L37 (Lovelace) - Derm Manifestations of Systemic Disease

Osler nodes

seen in bacterial endocarditis - due to septic emboli

• painful

• erythematous nodule with pale center

• on fingers and toes

osler node

janeway lesion

seen in bacterial endocarditis - due to septic emboli

• not painful

• angular hemorrhagic lesion of palms and soles

janeway lesion

splinter hemorrhages

spilnter hemorrhages

seen in bacterial endocarditis - due to septic emboli

• black linear lesion under nail plate

Jones criteria

met in rheumatic fever

i

Carditis

Polyarthritis

Sydenham Chorea

Erythema Marginatum

Subcutaneous nodules

erythema marginatum

seen in acute rheumatic fever

• trunk and upper arms and legs - almost NEVER on face, palms, or soles

• pink or red macule or papules that spread outwards in a circular shape

• as lesions advance, edges become raised and red and central clearing forms

erythema marginatum

subcutaneous nodule

seen in rheumatic fever

• painless

• over joints, back of scalp, over vertebrae

• firm, round, mobile nodules between .5-2cm

• usually only when severe carditis is present

• appear in later phase of rheumatic fever

subcutaneous nodules

stasis dermatitis

chronic LE edema —> hyperpigmentation and LE swelling

typical presentation: erythema, scale, pruritus, erosions, exudate, crust

• lower 1/3 of legs, superior to medical malleolus

• bilateral or unilateral

• may develop lichenification

• often has varicose veins and hemosiderin deposits (pinpoint yellow-brown macules)

lipodermatosclerosis

Long-term uncontrolled edema (stasis dermatitis) pay progress to lipodermatosclerosis

• fat necrosis —> permanent sclerosis

• “inverted champagne bottle” legs

• may have acute inflammatory episodes with pain and erythema (can be mistaken for cellulitis)

Elephantiasis verrucosa nostra

Long-term uncontrolled edema (stasis dermatitis) may progress to elephantiasis verrucosa nostra

• inflammation of draining lymphatics —> damaged vessels —> lymphatic insufficiency

• pebbly, hyperkeratotic, rough overlying skin

• ulceration in this scenario would be super hard to treat

stasis dermatitis

lipodermatosclerosis

elephantiasis verrucosa nostra

cholesterol embolus

result of cholesterol mobilization from atherosclerotic plaques lining walls

can be sporadic, but usually with invasive vascular procedures or therapies (anticoagulation, thrombolytics)

• scattered, violaceous, reform (net-like) vascular patches

cholesterol embolus

livedo reticularis

livedo reticularis

well-know, common

• macular, violaceous, connecting rings in net-like pattern

caused by anatomy and physiology of cutaneous microvascular system

associated with

• antiphospholipid syndrome

• sneddon’s syndrome (rare, livedo reticularis + cerebrovascular lesions)

• cryoglobulinemia, cryofibrinogenemia

petechiae

petechiae

tiny hemorrhage spots under skin or in mucous membranes

• purple, red, or brown dots

• not palpable!!!

caused by:

• infection

  • RMSF

  • hemolytic uremic syndrome

  • viral hemorrhagic fevers (ebola)

• thrombocytopenia

  • TTP

  • ITP

  • leukemia

• nutritional deficiency

  • Vit C (scurvy!)

Stewart-Treves Syndrome

development of an aggressive lymphangiosarcoma at site of chronic lymphedema

(lymphedema is consequence of radical mastectomy)

• 90% upper limb

• chronic lymphedema + multiple red-blue macule or nodules

Stewart-Treves Syndrome

Peau d’orange

inflammatory brest carcinoma

type of cancer in which breast cells block lymph vessels in skin —> red and swollen breast

• may also see dimpling or pitting

• may also see inverted nipple

peau d’orange

rough, bumpy, pitted skin appearing on breast

• looks like an orange peel

caused by

• inflammatory breast cancer

• mastitis

Paget’s Disease

Paget’s Disease of the Breast

MALIGNANT

• one breast (usually)

• similar to eczema on outside: dry, scaling nipple and areola

  • eczema usually affects both breasts!!

• MC in F >50

***associated with underlying invasive breast cancer or ductal carcinoma in situ

Extramammary Paget’s Disease

Extramammary Paget’s Disease

MALIGNANT (adenocarcinoma)

• slow growing, non-invasive

  • confined to epidermis usually - can extend into deeper dermis

• mostly found in GU: vulva, penis, scrotum, perineum

  • can also be found in armpit/axillary region

• looks like a rash - often mistaken for eczema

Necrolytic migratory erythema

associated with glucagonoma syndrome

• genital and anal region, butt, groin, lower legs

• rash fluctuates in severity (we don’t know why the rash appears)

  • initially: ring-shaped red area that blisters, erodes, and then crusts

  • pruritic and painful

  • sore mouth tongue, sore mouth, cracked dry lips, ridging of nails

Glucagonoma syndrome

• adults >50

• slow-growing tumor in alpha cells of pancreas —> excessive glucagon excretion —>

  • diabetes

  • weight loss

  • diarrhea

  • neuro and psych sx

  • venous thrombosis

  • anemia

necrolytic migratory erythema

necrolytic migratory erythema

Carcinoid syndrome

neuroendocrine tumor of GI tract

• episodic flushing***

• diarrhea

• wheezing

• right valvular heart disease

• niacin deficiency (pellagra)

• increased urinary 5-HIAA (serotonin metabolite)

Trousseau syndrome

migratory superficial thrombophlebitis in pts with occult or recently diagnosed visceral malignant disease

Trousseau Syndrome

Sign of Leser-Trelat

Sign of LEser Trelat

sudden development of multiple pruritic seborrheic keratoses

often associated with malignancy

• roughly half are adenocarcinomas (MC = stomach, breast, colon, rectum)

• can also be seen in lymphoma, leukemia, squamous cell carcinoma

Necrobiosis Lipoidica Diabeticorum

Necrobiosis Lipoidica Diabeticorum

granulomatous skin disorder

• shins of diabetics

• begins as dull red papule or plaque —> slowly enlarges into one or more yellow-brown patches with a red rim

• central atrophy - shiny, pales, thinned, prominent blood vessels

• may have reduced sweating and sensation

Necrobiosis Lipoidica Diabeticorum

Pyoderma gangrenosum

Pyoderma gangrenosum

uncommon; recurrent and chronic ulcer

NOT ACTUALLY INFECTIOUS OR GANGRENOUS —> associated with systemic disease (has have underlying conditions like IBD)

• pathergy: exaggerated skin lesions in response to minor trauma

• undetermined border

Pellagra

Pellagra

Niacin (Vit B3) deficiency

• begins as rash without defined borders that resembles sunburn —> become severe with darker pigmentation, blisters, skin sloughing on face, neck, arms, legs

Vit B2 deficiency

deficiency in riboflavin

causes:

• stomatitis of mouth and tongue (magenta tongue!!!!!!!!!)

• cheilosis: inflammation of lips, scaling and tissues at corners of mouth

Scurvy

deficiency of ascorbic acid (Vit C)

• swollen guns

• easy brusing

• petechiae

• perifollicular hemorrhages***

• “corkscrew” hears***

scurvy

Zinc deficiency

acquired:

• angular chelitis

• sclay plaques on areas of friction

• nail dystrophy

• hair loss

acrodermatitis enteropathica (inherited): defect in zinc absorption

• angular chelietis

• gluteal, perineal, and aural burn-like psoriasiform lesions

zinc deficiency

Kwashiorkor

protein malnutrition

• edema

• anemia

• fatty liver

• hyperkeratosis***

• dyspigmentation****

Kwashiorkor