Ch10 Syndromes

Question 1

what are the 4 syndromes/skin disorders associated with papillomatosis?

Answer 1

nevus unius lateris, acanthosis nigricans, focal dermal hypoplasia (Goltz-Gorlin) syndrome, and Down syndrome

Question 2

What syndrome is associated with visceral malignancies, sebaceous adenomas and carcinomas, and keratoacanthomas? Whats the inheritance pattern?

Answer 2

Muir-Torre syndrome…AD

Question 3

What 3 conditions/syndromes are associated with multiple lentigo simplex?

Answer 3

lentiginosis profusa, Peutz-Jeghers syndrome, and LEOPARD

L – lentigines (dark freckles) on the head and neck
E – electrocardiogram (EKG) abnormalities
O – ocular hyperteleorism (wide-spacing of the eyes)
P – pulmonary stenosis
A – abnormal genitalia
R – retardation of growth
D – deafness (sensorineural type)

Question 4

What syndrome is associated with giant or multiple congenital nevi in conjunction with melanotic neoplasms of the CNS like leptomeningeal melanosis and melanoma

Answer 4

neurocutaneous melanosis

Question 5

What 5 syndromes/conditions are associated with being risk factors for the development of actinic keratoses?

Answer 5

albinism, Rothmund-Thompson syndrome, Cockayne syndrome, xeroderma pigmentosum, and Bloom syndrome

Cockayne syndrome: neurodegenerative disorder characterized by growth failure, impaired development of the nervous system, abnormal sensitivity to sunlight (photosensitivity), eye disorders and premature aging.

Question 6

What are the two heritable conditions associated with multiple keratoacanthomas?

Answer 6

Ferguson-Smith syndrome and Witten-Zak syndrome

Question 7

What syndrome is associated with nodular lesions (KA’s)

and primarily affects patients of Scottish descent?

Answer 7

Ferguson-Smith syndrome

Question 8

What syndrome is associated with a mixture of variably sized keratoachanthomas?

Answer 8

Witten-Zak syndrome

Question 9

What syndrome manifests later in life as hundreds or thousands of small papules of the skin and upper digestive tract and may be associated with internal
malignancy?

Answer 9

Grzybowski syndrome

Question 10

What are the two names for the iron deficiency syndrome that associated with an elevated risk for squamous cell carcinoma of the esophagus, oropharynx, and posterior mouth?

Answer 10

Plummer-Vinson or Paterson-Kelly syndrome

Question 11

Several genodermatoses are associated with basal

cell carcinoma, including these 7

Answer 11

1. nevoid basal cell carcinoma
   syndrome 2. xeroderma pigmentosum 3. albinism 4. Rasmussen syndrome 5. Rombo syndrome 6. Bazex-Christol-Dupré syndrome, and 7. Dowling-Meara
   subtype of epidermolysis bullosa simplex