Vesiculobullous, Autoimmune & Connective Tissue Disease Flashcards
If positive in a newly diagnosed patient with Dermatomyositis, what autoantibody results would indicate your patient is at an increased risk for interstitial lung disease?
Anti-Jo-1
Which finding is 2 times more common in juveniles than adults with dermatomyositis?
Calcinosis
Which cutaneous findings are considered pathognomonic for dermatomyositis?
Periorbital heliotrope and gottron’s papules
You have a patient with refractory dermatomyositis and want to order IVIG. You are reviewing the baseline labs. What result would indicate the patient is at increased risk for anaphylaxis?
Low or absent IgA level
What lab results would be most likely indicating an increased risk of life threatening bone marrow toxicity in a patient taking Azathioprine?
Low or absent TPMT activity
Ehlers-Danlos syndrome is caused by a genetic mutation in
Collagen fibers
The most common type of Ehlers-Danlos syndrome
Classic type
A baby born with bilateral hip displacement indicating which type of Ehlers Danlos syndrome?
Arthrochalasia Type
The term micrognathia refers to
Undersized jaw
The typical facial appearance that occurs with some types of Ehlers Danlos Syndrome
Eyes that appear to be staring
Typical skin findings in Ehlers-Danlos Classic Type include
Increased elasticity
The perilesional biopsy of a patient with a suspected linear IgA bullous dermatitis should demonstrate
Neutrophils
What medication is most likely to cause linear IgA bullous dermatitis?
Vancomyocin
More than half of all children with linear IgA bullous dermatitis will demonstrate lesions in which body area?
Genitalia
Adult onset of linear IgA bullous dermatitis is more likely to occur in which age group?
60 to 70 years of age
The lesions of linear IgA bullous disease in an adult patient are most likely to occur in what body area?
Extensor areas of extremities
Definitive diagnosis for linear IgA bullous dermatitis is
Linear deposits of IgA autoantibodies on the DIF
The drug of choice to treat linear IgA bullous dermatitis is
Dapsone
A lesion that has a scleortic center appearing shiny, white with a violaceous outer ring describes which type of morphea lesion?
Progressive Lesion
Which type of biopsy is recommended for suspected morphea?
Punch
Which treatment option has the highest degree of efficacy in treatment of morphea?
PUVA
Which type of pemphigus is the most common?
Pemphigus Vulgaris
Which medications are most likely to cause drug-induced pemphigus?
Captopril, penicillamine, thiol drugs, PCN, Cephalosporin, vancomyocin, ace inhibitors.
Paraneoplastic pemphigus is associated with
Lymphoma
First line treatment of pemphigus is
Systemic glucocorticoids
Histology examination of a patient with suspected pemphigus should demonstrate an abundance of what cell in the dermis?
Eosinophils
A skin finding that occurs where the tip layers of skin shear away from lower layers with slight rubbing describes
Nikolsky sign
The most common type of autoimmune blistering disorder is
Bullous pemphigoid
The onset of bullous pemphigoid usually starts after age
60
The initial antigen involved in the development of bullous pemphigoid is
BP antigen 180
Bullous pemphigoid has a significant association with what condition?
Neurological conditions
Drug induced bullous pemphigoid can be associated with which medication
Furosemide
A patient suspected of having bullous pemphigoid is to have a diagnostic punch biopsy(DiF). The correct location to perform this lesion is:
Perilesional area
The recommended first line treatment for a patient with severe bullous pemphigoid is
Systemic corticosteroids
The lesions of cicatricial pemphigoid are most likely to occur where
Eyes and mouth
Pemphigoid gestational generally occurs during
Both 2nd and 3rd trimesters
Lesions of pemphigoid gestationis often start where
Umbilicus
A patient diagnosed with dermatitis herpetiformis has just started dapsone therapy. When he presents for his 4 week check, he complains of fever, itchy rash, and right lower quadrant abdominal pain. Your first concern is
Dapsone hypersensitivity syndrome
Your patient with newly diagnosed dermatitis herpetiformis asks if he will have GI symptoms like celiac disease. Your response is
20%
You have a patient with a diagnosis of new onset sarcoidosis. What is the likelihood your patient has associated pulmonary disease?
90-95%
Lupus period can be associated with
Development of lytic and cystic bone lesions of the hands and feet
Lofgren syndrome is a triad of which symptoms
Erythema nodosum, polyarthritis and hilar lymphadenopathy (also fever)
Erythema nodosum can be described as
Most common non-specific eruption of sarcoidosis
Epidermolysis bullosa simplex located form primarily affects
Palms and soles
What are characteristics of Kindler syndrome of epidermolysis bullosa?
Skin fragility, dyspigmentation, acral blister formation at birth, photosensitivity in childhood, diffuse palmoplantar hyperkeratosis
The cleavage line associated with epidermolysis simplex is located at
Intraepidermal
A patient is suspected of having epidermolysis bullosa and needs a biopsy to confirm the diagnosis. The correct technique includes:
Obtaining 1/2 to the whole blister with surrounding skin
Treatment of pruritis symptoms in patients with epidermolysis bullosa should include antihistamines and topical application of
Petroleum jelly
Abnormalities of scleroderma include
Vascular dysfunction, tissue fibrosis and autoantibody production
What percentage of scleroderma patients experience Raynaud’s phenomenon?
95%
Patients with diffuse scleroderma may develop scar tissue in
Heart, lungs, skin
Mortality associated with scleroderma is usually due to problems with which system?
Pulmonary
The CREST acronym stands for
C - calcinosis
R - Raynaud’s
E - esophageal dysfunction
S - sclerodactyl
T - telangiectasia
The early stages of scleroderma begin with
Edema in the fingers
Lupus erythemstous is more common in
African American Females
An environmental trigger for lupus erythematous is
UV exposure
Which type of lupus erythematous is characterized by scarring alopecia, follicular plugging and indurated lesions?
Discoid lupus erythematous
Which medication is associated with drug-induced subacute lupus erythematous
Hydrochlorothiazide
The inflammation in lupus panniculitis is in the
Subcutaneous tissue
Neonatal lupus can be associated with
Heart block
Systemic lupus erythematous is commonly associated with
Weight loss
Acute cutaneous lupus erythematous may mimic what conditions?
Rosacea, sunburn, drug induced photosensitivity
The systemic drug of choice in treatment of lupus erythematous is
Hydroxychloroquine sulfate
A potential adverse effect associated with antimalarial medicatio is
Eye toxicities
