Fast Facts for Board Review

1. Which of the following enzymes of the heme synthesis pathway is found in the mitochondria?

a. δ-aminolevulinic oxidase

b. porphobilinogen deaminase

c. protoporphyrinogen oxidase

d. uroporphyrinogen decarboxylase

e. uroporphyrinogen III synthase

2. A patient with variegate porphyria uniquely has plasma fluorescence at the following wavelength:

a. 311 nm

b. 366 nm

c. 410 nm

d. 626 nm

e. 630 nm

3. What laboratory result would be expected to be abnormal in a patient with a defect in ferrochelatase?

a. elevated δ-aminolevulinic acid

b. elevated erythrocyte protoporphyrins

c. elevated fecal coproporphyrins

d. elevated urinary porphobilinogen

e. elevated urinary uroporphyrins

4. What clinical feature would be present in a newborn diagnosed with transient erythroporphyria of infancy?

a. dark red urine

b. immediate burning photosensitivity

c. red teeth that fluoresce under Wood lamp

d. skin purpura

e. urine that fluoresces pink under Wood lamp

5. What medication should be avoided in a patient with a defect in porphobilinogen deaminase?

a. captopril

b. furosemide

c. griseofulvin

d. naproxen

e. tetracyclines

1. Which of the following enzymes of the heme synthesis pathway is found in the mitochondria?

a. δ-aminolevulinic oxidase

b. porphobilinogen deaminase

c. protoporphyrinogen oxidase

d. uroporphyrinogen decarboxylase

e. uroporphyrinogen III synthase

2. A patient with variegate porphyria uniquely has plasma fluorescence at the following wavelength:

a. 311 nm

b. 366 nm

c. 410 nm

d. 626 nm

e. 630 nm

3. What laboratory result would be expected to be abnormal in a patient with a defect in ferrochelatase?

a. elevated δ-aminolevulinic acid

b. elevated erythrocyte protoporphyrins

c. elevated fecal coproporphyrins

d. elevated urinary porphobilinogen

e. elevated urinary uroporphyrins

4. What clinical feature would be present in a newborn diagnosed with transient erythroporphyria of infancy?

a. dark red urine

b. immediate burning photosensitivity

c. red teeth that fluoresce under Wood lamp

d. skin purpura

e. urine that fluoresces pink under Wood lamp

5. What medication should be avoided in a patient with a defect in porphobilinogen deaminase?

a. captopril

b. furosemide

c. griseofulvin

d. naproxen

e. tetracyclines

1. Which of the following enzymes of the heme synthesis pathway is found in the mitochondria?

a. δ-aminolevulinic oxidase

b. porphobilinogen deaminase

c. protoporphyrinogen oxidase

d. uroporphyrinogen decarboxylase

e. uroporphyrinogen III synthase

2. A patient with variegate porphyria uniquely has plasma fluorescence at the following wavelength:

a. 311 nm

b. 366 nm

c. 410 nm

d. 626 nm

e. 630 nm

3. What laboratory result would be expected to be abnormal in a patient with a defect in ferrochelatase?

a. elevated δ-aminolevulinic acid

b. elevated erythrocyte protoporphyrins

c. elevated fecal coproporphyrins

d. elevated urinary porphobilinogen

e. elevated urinary uroporphyrins

4. What clinical feature would be present in a newborn diagnosed with transient erythroporphyria of infancy?

a. dark red urine

b. immediate burning photosensitivity

c. red teeth that fluoresce under Wood lamp

d. skin purpura

e. urine that fluoresces pink under Wood lamp

5. What medication should be avoided in a patient with a defect in porphobilinogen deaminase?

a. captopril

b. furosemide

c. griseofulvin

d. naproxen

e. tetracyclines

1. What malignancy is most commonly associated with alopecia neoplastica?

a. breast cancer
b. gastric cancer
c. lung cancer
d. pancreatic cancer
e. renal cancer

2. What urine test would be ordered for a patient with suspected carcinoid syndrome?

a. cortisol
b. histamines
c. 5-hydroxyindoleacetic acid
d. protein level
e. vanillylmandelic acid

3. Which type of amyloid should a patient with multiple myeloma and primary systemic amyloidosis exhibit?

a. protein derived from β-amyloid protein
b. protein derived from β2-microglobulin protein
c. protein derived from immunoglobulin light chains
d. protein derived from keratin
e. protein derived from serum amyloid A protein, an acute-phase reactant

4. Antiepiligrin pemphigoid is most commonly characterized by autoantibody production against:

a. β4-integrin
b. bullous pemphigoid antigen 2
c. laminin-6
d. laminin-332
e. type VII collagen

5. What is the most common malignancy seen in patients who are diagnosed with acquired ichthyosis?

a. chronic lymphocytic leukemia
b. Hodgkin lymphoma
c. multiple myeloma
d. non-Hodgkin lymphoma
e. Waldenström macroglobulinemia

1. What malignancy is most commonly associated with alopecia neoplastica?

a. breast cancer
b. gastric cancer
c. lung cancer
d. pancreatic cancer
e. renal cancer

2. What urine test would be ordered for a patient with suspected carcinoid syndrome?

a. cortisol
b. histamines
c. 5-hydroxyindoleacetic acid
d. protein level
e. vanillylmandelic acid

3. Which type of amyloid should a patient with multiple myeloma and primary systemic amyloidosis exhibit?

a. protein derived from β-amyloid protein
b. protein derived from β2-microglobulin protein
c. protein derived from immunoglobulin light chains
d. protein derived from keratin
e. protein derived from serum amyloid A protein, an acute-phase reactant

4. Antiepiligrin pemphigoid is most commonly characterized by autoantibody production against:

a. β4-integrin
b. bullous pemphigoid antigen 2
c. laminin-6
d. laminin-332
e. type VII collagen

5. What is the most common malignancy seen in patients who are diagnosed with acquired ichthyosis?

a. chronic lymphocytic leukemia
b. Hodgkin lymphoma
c. multiple myeloma
d. non-Hodgkin lymphoma
e. Waldenström macroglobulinemia

1. What malignancy is most commonly associated with alopecia neoplastica?

a. breast cancer
b. gastric cancer
c. lung cancer
d. pancreatic cancer
e. renal cancer

2. What urine test would be ordered for a patient with suspected carcinoid syndrome?

a. cortisol
b. histamines
c. 5-hydroxyindoleacetic acid
d. protein level
e. vanillylmandelic acid

3. Which type of amyloid should a patient with multiple myeloma and primary systemic amyloidosis exhibit?

a. protein derived from β-amyloid protein
b. protein derived from β2-microglobulin protein
c. protein derived from immunoglobulin light chains
d. protein derived from keratin
e. protein derived from serum amyloid A protein, an acute-phase reactant

4. Antiepiligrin pemphigoid is most commonly characterized by autoantibody production against:

a. β4-integrin
b. bullous pemphigoid antigen 2
c. laminin-6
d. laminin-332
e. type VII collagen

5. What is the most common malignancy seen in patients who are diagnosed with acquired ichthyosis?

a. chronic lymphocytic leukemia
b. Hodgkin lymphoma
c. multiple myeloma
d. non-Hodgkin lymphoma
e. Waldenström macroglobulinemia

1. What is the antigen targeted in ocular cicatricial pemphigoid?

a. α6 subunit of α6β4 integrin

b. β4 subunit of α6β4 integrin

c. desmocollin

d. laminin-5

e. laminin-6

2. Which of the following conditions is most commonly associated with paraneoplastic pemphigus?

a. Castleman disease

b. chronic lymphocytic leukemia

c. multiple myeloma

d. non-Hodgkin lymphoma

e. solid organ adenocarcinomas

3. A patient develops multiple vesicles and bullae arranged in a crown of jewels configuration after receiving an antibiotic in the hospital for sepsis. Which of the following drugs is the most likely cause?

a. chloramphenicol

b. ertapenem

c. meropenem

d. piperacillin-tazobactam

e. vancomycin

4. What is the suspected vector for fogo selvagem?

a. Anopheles

b. Glossina

c. Lutzomyia

d. Phlebotomus

e. Simulium

5. Chronic bullous dermatosis of childhood is characterized by autoantibody production against:

a. BPAg2 LAD-1

b. C terminal of BPAg2

c. collagen VII

d. collagen IV

e. NC16A domain of BPAg2

1. What is the antigen targeted in ocular cicatricial pemphigoid?

a. α6 subunit of α6β4 integrin

b. β4 subunit of α6β4 integrin

c. desmocollin

d. laminin-5

e. laminin-6

2. Which of the following conditions is most commonly associated with paraneoplastic pemphigus?

a. Castleman disease

b. chronic lymphocytic leukemia

c. multiple myeloma

d. non-Hodgkin lymphoma

e. solid organ adenocarcinomas

3. A patient develops multiple vesicles and bullae arranged in a crown of jewels configuration after receiving an antibiotic in the hospital for sepsis. Which of the following drugs is the most likely cause?

a. chloramphenicol

b. ertapenem

c. meropenem

d. piperacillin-tazobactam

e. vancomycin

4. What is the suspected vector for fogo selvagem?

a. Anopheles

b. Glossina

c. Lutzomyia

d. Phlebotomus

e. Simulium

5. Chronic bullous dermatosis of childhood is characterized by autoantibody production against:

a. BPAg2 LAD-1

b. C terminal of BPAg2

c. collagen VII

d. collagen IV

e. NC16A domain of BPAg2

1. What is the antigen targeted in ocular cicatricial pemphigoid?

a. α6 subunit of α6β4 integrin

b. β4 subunit of α6β4 integrin

c. desmocollin

d. laminin-5

e. laminin-6

2. Which of the following conditions is most commonly associated with paraneoplastic pemphigus?

a. Castleman disease

b. chronic lymphocytic leukemia

c. multiple myeloma

d. non-Hodgkin lymphoma

e. solid organ adenocarcinomas

3. A patient develops multiple vesicles and bullae arranged in a crown of jewels configuration after receiving an antibiotic in the hospital for sepsis. Which of the following drugs is the most likely cause?

a. chloramphenicol

b. ertapenem

c. meropenem

d. piperacillin-tazobactam

e. vancomycin

4. What is the suspected vector for fogo selvagem?

a. Anopheles

b. Glossina

c. Lutzomyia

d. Phlebotomus

e. Simulium

5. Chronic bullous dermatosis of childhood is characterized by autoantibody production against:

a. BPAg2 LAD-1

b. C terminal of BPAg2

c. collagen VII

d. collagen IV

e. NC16A domain of BPAg2