SM_266a: Autoimmune Systemic Diseases

Question 1

Vitiligo is ____

Answer 1

Vitiligo is an acquired autoimmune disorder of the skin in which destruction of melanocytes leads to well-demarcated patches of depigmentation

(often devastating for those affected)

Question 2

Vitiligo is characterized by ____

Answer 2

Vitiligo is characterized by well demarcated areas of pigmentation

Question 3

Vitiligo may be associated with ____

Answer 3

Vitiligo may be associated with other autoimmune conditions such as thyroid dysfunction, alopecia areata, and diabetes mellitus

(consider checking TSH)

Question 4

Consider checking ____ in patients with vitiligo

Answer 4

Consider checking TSH in patients with vitiligo

Question 5

Vitiligo is treated with ____, ____, ____, ____, or ____

Answer 5

Vitiligo is treated with phototherapy (light therapy), topical steroids, topical immunosuppressants (e.g. Tacrolimus), laser, or depigmentation

Question 6

Alopecia areata is ____

Answer 6

Alopecia areata is an acquired autoimmune disorder targeting hair follicle structures resulting in recurrent patches of non-scarring alopecia

(often associated with significant psychosocial and emotional distress)

Question 7

Alopecia areata most commonly presents as ____

Answer 7

Alopecia areata most commonly presents as well demarcated round patches of alopecia

Question 8

Alopecia areata has possible association with ____

Answer 8

Alopecia areata has possible association with other autoimmune conditions such as thyroid disease, vitiligo, and diabetes

(check TSH)

Question 9

Check ____ in patients with alopecia areata

Answer 9

Check TSH in patients with alopecia areata

Question 10

Acrodermatitis enteropathica is a ____ deficiency and presents as ____

Answer 10

Acrodermatitis enteropathica is a zinc deficiency and presents as perioral / perianal erosive scaly rash

Question 11

Acrodermatitis enteropathica appears in ____ if genetic

Answer 11

Acrodermatitis enteropathica appears in babies days to weeks after birth or after weaning from breast feeding if genetic

Question 12

Scurvy is ____ deficiency and presents as ____

Answer 12

Scurvy is ascorbic acid deficiency and presents as bleeding, gingivae, petechiae, ecchymoses, follicular hyperkeratosis, and corkscrew hairs

Question 13

Lynch syndrome (hereditary non-polyposis colorectal cancer) is associated with ____, ____, ____, and ____ carcinomas and ____ adenoma/carcinoma in Muir-Torre

Answer 13

Lynch syndrome (hereditary non-polyposis colorectal cancer) is associated with colorectal, endometrial, ovarian, and gatric carcinomas and sebaceous adenoma/carcinoma in Muir-Torre

Question 14

Sweet’s syndrome (acute febrile neutrophilic dermatosis) is characterized by ____

Answer 14

Sweet’s syndrome (acute febrile neutrophilic dermatosis) is characterized by neutrophilic infiltrate

Question 15

Sweet’s syndrome (acute febrile neutrophilic dermatosis) involves ____ and ____ and is treated with ____

Answer 15

Sweet’s syndrome (acute febrile neutrophilic dermatosis) involves fever and general malaise and is treated with oral prednisone

Question 16

Sweet’s syndrome (acute febrile neutrophilic dermatosis) is associated with ____, ____, ____ and ____

Answer 16

Sweet’s syndrome (acute febrile neutrophilic dermatosis) is associated with malignancy (especially AML), inflammatory bowel disease, drugs (G-CSF), pregnancy, and infections (Streptococcus URI, Yersiniosis)

Question 17

Cirrhosis involves ____ and ____

Answer 17

Cirrhosis involves dilated abdominal veins (caput medusae) and spider angiomas

Question 18

Lichen planus is an ____ that occurs after ____ and presents as ____

Answer 18

Lichen planus is an idiopathic inflammatory skin disease that occurs after Hepatitis C presents as flat topped violaceous papules

Question 19

Renal disease includes ____ and ____

Answer 19

Renal disease includes pruritis and calciphylaxis

Question 20

Describe pruritis

Answer 20

Pruritis

• Possibly related to increase in tissue mast cells
• Localized or generalized
• Worse with hemodialysis
• Treatment: emollients, UV light, gabapentin, anti-histamines

(renal disease)

Question 21

Calciphylaxis is ____ that presents as ____

Answer 21

Calciphylaxis is progressive vascular calcification of the skin and soft tissue that presents as violaceous reticulated patches

Question 22

Calciphylaxis progresses from ___ to ___ to ___

Answer 22

Calciphylaxis progresses from bullae to tissue necrosis to ulceration

Question 23

Calciphylaxis is ____ and affects ____ areas of the body

Answer 23

Calciphylaxis is extremely painful and affects painful areas of the body

Question 24

Describe sarcoidosis

Answer 24

Sarcoidosis

• Lupus pernio: papulonodules and plaques involving areas affected by cold - nose, ears, cheeks
• Lofgren’s syndrome: erythema nodosum + hilar adenopathy + fever + polyarthritis + acute iritis
• Can affect virtually any organ system

Question 25

Lupus pernio occurs in \_\_\_\_, presents as \_\_\_\_, and has a strong association with ____ disease

Answer 25

Lupus pernio occurs in sarcoidosis, presents as papulonodules and plaques involving areas affected by cold (nose, ears, cheeks), and has a strong association with chronic lung and upper respiratory tract disease

Question 26

Lofgren's syndrome occurs in ____ and involves \_\_\_\_, \_\_\_\_, \_\_\_\_, \_\_\_\_, and \_\_\_\_

Answer 26

Lofgren's syndrome occurs in sarcoidosis and involves erythema nodosum, hilar adenopathy, fever, polyarthritis, and acute iritis

Question 27

Addison's disease is \_\_\_\_

Answer 27

Addison's disease is diffuse hyperpigmentation from activity of melanocyte-stimulating hormone (endocrine disorder)

Question 28

Diabetes mellitus manifests as ____ on the skin which is \_\_\_\_

Answer 28

Diabetes mellitus manifests as acanthosis nigricans on the skin which is velvety, hyperpigmented plaques in flexural surfaces (most commonly occurs in overweight persons with insulin resistance)

Question 29

Acanthosis nigricans in diabetes mellitus most commonly occurs in ____ people with \_\_\_\_

Answer 29

Acanthosis nigricans in diabetes mellitus most commonly occurs in overweight people with insulin resistance (velvety, hyperpigmented plaques in flexural surfaces)

Question 30

Describe pyoderma gangrenosum

Answer 30

Pyoderma gangrenosum * Ulcer with necrotic, undermined borders * Painful, typically lower extremities * Associated with inflammatory bowel disease, arthritis, monoclonal gammopathy, CML * Treatment is systemic steroids, TNF inhibitors, rituximab

Question 31

Pyoderma gangrenosum is ____ that is ____ and typically occurs on \_\_\_\_

Answer 31

Pyoderma gangrenosum is ulcer with necrotic undermined borders that is painful and typically occurs on lower extremities

Question 32

Pyoderma gangrenosum is associated with \_\_\_\_, \_\_\_\_, \_\_\_\_, and \_\_\_\_

Answer 32

Pyoderma gangrenosum is associated with inflammatory bowel disease, arthritis, monoclonal gammopathy, and CML

Question 33

Pyoderma gangrenosum is treated with \_\_\_\_, \_\_\_\_, and \_\_\_\_

Answer 33

Pyoderma gangrenosum is treated with systemic steroids, TNF inhibitors, and rituximab

Question 34

Dermatitis herptiformis is ____ manifestation of ____ and involves \_\_\_\_

Answer 34

Dermatitis herptiformis is cutaneous manifestation of celiac disease (gluten sensitive enteropathy) and involves intense pruritis (treatment is gluten free diet and dapsone)

Question 35

Dermatitis herpetiformis is treated with ____ and \_\_\_\_

Answer 35

Dermatitis herpetiformis is treated with gluten free diet and dapsone