Board Review - In Partnership with Derm In-Review openaccess articles

June 2015 | Volume 14 | Issue 6 | Feature | 631 | Copyright © 2015

Kathleen Sikora Viscusi, MD

Abstract

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table 1

An 86-year-old Caucasian female with no significant past medical history presents with a 5-centimeter exophytic ulcerated nodule, as shown in Image A. Biopsy is performed and shows peripheral palisading, retraction artifact, fibromyxoid stroma, and strands of basophilic staining cells. After thorough discussion of treatment options, the patient is started on vismodegib 150 mg once daily. She returns for follow-up 45 days later, as shown in Image B. All of the following are true regarding the treatment medication except:

A.  Vismodegib targets a 7-transmembrane G-protein-coupled receptor.
B.  GLI1 expression is upregulated during treatment with vismodegib.
C.  In plasma, vismodegib is heavily bound to proteins, including high affinity binding with alpha 1-acid glycoprotein.
D.  Vismodegib has an estimated half-life of 12 days after single dose administration and four days after continuous once daily administration.
E.  Vismodegib is an oral small-molecule inhibitor of the hedgehog pathway (Hh).

table 2

A 65 year-old Caucasian female complains of a lifelong history of progressive “cysts” on her face and body, as per the clinical image. She has not sought treatment for these in the past. History is significant for an unknown type of leukemia as a child treated successfully with a bone marrow transplant as well as similar “cysts” affecting her mother and maternal grandfather. Clinical exam reveals innumerable dermal and subcutaneous soft, mobile nodules and multiple (>10) evenly bordered tan/brown patches on the trunk and extremities, ranging from 2-4 cm in size. Based on the history and clinical presentation, which of the following tumors was most likely also present when the patient was a child?

A.  Angiofibromas
B.  Pilomatricomas
C.  Juvenile xanthogranulomas
D.  Basal cell carcinomas
E.  Trichoepitheliomas

table 3

A 45-year-old female patient presents with cosmetic concern regarding asymptomatic freckling on the lower lip of approximately 5 years duration. She reports gradual increase in pigmentation and number of lesions in the first 3 years, with lesions more or less stable in size and number during the most recent 2 years. She is otherwise healthy and denies any other somatic complaints. Personal and family history is negative for melanoma or other types of skin cancer, pigmentary abnormalities, colon polyps or cancer, cardiac disease and arrhythmias, or endocrine disorders. Complete physical exam is unremarkable other than ill-defined hyperpigmentation on the upper and lower vermilion of the lips and multiple brown macules on the lower lip, as shown, as well as a 2 mm wide longitudinal pigmented band on the right 3rd fingernail. Biopsy of a representative lesion on the lower lip reveals increased epithelial basal layer pigmentation and normal number and appearance of melanocytes. Based on clinical and histologic correlation, the patient is diagnosed with Laugier-Hunziker syndrome. What the most appropriate next step in managing this patient?

A.  Upper and lower endoscopy
B.  Reassurance as the pigmentation will fade naturally with time
C.  ACTH stimulation text
D.  Cardiac evaluation
E.  Q-switched alexandrite laserA.

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