Cutaneous Manifestations of Systemic Diseases Flashcards
How would you describe the lesions?
What is the diagnosis?
Numerous well-demarcated pink plaques with some overlying scale on the buttocks and lower back
Psoriasis
Psoriasis
●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
Psoriatic Arthritis
●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)
Clinical Presentation- Psoriasis
- 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
plaque psoriasis
Guttate Psoriasis
•Children and adolescents
- Frequently preceded by a URI
- >50%, an elevated anti-streptolysin O titer is found, indicating a recent streptococcal infection
What type of psoriasis is this?
Pustular Psoriasis
If you culture a pustule, what will it grow?
A.Staphylococcus aureusB.Streptococcus pyogenesC.Pseudomonas aeruginosaD.None of the above, the pustules are sterile
A.None of the above, the pustules are sterile
Special Locations
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.
Psoriasis and Health Inequities
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)
Treatment-Psoriasis
•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
How would you describe the lesions?
Violaceous papules and plaques with white scale on the dorsal foot.
Lichen Planus
Lichen Planus
●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
Wickham Striae
a network of fine white lines called “Wickham striae”-
in lichen planae
Lichen Planus Nail Involvement
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.
A large annular plaque with smaller oval-shaped, scaly papules and plaques on the back
Pityriasis Rosea
Pityriasis Rosea
•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
Pityriasis Rosea
Hyperpigmented, velvety plaques on the posterior neck
Acanthosis Nigricans
What labs do you want to check?- acanthuses nigoracans
A.LFTsB.Vitamin DC.TSHD.Hemoglobin A1CE.None of the above
A.Hemoglobin A1C
Acanthosis Nigricans- types
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
Malignancy Associated- acanthuses nigricans
Atypical (palmar, perioral, or mucosal) distributions or acute-onset
Oval-shaped ulcer with a violaceous border and overlying eschar
Pyoderma Gangrenosum
Pyoderma Gangrenosum
•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
Pyoderma Gangrenosum- clinical features
•Painful•Frequently occurs on lower extremities, shin•Purulent base with an irregular, undermined and overhanging, gunmetal-colored border which extends centrifugally
Treatment - Pyoderma grangrenosum
Do Not Debride
•Commonly misdiagnosed as a skin infection•Displays pathergy: minor skin trauma (e.g., needlestick) may initiate and worsen lesions
Flat-topped yellow papules on the medial and lower eyelids
Xanthelasma
Xanthelasma
•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
Other Plane Xanthomas
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
Depigmented patches on the dorsal hands
Vitiligo
Vitiligo
•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
Focal Vitiligo
•Confined to a limited area•Most responsive to medical management
most responsive to medical management, with the exception of the segmental subtype.
Segmental Vitiligo
•Quasi-dermatomal distribution•Usually unilateral•Decreased progression after 2 years•Usually more resistant to medical treatment•Most responsive to surgical treatment
Generalized Vitiligo
•Widespread involvement•Often symmetric
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
Treatment- vitiligo
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
Depigmentation
•Removal of remaining skin color•Patients with either >50% BSA or resistant involvement of cosmetically sensitive areas•Usually irreversible
Psychosocial Challenges
•Quality of life impairment•Psychological Disorders (anxiety and depression) in 30% of patients with vitiligo•Consider behavioral health referral
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
Erythematous, tender nodules bilaterally on the shins and dorsal feet
Erythema Nodosum
Erythema Nodosum
•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
Corkscrew hairs and perifollicular hemorrhage, gingival erosions
Scurvy
Scurvy
•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
What is the diagnosis?
What is the associated systemic disorder?
•Lichen Planus
HCV
•Xanthelasma
•Hyperlipidemia
What is the diagnosis?
•Psoriasis-
What is the most commonly associated systemic disorder?
•Arthritis
What is the diagnosis?
•Vitiligo-
What is the most commonly associated systemic disorder?
•Thyroid Disease
What is the diagnosis?
•Scurvy-
What is the associated systemic disorder?
•Vitamin C Deficiency
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
What is the diagnosis?
•Pityriasis rosea-
What viruses have been proposed to be associated with it?
•HHV-7, less so HHV-6
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
A.Decreased number of melanocytes in vitiligo; normal number of melanocytes in albinism
