread blisters along her abdomen and arms. The patient's caretaker denies any recent burns, and her reported past medical history is unremarkable aside from an upper respiratory infection a few weeks ago. The blisters are tense, and filled with either blood-stained or clear fluid. They appear to be pruritic, as evidenced by the patient’s frantic scratching and the presence of multiple scratch marks along the blisters and adjacent skin. Rubbing of the blister and neighboring skin did not result in breakage or new blister formation. Her wounds are dressed, and the patient is prescribed both oral prednisone and topical corticosteroids. Which of the following is the most likely pathophysiology of this disease?
A. An autosomal dominant defect of uroporphyrinogen decarboxylase causing abnormalities during heme synthesis
B. Autoantibodies attack Type XVII collagen, causing a split in between the dermis and epidermis
C. Autoantibodies against desmoglein 1 and desmoglein 3, causing loss of cohesion between the epidermis and keratinocytes
D. Deposition of IgA antibodies in the papillary dermis
E. Type IV delayed hypersensitivity reaction to the upper respiratory infection
B. Autoantibodies attack Type XVII collagen, causing a split in between the dermis and epidermis
A 35-year-old man comes to the clinic because of painful severe blistering over his buttocks for the past week. About a year ago, he noticed a similar outbreak on his inner thighs, but it receded within a few days on its own. Physical examination shows that blisters are tense, and rubbing the affected skin does not result in “popping” of the blisters. A biopsy shows extensive inflammatory infiltrates abundant with eosinophils. Which of the following cellular structures, if defective, is most likely involved in the formation of these blisters?
A. Gap Junctions
B. Hemidesmosomes
C. Lamellar bodies
D. Macula adherens
E. Zonula occludens
B. Hemidesmosomes
A 67-year-old woman comes to the emergency room because of a widespread, blistering rash. She denies use of any new products or recent burns. Physical examination shows the affected areas to be negative for Nikolsky sign, with blisters being hard, tense and difficult to burst. Which of the following would be the most appropriate first-line treatment of this condition?
A.Rupture the blister and clean thoroughly so as to prevent infection
B. Systemic steroids and immunosuppressive agents, such as prednisone and azathioprine, respectively
C. Apply opaque sunblock when in the sun, and weekly or biweekly phlebotomy
D. Intramuscular injections of gold thiomalate
E.Topical antibacterials, such as 1% silversulfadiazole or 0.5% silver nitrate solution
B. Systemic steroids and immunosuppressive agents, such as prednisone and azathioprine, respectively
A 60-year-old woman comes to the dermatology clinic because of blisters appearing on her face. Three lesions are noted on physical examination, each measuring between 1 and 2 cm, and are filled with clear fluid. She does not have any similar lesions on her mucous membranes. She says these lesions have appeared before, and they usually heal without scarring. Immunofluorescence shows a linear pattern of IgG antibody deposition. The antibody is most likely against which component of the skin?
A. Desmosomes
B. Hemidesmosomes
C. E-cadherins
D. Connexons
E. Actin filaments
B. Hemidesmosomes