CME examination

CME examination

To claim CME credit, complete this case-based posttest online at http://www.aad.org/JAAD/. Note: CME quizzes are available after the first of the mont...

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To claim CME credit, complete this case-based posttest online at http://www.aad.org/JAAD/. Note: CME quizzes are available after the first of the month in which the article is published. A minimum score of 70% must be achieved to claim CME credit. If you have any questions, please contact the Member Resource Center of the American Academy of Dermatology at toll-free (866) 503-SKIN (7546), (847) 240-1280 (for international members), or by e-mailing [email protected].

CME examination Identification No. JB1212 December 2012 issue of the Journal of the American Academy of Dermatology.

Questions 1-4, Chantorn R, Lim HW, Shwayder TA. J Am Acad Dermatol 2012;67:1113-27.

Directions for questions 1-5: Choose the single best response. A 2-year-old Japanese-American boy presents with a history of severe sunburn reaction that has been occurring since birth. When he was 3 days old, he developed neonatal hyperbilirubinemia and was treated with blue light phototherapy. After 3 hours of exposure to the blue light, he developed generalized erythroderma that persisted for several days. He has microcephaly, and also has marked freckling on the sun-exposed areas of his body since he was 1 year of age. His developmental milestones are delayed, and he appears to have eye pain when exposed to sunlight. 1. What is the most likely abnormality in this patient? a. Chromosomal instability b. Defective nucleotide excision repair of ultraviolet lighteinduced DNA damage c. Defective repair of actively transcribed DNA d. Elevated erythrocyte protoporphyrin levels e. Reduction of cysteine-rich protein in hair shaft 2. What is the earliest neurologic finding found in this disease? a. Ataxia b. Diminished deep tendon reflex c. Microcephaly d. Seizure e. Spasticity

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The patient was followed in your office regularly. At 8 years of age, he was diagnosed with squamous cell carcinoma on the forehead. 3. During counseling, you informed his parents that compared to the general population, the risk of developing cutaneous malignancy in patients with this condition is which of the following? a. 10-fold higher b. 100-fold higher c. 1,000-fold higher d. 10,000-fold higher e. No appreciable difference A 1-year-old white boy presents with a history of recurrent skin eruptions exacerbated by sun exposure. It gradually evolves to reticulated hyper- and hypopigmented patches on sun-exposed areas. He also has sparse hairs and limb defects on both hands. His developmental milestones are delayed. 4. What is the most common malignancy found in this disease? a. Basal cell carcinoma b. Leukemia and lymphoma c. Malignant melanoma d. Osteosarcoma e. Squamous cell carcinoma 5. Mutations in which of the following genes is responsible for this disease? a. BLM helicase b. ERCC6 c. POLH d. RECQL4 e. XPC