CME examination Identification No. 884-101

See "Instructions for Category 1 CME Credit" on page 33A of the front advertising section.

Questions 1-33, Fulk CS: J AN ACAD DEthMATOL 10: 1-16, 1984.

Directions for questions 1-I1: Give single best re- sponse. 1. Most primary hyperpigmentation conditions are

a. inherited in an autosomal dominant mode b. inherited in an autosomal recessive mode c. inherited in a sex (X) linked recessive mode d. inherited in a sex (X) linked dominant mode e. occur sporadically with no known genetic pre-

disposition

2. Caf~ au lair macules are especially prominent in size and number in neurofibromatosis, in A1- bright's syndrome, and in a. Gaucher's syndrome b. Westerhof's syndrome c. Watson's syndrome d. Lescke's syndrome e. ataxiartelangiectasia

3. Which one of the following pigmented lesions is generally large (greater than 1.5 cm) when seen at birth? a. Mongolian spot b. Caf6 au lait spot c. Nevocytic hyperpigmented birthmark d. Lentigo simplex e. Epidermal nevus

4. The sign of Leser-Trglat is especially associated with a. adenocarcinoma of the female mammary gland b. adenocarcinoma of the gastrointestinal tract c. squamous cell carcinoma of the bronchus d. transitional cell carcinoma of the urinary

bladder e. carcinoma of the thyroid gland

5. The de Sanctis-Cacchione syndrome is a variant of a. hereditary acrokeratotic poikiloderma b. dyskeratosis congenita c. Fanconi's anemia d. Wemer's syndrome e. xeroderma pigmentosum

6. A patient wifla which of the following appears chronically tired or as an abuser of depressant drugs or alcohol? a. Dyschromatosis universalis b. Da Costa's syndrome

c. Incontinentia pigmenti d. Hereditary acrokeratotic poikiloderma e. Familial perioral melanosis

7, Marked eosinophilia during infancy is characteris- tic of a. Fanconi's anemia b. Werner's syndrome c. dyskeratosis congenita d. hereditary acrokeratotic poikiloderma e. incontinentia pigmenti

8. Congenital pseudarthrosis and cervicothoracic kyphoscoliosis are characteristic of a. Watson's syndrome b. neumfibromatosis c. acromelanosis progressiva d. acropigmentation symmetrica of Dohi e. dermatopathia pigmentosa reticularis

9. Reported associations with the nevus of Ota in- clude all of the following, except a. Sturge-Weber syndrome b. Osler-Weber-Rendu syndrome c. Klippel-Trrnaunay-Weber syndrome d. neurosensory bearing loss e. spinocellular degeneration

10. Characteristics of the Moynahan syndrome include all of the following, except a. early appearance of hyperpigmented macules b. growth retardation c. genital abnormalities d. mental retardation e. psychic infantilism

11. All of the following are characteristics of Becket's pigmented hairy hamartoma, except a. most cases are developed prior to the age of 20

years b. the shoulder is the most common site c. hair is present in slightly more than half of the

patients d. pruritus or underlying hypoplasia is common e. histologically, numerous superficially located

nevocellular nevus cells are present

Directions for questions 12-16: Select the one let- tered item that is most closely related to each numbered item.

a. Werner's syndrome b. Franceschetti-Jadassohn-Naegeli syndrome c, Moynahan syndrome d. Peutz-Jegbers syndrome e. Confluent and reticulated papillomatosis

Gougerot and Carteaud of

17

18 CME examination Journal of the

American Academy of Dermatology

12. Endomyocardial fibrosis (congenital mitral stenosis) 13, Hamartomas of the bowel 14, Atrophy of subcutaneous fat and muscle 25, Palmoplantar hyperkeratosis and functional hypo-

hidrosis 16. Often misdiagnosed as finea versicolor by non-

dermatologists

Directions for questions 17-2]. Select the one let- tered item that is most closely related to each numbered item.

a, Autosomai dominant inheritance b. Autosomal recessive inheritance c. X-linked dominant inheritance d, X-linked recessive inheritance e, Y-linked inheritance

1"7. Incontinentia pigmenti 18. Centrofacial lentiginosis 19. Berlin's syndrome 20. Da Costa's syndrome 21. Poikiloderma congenitale of Rothmund and

Thomson

Directions for questions 22-26: For each num- bered item choose the appropriate lettered item,

a, Ephelides (freckles) b. Lentigines c. Both d. Neither

22. Become more prominent during summer months 23. Frequency is increased in redheaded and fair-

skinned persons 24, Elongated rete ridges 25, Increased numbers of basal melanocytes 26, May show basal melanization

Directions for questions 27-31: For each num- bered item choose the appropriate lettered item.

a. Albright's syndrome b. Von Recklinghausen's syndrome (neurofi-

bromatosis) c, Both d. Neither

27, Caf~ au lait macules usually appear prior to other changes

28. Clinical appearance of individual caf6 au lait macules is diagnostic

29, Giant melanosomes in adults 30, Crowe's sign 31, Lisch nodules

Dh'ections for questions 32 and 3.?: Indicate cor- rect answe/~s'. All, some, or none of the choices may be correct, 32, Major characteristics of the multiple lentigines

("LEOPARD") syndrome include a, multiple lentigines located primarily on the ex-

tremities b, ocular hypertelorism c. pulmonary fibrosis d, electrocardiographic abnormalities, especially

conduction defects e, neurosensory deafness

33, Endocrinopathies that may be present in Albright's syndrome include a, precocious puberty in female patients b, acromegaly c, hyperthyroidism d. hyperparathyroidism e, Cushing's syndrome