Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of...

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Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology

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Page 1: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Cutaneous Manifestations of Systemic Disease

Holly Edmonds, MDChief Resident

Department of Dermatology

Page 2: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Brief Review of terminology…

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Zits???

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Red Rash???

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“Nasty… just call the derm service and get a consult!”

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You have to learn the language!

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Description of a skin lesion:

Type

Shape

Arrangment

Distribution

OH!!! DATS what that rash is!!!

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TYPE

Primary lesion

Secondary lesion

Color

Palpation

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MACULE/PATCH

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PLAQUE

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PAPULE

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NODULE

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VESICLE/BULLA

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PUSTULE

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WHEAL

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SCALE

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CRUST

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LICHENIFICATION

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EROSION

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ULCER

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ATROPHY

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EXCORIATION

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FISSURE

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SCAR

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Color

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Color

http://dermnetnz.org/common/image.php?path=/lesions/img/blue3.jpg
http://dermnetnz.org/common/image.php?path=/dermal-infiltrative/img/s/xanthelas1.jpg
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Color

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Palpation

Consistency – Firm, Soft, Fluctuant, Boardlike

Temperature deviation (hot or cold)

Mobility

Presence of tenderness

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Margination

Ill-defined

vs.

Well-defined

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SHAPE

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SHAPE

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SHAPE

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SHAPE

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SHAPE

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ARRANGEMENT

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ARRANGEMENT

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ARRANGEMENT

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ARRANGEMENT

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ARRANGEMENT

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DISTRIBUTION

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DISTRIBUTION

http://www.dermnet.com/image.cfm?passedArrayIndex=9&moduleID=5&moduleGroupID=66
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DISTRIBUTION

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DISTRIBUTION

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DISTRIBUTION

Page 62: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Cutaneous Manifestations

Diabetes Mellitus Renal DiseaseGastrointestinal disordersRheumatologic diseaseHepatitis CThyroid diseaseParaneoplastic diseaseNutritional disease

Page 63: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Diabetes Mellitus

Acanthosis Nigricans

Diabetic Dermopathy

Bullosis Diabeticorum

Necrobiosis Lipoidica

Diabetic Foot Ulcers

Page 64: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Acanthosis Nigricans

African Americans and Hispanics > Caucasians

Associated with obesity, insulin resistance

Hyperpigmented velvety plaques of the flexures

Genetic sensitivity of the skin to hyperinsulinemia

Malignant form a/w gastric ACA

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Acanthosis Nigricans

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Acanthosis Nigricans

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Diabetic Dermopathy

AKA “shin spots” or pigmented pretibial papules

Most common cutaneous manifestation of diabetes

Benign asymptomatic red brown macules on shins

No treatment needed

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Diabetic Dermopathy

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Bullosis Diabeticorum

Rapid onset painless, tense blisters on hands and feet

Trauma and microvascular disease may play a role

Spontaneous healing in 2-5 weeks

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Bullosis Diabeticorum

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Necrobiosis Lipoidica

20-35% of patients with NLD have diabetes, only about 1-3% of diabetics have NLDStart as red-brown papules and progress to well defined yellow-brown atrophic plaques with irregular violaceous borders and telangiectasias. Shins #1 site. Ulceration in 35%. Glucose control will not clear NLD

Page 72: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Necrobiosis Lipoidica Diabeticorum

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Diabetic Eruptive Xanthomas

Seen in uncontrolled diabetes, hypertriglyceridemia

Sudden crops on firm, non-tender yellow papules with a red rim on extensors

Control glucose and lipid reduction will reduce lesions

Page 74: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Diabetic Eruptive Xanthomas

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Diabetic Neurotropic Ulcers

Peripheral neuropathy leads to unnoticed trauma

Vascular complications may lead to ulcers and complicate ulcer healing

Risk of amputation goes up 8x once these develop

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Diabetic Neurotropic Ulcers

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Renal disease

Pruritis

Perforating dermatosis

Calciphylaxis

Nephrogenic Fibrosing Dermopathy

Page 78: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Pruritis

Most common cutaneous manifestation of renal disease

Seen in both peritoneal and hemodialysis patients

Unknown mechanism, unsatisfactory therapy- UVB helps the most

Page 79: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Pruritis

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Perforating Disorder

Acquired Perforating Dermatosis of ESRD

Umbilicated papules/nodules with central hyperkeratotic core

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Perforating dermatosis

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Perforating dermatosis

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Calciphylaxis

Painful purpuric plaques and retiform purpura

More proximal lesions = poorer prognosis

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Calciphylaxis

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Nephrogenic Fibrosing Dermopathy

Woody, indurated plaques with peau d’orange appearance

Usually spares the face, palms, soles

Associated with gadolinium contrast for MRIs

Page 86: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Nephrogenic Fibrosing Dermopathy

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Gastrointestinal disorders

Henoch Schonlein Purpura

Dermatitis Herpetiformis

Inflammatory Bowel Disease

Page 88: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Henoch Schonlein Purpura

Palpable purpura, urticaria, necrotic ulcers on buttocks, distal legs

Symmetric

IgA vasculitis

GI symptoms + arthritis, long term concern for hypertension and renal involvement

Usually under 20, following an URI

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HSP

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HSP

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Dermatitis Herpetiformis

Symmetric, grouped vesicles on extensors

Very pruritic!

All patients have gluten-sensitive enteropathy, only 20% symptomatic

Associated with HLA-DQ2, Hashiomoto’s thyroiditis, lymphoma, IDDM

Cutaneous findings are due to autoantibodies to epidermal transglutaminase

Treatment- rapidly responsive to dapsone

Page 92: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Dermatitis Herpetiformis

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Inflammatory Bowel Disease

Crohn’s and Ulcerative Colitis

Page 94: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Oral Crohn’s

• Linear ulcerations, cobblestoned oral

mucosa

• UC may have aphthous ulcers that develop as

IBD flares

Page 95: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Metastatic Crohn’s• See fissures and

fistulas with Crohn’s

• Metastatic Crohn’s are

nodules, plaques and ulcerations

usually in intertriginous

areas which can mimic erythema

nodosum

Page 96: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Erythema Nodosum• Tender red nodules on

anterior lower legs, precedes or occurs with

IBD flares, UC more common

• Most EN is idiopathic, also can be related to oral

contraceptives or abx, preceding strep or mycobacterial infxn

Page 97: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Pyoderma Gangrenosum

• More common in UC• Papules, pustules, hemorrhagic blisters enlarge and ulcerate with dusky

undermined edges• Frequently on legs or around stoma sites• Treat with steroids, often gets better as IBD gets better

Page 98: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Rheumatologic Disorders

Lupus Erythematosis

Dermatomyositis

Reiter’s Disease

Page 99: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Lupus Erythematosis

Classification:– Systemic Cutaneous Lupus (SLE)– Subacute Cutaneous Lupus (SCLE)– Discoid Lupus (DLE)– Neonatal Lupus

Page 100: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

SLE

+ANA

+Sm and dsDNA

Butterfly Rash

Poikiloderma

Photodistrubited erythematous, papular scaling eruption sparing knuckles.

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SLE

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SLE

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SLE

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Dermatomyositis

Poikiloderma favoring scalp, periocular (Heliotrope rash), and extensor skin sites Nailfold telangiectasiasGottron’s papules

Page 105: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Dermatomyositis

http://dermnetnz.org/common/image.php?path=/immune/img/dm3.jpg
Page 106: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Dermatomyositis(periungal telangiectasias, gottrons

papules)

Page 107: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Reiter’s Disease

Urethritis, arthritis, ocular findings, and oral ulcers in addition to psoriasiform skin lesions. Keratoderma blenorrhagicum (feet)Balanitis circinata (penis)

Page 108: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Reiter’s DiseaseKeratoderma Blenorrhagicum

Page 109: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Reiter’s Disease(balanitis circinata)

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Hepatitis C virus

Porphyria Cutanea Tarda

Lichen Planus

Pruritis

Page 111: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Porphyria Cutanea Tarda

Vesicles and bullae on sun-exposed areas, scarring with milia

Hypertrichosis

Fragile skin with sclerodermoid changes

Page 112: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

PCT

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PCT(hypertrichosis)

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Lichen Planus

Purple, pruritic, polygonal papules

Koebner phenomenon

Wickham’s striae

50% with mucosal involvement

Page 115: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Lichen Planus

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Mucosal Lichen Planus

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Pruritis

Excoriations, lichenification, and prurigo nodularis

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Prurigo Nodularis

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Pruritis

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Thyroid disease

Graves disease

Hyperthyroidism

Hypothyroidism

Page 121: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Graves Disease

Thyroid dermopathy (pretibial myxedema)

Symmetric, non-pitting yellow-brown waxy papules/plaques

Due to increased hyaluronic acis in dermis

Page 122: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Thyroid Dermopathy

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Hyperthyroidism

Warm, moist skin

Flushing, palmar erythema

Associated with reversible alopecia and vitiligo

Page 124: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Vitiligo

Page 125: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Hypothyroidism

Dry, cool skin

Generalized myxedema

Yellow hue from carotenemia

Purpura from delayed wound healing

Alopecia, madarosis

Page 126: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Carotenemia

Page 127: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Paraneoplastic Disorders

Acanthosis Nigricans

Dermatomyositis

Cushing’s Disease

Sign of Lesser-Trelat

Paraneoplastic Pemphigus

Hypertrichosis Lanuginosa

Page 128: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Acanthosis Nigricans

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Dermatomyositis

http://dermnetnz.org/common/image.php?path=/immune/img/dm3.jpg
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Cushing’s syndrome(buffalo hump and striae)

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Sign of Lesser-Trelat

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Paraneoplastic Pemphigus

http://162.129.70.33/images/pemphigus_2_010526.jpg
Page 133: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Erythema Gyratum Repens(associated with cancers above diaphragm)

Page 134: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Hypertricosis Lanuginosa Acquisita

Page 135: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Nutritional Disorders

1. Marasmus

2. Kwashiorkor

3. Pellagra

4. Scurvy

5. Zinc deficiency

Page 136: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Marasmus

Marasmus = protein/calorie malnutrition.

Cutaneous Manifestations:– Emaciation with thin, lax, and wrinkled skin.– Fine scaling with hyperpigmentation.– Follicular hyperkeratosis– Purpura– Thin hair and nails.

Page 137: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Marasmus

Page 138: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Marasmus

Page 139: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Kwashiorkor

Decreased protein intake

Dyschromia (irregular pigment)

Hypopigmentation.

Superficial desquamation with areas of erosion (flaky paint)

Petechia/purpura

Thin hair/nails

Page 140: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Kwashiorkor

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Kwashiorkor

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Pellagra

Niacin deficiency (Vit B3)

Triad of dermatitis, diarrhea, dementia

Photosensitive eruption around neck known as “Casal’s necklace”

Page 143: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Pellagra

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Pellagra

Casal’s neckace

Page 145: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Scurvy

Vitamin C deficiency

Follicular hyperkeratosis with corkscrew hairs

Perifollicular hemorrhage

Gingival hypertrophy with erosive bleeding gums.

Page 146: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Scurvy

Page 147: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Zinc Deficiency

Acquired

-deficient intake, high fiber intake, malabsorption

Inherited (acrodermatitis enteropathica) zinc deficiency

-defect in intestinal absorption of zinc

Dermatitis, diarrhea, alopecia

Periorificial and acral distribution

Page 148: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Acrodermatitis Enteropathica

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Acrodermatitis Enteropathica

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A couple of randoms you should know…

Page 151: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Sarcoidosis

Multisystem granulomatous disease

Skin affected in 20-35%

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Neurofibromatosis

Von Reckinghausen’s disease

Autosomal dominant

Neurofibromin gene, Chr 17

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http://dermnetnz.org/common/image.php?path=/systemic/img/nf4.jpg
Page 158: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Tuberous Sclerosis

Autosomal Dominant

Hamartin and Tuberin (TSC 1 and 2)

MR, Seizures (variable)

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QUIZ TIME!!!

Page 166: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Very itchy.

Page 167: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Lichen Planus (HCV)

Page 168: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Anterior lower leg

Page 169: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Necrobiosis Lipoidica (DM)

Page 170: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

?

Page 171: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Pyoderma Gangrenosum (IBD)

Page 172: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

?

Page 173: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Porphyria Cutanea Tarda

Page 174: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

?

Page 175: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

SLE

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?

Page 177: Cutaneous Manifestations of Systemic Disease Holly Edmonds, MD Chief Resident Department of Dermatology.

Basal Cell Carcinoma

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Nodulocystic Acne

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Xanthelasma

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Herpes Labialis

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Nevus Sebaceous


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