Cutaneous Manifestations of Systemic Diseases

Question 1

How would you describe the lesions?
What is the diagnosis?

Answer 1

Numerous well-demarcated pink plaques with some overlying scale on the buttocks and lower back

Psoriasis

Question 2

Psoriasis

Answer 2

●Immune-mediated, polygenic disease that may be elicited by environmental triggers (e.g., trauma, infections, medications, psychological stress)●Systemic disease: psoriatic arthritis, metabolic syndrome and cardiovascular disease●Significant impact on quality of life

Question 3

Psoriatic Arthritis

Answer 3

●Most commonly associated systemic disease, seen in up to 20-30% of patient ●Destructive●May precede, coincide or develop after skin lesions

●Key characteristics●Joint stiffness > 30 minutes in the morning (after waking) or after prolonged inactivity●Improves with activity (vs osteoarthritis / DJD, which typically worsens with activity)

Question 4

Clinical Presentation- Psoriasis

Answer 4

• Sharply demarcated erythematous papules and plaques with scale (hallmarks: erythema, thickening, scale)•Occasionally pustules can be seen (generalized pustular psoriasis, palmoplantar pustulosis)
• Lesions are sometimes surrounded by a pale blanching ring•Woronoff’s ring
• Pinpoint bleeding seen when surface scale is removed
• Auspitz sign

Question 5

Answer 5

plaque psoriasis

Question 6

Answer 6

Guttate Psoriasis
•Children and adolescents

• Frequently preceded by a URI
• >50%, an elevated anti-streptolysin O titer is found, indicating a recent streptococcal infection

Question 7

What type of psoriasis is this?

Answer 7

Pustular Psoriasis

Question 8

If you culture a pustule, what will it grow?

A.Staphylococcus aureusB.Streptococcus pyogenesC.Pseudomonas aeruginosaD.None of the above, the pustules are sterile

Answer 8

A.None of the above, the pustules are sterile

Question 9

Special Locations

Answer 9

Nail plate pitting, distal onycholysis, oil drop changes, and subungual and proximal hyperkeratosis are seen. There is also proximal nail-fold inflammation with loss of the cuticle, especially of the forefingers. Patients with nail involvement appear to have an increased incidence of psoriatic arthritis.

Shiny erythematous plaques of the inframammary folds that lack scale.

Question 10

Psoriasis and Health Inequities

Answer 10

Among historically marginalized racial/ethnic groups, particularly Black patients

•May be more severe•Quality-of-life burden appears to be greater, independent of any objective disease severity differences•

Barriers to care

•Less likely to see a dermatologist•Dermatologists less familiar with presentation•Violaceous, grey or hyperpigmented•Less likely to be prescribed a biologic

red color is often not quite as apparent.

purple or violaceous or can simply look hyperpigmented, the latter of which can be mistaken as inactive disease or post-inflammatory hyperpigmentation (PIH)

Question 11

Treatment-Psoriasis

Answer 11

•Limited skin disease ​•Topicals (e.g., corticosteroids, vitamin D analogues, retinoids, anthralin, tar)

​•More severe skin disease•Systemic therapies•Biologics (target the part of the immune system that is overactive in psoriasis)•Apremilast, methotrexate, acitretin, cyclosporine,•Phototherapy ​

Question 12

How would you describe the lesions?

Answer 12

Violaceous papules and plaques with white scale on the dorsal foot.

Lichen Planus

Question 13

Lichen Planus

Answer 13

●Unclear etiology​●PPPP = Purple, polygonal, papules and plaques​, mucosa and skin●Some cases are drug-induced ​○ACE inhibitors, beta-blockers, thiazide diuretics, antimalarials, penicillamine, gold salts ​●Described in association with hepatitis C in certain geographical areas (Japan and Mediterranean regions)●Cutaneous lesions typically resolve within 6 months (> 50%) to 18 months (85%).​●Wickham striae in oral lesions​

Question 14

Answer 14

Wickham Striae

a network of fine white lines called “Wickham striae”-

in lichen planae

Question 15

Answer 15

Lichen Planus Nail Involvement

Question 16

A 24-year-old M presents with this eruption. It started a few weeks ago as one large spot on his belly, and then he developed numerous, mildly itchy, additional spots. They are itchy. He reports that he had a previous cold, but is otherwise healthy.

Answer 16

A large annular plaque with smaller oval-shaped, scaly papules and plaques on the back

Pityriasis Rosea

Question 17

Pityriasis Rosea

Answer 17

•Primarily adolescents and young adults, favors the trunk and proximal extremities•Precise cause is unknown; viral etiology is frequently proposed; HHV-7 and, less so, on HHV-6•“Herald patch” followed by lesions that are oval shaped, scaly (collarette) and oriented along skin cleavage lines (Christmas tree distribution)•Self-resolving in 6-8 weeks

They are parallel to the natural orientation of collagen fibers in the dermis, as well as the underlying muscle fibers.

herald patch” because it heralds the onset of the disease.

Question 18

Answer 18

Pityriasis Rosea

Question 19

Answer 19

Hyperpigmented, velvety plaques on the posterior neck

Acanthosis Nigricans

Question 20

What labs do you want to check?- acanthuses nigoracans

A.LFTsB.Vitamin DC.TSHD.Hemoglobin A1CE.None of the above

Answer 20

A.Hemoglobin A1C

Question 21

Acanthosis Nigricans- types

Answer 21

Several Types

•Obesity-associated: most common; associated with DM, insulin resistance, high BMI, metabolic syndrome and PCOS•Malignancy-associated: gastric cancer•Drug-induced: niacin (nicotinic acid)•Others include OCPs, corticosteroids, diethylstilbestrol, heroin, fusidic acid, methyltestosterone, protease inhibitors, and folate.•Syndromic: HAIR-AN (hyperandrogenism, insulin resistance, and acanthosis nigricans) syndrome and acromegaly

Question 22

Malignancy Associated- acanthuses nigricans

Answer 22

Atypical (palmar, perioral, or mucosal) distributions or acute-onset

Question 23

Answer 23

Oval-shaped ulcer with a violaceous border and overlying eschar

Pyoderma Gangrenosum

Question 24

Pyoderma Gangrenosum

Answer 24

•Chronic, inflammatory, ulcerative skin disease•Uncertain etiology•Associated illnesses include inflammatory bowel disease, arthritis, monoclonal gammopathies, and other hematologic disorders•Rx: systemic steroids, though no uniformly effective therapy

Question 25

Pyoderma Gangrenosum- clinical features

Answer 25

•Painful•Frequently occurs on lower extremities, shin•Purulent base with an irregular, undermined and overhanging, gunmetal-colored border which extends centrifugally

Question 26

Treatment - Pyoderma grangrenosum

Answer 26

Do Not Debride

•Commonly misdiagnosed as a skin infection•Displays pathergy: minor skin trauma (e.g., needlestick) may initiate and worsen lesions

Question 27

Answer 27

Flat-topped yellow papules on the medial and lower eyelids

Xanthelasma

Question 28

Xanthelasma

Answer 28

•Plane xanthoma•Yellowish macules or slightly elevated papules or plaques on the eyelids, upper medial lids predominantly•Onset fourth or fifth decade of life•Asymptomatic•Approximately 50% of patients have a lipid disorder

Question 29

Other Plane Xanthomas

Answer 29

Site provides clue to underlying disease

•Intertriginous (antecubital fossae/finger web spaces):•Homozygous familial hypercholesterolemia•Palmar creases•Dysbetalipoproteinemia (increase triglycerides and cholesterol)•Plane xanthomas in a normolipemic patient•Check for underlying monoclonal gammopathy

Question 30

Answer 30

Depigmented patches on the dorsal hands

Vitiligo

Question 31

Vitiligo

Answer 31

•Multifactorial disorder involving the autoimmune destruction of melanocytes•No racial or gender predilection•~ 20% have at least 1 comorbid autoimmune disease•Autoimmune thyroid disease: ~15.1%•Check baseline TSH, annual recheck vs symptom screen at follow-up•Depigmented macules and patches on the body•Several subtypes: focal, segmental, generalized, acrofacial

Less common associations: AA, IBD, pernicious anemia, DLE, Guillain-Barre, linear morphea, myasthenia gravis, Sjogren

Question 32

Focal Vitiligo

Answer 32

•Confined to a limited area•Most responsive to medical management

most responsive to medical management, with the exception of the segmental subtype.

Question 33

Segmental Vitiligo

Answer 33

•Quasi-dermatomal distribution•Usually unilateral•Decreased progression after 2 years•Usually more resistant to medical treatment•Most responsive to surgical treatment

Question 34

Generalized Vitiligo

Answer 34

•Widespread involvement•Often symmetric

Question 35

Answer 35

Acrofacial Vitiligo

•Cases in which there is involvement of the fingers/toes and perioral regions•Lesions on ANY part of the body are less responsive to all treatments

Question 36

Treatment- vitiligo

Answer 36

Goal: halt progression and induce repigmentation

•First line: topical steroids and topical calcineurin inhibitors•Systemic steroids for rapidly progressing disease•Phototherapy•Skin grafts•Cover-up makeup (Dermablend)•Depigmenting agents (Monobenzylether of hydroquinone)•JAK inhibitors

Question 37

Depigmentation

Answer 37

•Removal of remaining skin color•Patients with either >50% BSA or resistant involvement of cosmetically sensitive areas•Usually irreversible

Question 38

Psychosocial Challenges

Answer 38

•Quality of life impairment•Psychological Disorders (anxiety and depression) in 30% of patients with vitiligo•Consider behavioral health referral

Question 39

Answer 39

Oculocutaneous Albinism

•Genetic disorder, OCA gene•Normal number of melanocytes•Reduced or absence of melanin from abnormalities in tyrosinase or enzymes involved in the biosynthesis and distribution of melanin

Question 40

Answer 40

Erythematous, tender nodules bilaterally on the shins and dorsal feet

Erythema Nodosum

Question 41

Erythema Nodosum

Answer 41

•Septal panniculitis (inflammation of the subcutaneous fat)•Tender, erythematous, subcutaneous nodules•Usually distributed symmetrically on the shins•May be accompanied by fever, arthralgias and malaise•Strep infections most common cause in children •Sarcoid, IBD and medications (OCPs) more commonly implicated in adults•Treatment: spontaneous resolution in some, rest, leg elevation, NSAIDS, identify and eliminate precipitating trigger

Question 42

Answer 42

Corkscrew hairs and perifollicular hemorrhage, gingival erosions

Scurvy

Question 43

Scurvy

Answer 43

•Acquired and prolonged vitamin C deficiency•Essential cofactor in cross-linking and stabilization of collagen•Deficiency causes impaired collagen synthesis à poor wound healing, capillary fragility, and bone abnormalities•Gingival bleeding, perifollicular petechiae, corkscrew hairs•Risk factors: alcohol use (poor dietary intake and decreased absorption), restrictive or fad diets, malabsorption due to GI disease

Question 44

What is the diagnosis?

What is the associated systemic disorder?

Answer 44

•Lichen Planus

HCV

Question 45

Answer 45

•Xanthelasma

•Hyperlipidemia

Question 46

Answer 46

What is the diagnosis?

•Psoriasis-

What is the most commonly associated systemic disorder?

•Arthritis

Question 47

Answer 47

What is the diagnosis?

•Vitiligo-

What is the most commonly associated systemic disorder?

•Thyroid Disease

Question 48

Answer 48

What is the diagnosis?

•Scurvy-

What is the associated systemic disorder?

•Vitamin C Deficiency

Question 49

Answer 49

What is the diagnosis?

•Acanthosis nigricans

For the most common type (obesity associated), what are the associated systemic disorders?

•Diabetes, insulin resistance, high BMI, metabolic syndrome and PCOS

For the malignant type, what is the most commonly associated malignancy?

•Gastric cancer

Question 50

Answer 50

What is the diagnosis?

•Pityriasis rosea-

What viruses have been proposed to be associated with it?

•HHV-7, less so HHV-6

Question 51

Which of the following is true?

A.Normal number of melanocytes in vitiligo; normal number of melanocytes in albinismB.Decreased number of melanocytes in vitiligo; decreased number of melanocytes in albinismC.Decreased number of melanocytes in vitiligo; normal number of melanocytes in albinismD.Normal number of melanocytes in vitiligo, decreased number of melanocytes in albinismE.Increased number of melanocytes in vitiligo, increased number of melanocytes in albinism

Answer 51

A.Decreased number of melanocytes in vitiligo; normal number of melanocytes in albinism