Manifestations of Systemic Disorders Flashcards
Pyoderma gangrenosum
Characteristics?
Associated with chronic underlying inflammatory or malignant? 5
- Characteristics
- Rapidly evolving, idiopathic, chronic and severely debilitating ulcerative skin disease
Associated with chronic underlying inflammatory or malignant disease:
- Chronic Ulcerative colitis**
- Chronic active hepatitis
- Rheumatoid arthritis
- Crohn’s disease
- Hematologic malignancies
Pyoderma gangrenosum
- Hx? 3
- PE? 4
- Most common sites?3
- History:
- Acute onset with
- painful hemorrhagic pustule or painful nodule
- surrounded by erythematous halo. - PE:
- Breakdown with ulcer formation with dusky red or purple borders, -irregular and raised,
- boggy with perforations that drain pus. - Most common sites:
- Lower extremities,
- buttocks,
- abdomen
Pyoderma gangrenosum
Treatment?
- Treat underlying disease
- Avoid trauma
- High dose systemic (IV) corticosteroids****
- Systemic immunosuppression
Pyoderma gangrenosum: what kind of systemic immunosuppressants would we use? 3
- Sulfasalazine
- Cyclosporine
- Infliximab
Diabetic Associations
5
- Acanthosis nigricans
- Necrobosis lipoidica diabeticorum
- Granuloma annulare
- Thrush
- Intertrigo
Acanthosis nigricans
Characteristics? 2
Associated with what? 3
Characteristics:
- Velvety thickening and
- hyperpigmentation of the skin.***** (buzz words)
Associated with:
- Endocrine disorders
- Obesity
- Internal malignancy (will affect the mucous membranes more so than the other causes)
Which endocrine disorders are associated with Acanthosis nigricans? 2
What malignancies are most common?
- Diabetes
- Insulin resistant syndromes** (most commonly)
GI most common (will affect the mucous membranes more so than the other causes)
Acanthosis nigricans
Hx? 2
PE? 4
Most common sites? 4
History:
- Insidious onset;
- first visible change is darkening of pigmentation
PE:
- Hyperpigmentation,
- velvety looking,
- skin line accentuated,
- surface becomes wrinkled or creased.
Most common sites:
- Axilla,
- neck (back and sides),
- groin,
- antecubital fossae
Acanthosis nigricans
Rx?
3
- R/O Diabetes
- Treat associated disorder
- Usually none required
Necrobiosis Lipoidica (NL), Diabeticorum 1. 50% of pts with what have this?
- PE? 4
- Necrobiosis Lipoidica (NL), Diabeticorum Rx?
- Most common area?
- 50% of patients with NL are insulin dependent diabetics (longstanding Type I)
- PE:
- Oval, violaceous patch that expands slowly.
- Advancing border is red and the central area turns yellow-brown.
- Telangiectasias become prominent.
- Ulceration is possible esp. after trauma.
3. Difficult to treat, refer to derm
4. On the shin
Granuloma Annular
- Characteristics? 3
- SLight associated with what?
- Common in who?
- Hx? 1
- PE? 2
- Most common site?3
- Characteristics (arranged in a ring)
- Self limiting,
- asymptomatic,
- chronic dermatosis - Slight association with diabetes
- More common in older women
- History:
- Slowly increases over months, duration is variable - PE:
- Smooth, shiny firm ring of flesh colored papules and plaques (1 to 5cm)
- Annular with central depression - Most common site:
- dorsum of hands and feet,
- extremities, and
- trunk
Granuloma Annular
Treatment?
3
1. Treatment (not necessary) Disappears in 75% of pts in 2 years. If needed: 2. Potent topical steroids 3. Intralesional injections of steroids
Intertrigo Characteristics? 2
Related to what infections? 3
Associated with what disease? 3
Most common sites? 4
Dx?
- Irritation in the skin folds
- Worse with heat and moisture
Related to
- Candida,
- Fungus Irritation and
- Secondary bacterial infection (Group A & B strep, pseudomonas)
Associated with
- Diabetes,
- HIV,
- obesity
MOst common sites?
- Axilla,
- groins,
- gluteal folds,
- overlapping abdominal panniculus
Dx?
1. KOH smear
Intertrigo
PE? 4
Treatment?
3
What should be avoided?
PE:
- Erythema,
- or – pruritis,
- tenderness,
- erythematous plaques
Treatment: 1. Keep cool and dry (hairdryer) Treatment based on cause 2. Antifungal/antibacterial powders 3. Zinc oxide ointment reduces friction
- Topical steroids should be avoided
- What is Thrush?
- What do the lesions look like?
- Associations? 3
- Treatment? 3
- Yeast infection, usually candida.
- White plaques or red erosive areas in the oral mucosa
- Associations
- Diabetes
- HIV
- Immunosuppresion - Treatment
- Antifungal troches,
- fluconazole,
- itraconazole
Other systemic disorders that manifest dermatologic symptoms
4
- LUPUS
- DERMATOMYOSITIS
- SCLERODERMA
- XANTHOMA
LUPUS #1 Chronic cutaneous (discoid) 1. What will the lesions look like? 3 2. Progression to what is uncommon? 3. Treatment? 2
- Scarring,
- dispigmented,
- scaly plaques on face primarily.
- Progression to systemic lupus is uncommon
- Treatment with
- potent topical steroids
- +/- antimalarials (hydroxycholorquine)
Lupus #2 Subacute cutaneous 1. What population more common? 2. What are the skin lesions here? 3. 50% meet criteria for what? 4. Can be induced by what? 5. Treatment with what? 2
- White women age 15-40
- Polycyclic scaly plaques in sun exposed areas
- 50% meet criteria for systemic lupus.
- Can be drug induced
- Treatment with
- antimalarials or other
- immunosuppressants.
#3 Systemic lupus erythematosus (SLE) 1. What will the skin manifestations look like? 4
- Skin involvment in SLE in what %?
- Treatment? 2
3 Systemic lupus erythematosus (SLE)
- “Butterfly” facial erythema which is nonscarring
- Photosensitivity
- Oral ulcers
- Discoid lupus
- Skin involvement in 80% of SLE
- Treatment:
- antimalarials and
- immunosuppression
Dermatomyositis
Dermatitis characteristics? 5
Myositis characteristics? 2
Treatment? 2
What is present in the majority of these pts?
- Erythema,
- photosensitivity,
- heliotrope eyelid rash
- Gottron’s papules
- Calcinosis cutis (calcium deposits in the nails)
- Weakness of proximal muscles
- Elevation in muscle enzymes
- Steriods and methotrexate
- Malignancy
Where are Gottron’s papulse found?
3
- knuckles,
- periungual telangiectasia,
- poikiloderma (red, white, brown)
Scleroderma
Clinical presentation?
(CREST) 5
Treatment?
- Calcinosis (calcified lesions in hands)
- Reynaud’s phenomenon
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasia
Treatment with systemic immunosuppressio
Progressive systemic sclerosis is what?
Treatment?
- Generalized sclerosis with internal organ involvement (heart, lungs, GI, kidney)
- Treatment with systemic immunosuppression
Xanthomas
- What are they?
- What do they look like? 2
- Associations? 4
- Common sites? 2
- Lipid deposits in skin and tendons.
- Yellow-brown, pinkish or orange
- macules, papules, plaques, nodules
Associations
- Hyperlipidemia
- Biliary cirrhosis,
- Diabetes,
- CRF
Common sites
- Upper and lower eyelids,
- inner canthus
What are the types of xanthomas?
3
- Eruptive xanthomas
- Tuberous xanthomas
- Tendinous xanthomas
Whats the most common of all the xanthomas?
Xanthelasma
creamy orange
Xanthelasma
- Most common causes unrelated to what?
- When seen in children or young adults, it is associated with what?
- Occurs in individuals over what?
- Most cases unrelated to hyperlipidemia
- Occurs in individuals >50 y/o
- familial hypercholesterolemia.
Xanthomas
1. DX? 2
- RX? 4
- Check for hyperlipideima, although >50% of these patients have no metabolic disturbance.
- If + hyperlipidemia, CV disease may be expected.
- Laser,
- excision,
- electrodesiccation or
- topical application of trichloroacetic acid.
DESQUAMATION DISORDERS
3
- Erythema Multiforme
- Stevens-Johnson Syndrome
- Toxic Epidermal Syndrome
Erythema Multiforme:
Multiple morphologic presentations
5
- Erythematous “targetoid” macules / patches
- Papules / plaques
- Vesicles / bullae
- Wheals (urticaria)
- Erosions
Erythema Multiforme
EM Minor characteristics? 2
Due to? 2
EM Major? 3
Due to? 3
- Little or no mucous membrane involvement
- Few (if any) systemic symptoms
- Often due to
- HSV (sometimes subclinical, often recurrent)
- Can be due to medications - Always with mucous membrane involvement
- 2 or more sites - Skin involvement more widespread
- Systemic symptoms such as fever
- Hepatitis,
- nephritis
- Often due to medications
Erythema Multiforme
Almost any medication!!!
BUT frequent of offenders? 4
- Sulfa containing drugs
- bactrim,
- dapsone - Anti-epileptic drugs
- phenytoin,
- carbemazepine,
- phenobarbital) - Antibiotics:
- penicillin,
- cephalosporins - Allopurinol
Stevens-Johnson Syndrome
Toxic Epidermal Necrolysis
1. Mucocutaneous drug-induced or idiopathic reaction patterns characterized by what?
2. followed by?
3. SJS is considered the maximal variant of what?
4. WHat is considered the maximal form of SJS?
- skin tenderness and erythema of skin and mucosa,
- followed by extensive cutaneous and muscosal epidermal necrosis and sloughing.
- SJS is considered a maximal variant of Erythema Multiforme Major.
- TEN is considered a maximal variant of SJS.
Stevens-Johnson Syndrome
Toxic Epidermal Necrolysis
1. Onset?
2. Associated with what? 4
- Onset at any age, but most common >40 y/o
- Associated with:
- Drugs are clearly the leading cause (Onset 1-3 weeks after drug exposure)
- Systemic Lupus
- HIV
- HLS-B12
Stevens-Johnson Syndrome
Toxic Epidermal Necrolysis
1. Prodrome? 5
2. Progression? 4
- Prodromes
- Rash - Morbilliform, diffuse erythema
- Fever
- Mouth lesions
- Flu like symptoms 1-3 days prior to mucocutaneous lesions
- Mucocutaneous lesion, skin pain, burning and itching - Progression
- Necrotic epidermis, macular areas
- Sheet-like loss of epidermis
- Flaccid blisters that spread with lateral pressure (Nickolsky sign) on erythematous areas.
- With trauma, full thickness epidermal detachment yielding exposed, red, oozing dermis resembling a second-degree burn.
Stevens-Johnson Syndrome
Toxic Epidermal Necrolysis
Other organs such as what may be involved? 3
- Hepatitis,
- Nephrititis,
- Pneumonitis
What does TEN stand for what?
Mortality?
Toxic Epidermal Necrolysis
5- 40% mortality rate with TEN
Drugs associated with SJS and TEN
14
- Sulfa drugs
- Barbituates
- Allopurinal
- Diclofenac
- Sulindac
- Ketoprofen
- Ibuprofen
- Naproxen
- Cephalosporins
- Fluroquinolones
- Vancomycin
- Aminopenicillins
- Rifampin
- Ethambutol
Em minor/major treatment? 3
SJS/TEN treatment? 6
EM Minor/Major:
- Antiviral prophylaxis to control HSV
- Steriod burst pack
- Early diagnosis and cessation of suspected causative drug
SJS/TEN:
1. Early diagnosis and cessation of suspected causative drug
- Supportive care – ICU, fluids.
- Systemic steroids controversial – maybe helpful in early disease.
- Growing evidence for the use of IVIG, halts progression if administered early.
- Treat eye lesions with Erythromycin ointment
- Transfer to burn unit if widespread sloughing
Drug rash
1. Can happen within what time period?
- Very common with what?
- What area?
- 2 days of the onset of therapy to weeks after therapy is over.
- Bactrim (Septra)
- Chest
Treatment of drug rash?
4
- Benadryl
- IV/IM steroids
- Steroid dose pack
- Try not to sweat at night, don’t use covers
Bacterial Endocarditis
- What bug? 2
- What is it? 3
- Incidence is increasing in who?
- Hx?4
- PE? 3
Characteristics
1. Staph Aureus, Strep Viridans
- Proliferation of microorganisms on the endocardium of the heart.
- Incidence is increasing in the
- elderly,
- IVDU, and
- those with prosthestic valves. - History
- Fever,
- chills/sweats,
- anorexia/wt loss
- malaise - PE
- Heart murmur
- Arterial emboli
- Splenomegaly
Bacterial Endocarditis skin lesions?
4
- Janeway lesions
- Osler’s node
- Subungual Splinter hemorrhage
- Petechial lesion
Describe the following:
- Janeway lesions 3
- Osler’s node 3
- Petechial lesion? 2
- Nontender,
- hemorrhagic maculopapular
- lesions on palms and soles.
- Painful,
- red nodules on
- fingertips
- Small, non-blanching, reddish-brown macules on extremities, upper chest, mucus membranes. Occur in crops.
-Asymptomatic red streaks in nail bed.
Bacterial Endocarditis
- Dx? 6
- Treatment? 5
- ID at risk patients and prophylaxis
- Blood cultures
- CBC,
- Chem panel,
- Coags,
- Echo
- Treatment:
- PCN-G
- Nafcillin
- Gentamycin
- Vanco in MRSA
- Zyvox in MRSA
Rocky Mountain Spotted Fever
- What bug?
- Common in what months?
- Can be fatal if not treated especailly in who?
- Hx? 3
- PE? 5
Characteristics
- Rickettsia rickettsii spirochete
- Common May thru September
- Can be fetal if not treated, especially in the elderly
History
- History of tick bite given in 60% of cases
- Ask about outdoor activity
- Prodrome of
- anorexia,
- irritability,
- malaise
PE
- 1-2 weeks after tick bite:
- Fever (>102), chills, weakness
- Headache,
- photophobia
- Joint pain and weakness
Rocky Mountain Spotted Fever
1. Skin manifestation? 3
- Rash begins where and spreads where?
- Evolves to what over hours and days? 2
- Initially 2 to 6mm,
- pink blanching macules
- begin on extremities & spread centrally.
- Characteristically,
- rash begins on wrists, forearms, ankles and
- later on palms - Evolve to papules & petechiae over hours to couple of days.
Rocky Mountain Spotted Fever
1. RX? 2
- Treatment:
-Doxycycline (except for PG)
-Chloramphenical (for PG)
Start antibiotics if diagnosis is even suspected!
Doxycyline even in children now, per the CDC!!!
Less effect on teeth than Tetracycline.
Mortality rate ~60% in elderly
Lyme disease: Can cause what type of problems? 4
What bug?
A disease that can cause
- skin,
- joint,
- heart and
- nervous system problems.
Spirochete
Transmitted by the bite of an infected tick or flea
Ticks that cause Lyme disease
3
- Deer tick: Transmits Lyme disease to humans. Found in north-central and northeastern U.S.
- Lone star tick: Found in Texas and has been know to transmit Lyme disease.
- Rocky Mountain tick: Can transmit Lyme disease as well as Rocky Mountain spotted fever.
What kind of rash for lyme?
Multiple
Erythema
Migrans
(Skin rash)
Lyme Disease: Signs and Symptoms
Stage I:
1. When is this stage?
2. What kind of symptoms develop in 7-14 days?
3. Symptoms include? 7
4. Skin lesions may appear as what?
5. Secondary skin rashes appear in nearly ____% of individuals with Lyme disease.
- 3 to 30 days after bite.
- Flu-like symptoms develop within 7 – 14 days.
- Symptoms include
- fatigue,
- headache,
- fever and chills,
- muscle and joint pain,
- nausea, vomiting,
- dizziness and, a
- non-productive cough. - Skin lesion(s) may appear as a small red circular rash around the bite and expand.
- 80%
Lyme Disease: Signs and Symptoms Stage II: 1. When does it happen? 2. Symptoms? 3. What develops in 60% of pts? 4. Fifteen percent of people infected with Lyme disease develop what?
Two stages of Lyme disease:
1. Stage II (Late) – May occur weeks or months after the onset of Lyme disease.
- Severe headache and neck pain or stiffness.
- Arthritis will develop in 60% of patients weeks or months after infection (rarely more than 2 years).
- neurological symptoms, including psychiatric problems.
Preventing Lyme Disease6
- Take protective measures when outdoors.
- Wear light-colored clothing so that ticks can be easily seen.
- Tuck pants into boots or socks.
- Use a repellant containing DEET.
- Walk in the center of trails, and avoid contact with high grass and brush at trail edges.
6 Keep pets free of ticks.
Meningococcemia Characteristics 1. What bug? 2. Highest incidence when? 3. Season? 4. Most rapidly lethal form of what?
- Neisseria meningitidis
- Highest incidence between 6 mos. and 3 years of age.
- Highest incidence, midwinter, early spring
- Most rapidly lethal form of septic shock
Meningococcemia
1. PE? 5
- Early Exanthem
- Color and Size?
- What will they look like?
- Where at? 4 - Later lesions will look like what? 3
- PE
- High fever,
- tachycardia,
- mild hypotension,
- signs of meningeal irritation
- patient appears acutely ill.
2. Early Exanthem Occurs soon after onset -Pink 2mm-10mm -macules/papules, -sparsely distributed on trunk/lower extremities, face, palate, conjunctivae.
Later lesions
- Petechiae in center of macules
- Lesion become hemorrhagic within hours, purpura
- Purpura fulminans, hemorrhagic bullae
Menigococcemia
Dx? 3
RX? 4
- Blood cultures
- Pus from nodular lesion shows gram neg. diplococci
- D-dimers
Treatment
- Cefotaxine (Claforin)
- Ceftriaxone (Rocephin)
- PCN-G
- Hemodynamic stabilization
Gonococcemia
- What bug?
- What does the early bacteremic phase consist of? 3
- Peak incidence in what age?
- males
- females - The classic skin lesions are what?
- Neisseria gonorrhoeae
- early bacteremic phase consisting of
- tenosynovitis,
- arthralgias, and
- dermatitis - peak incidence in
- males aged 20-24 years and
- females aged 15-19 years - The classic skin lesions are acral hemorrhagic pustules.
Gonococcemia
The joints most commonly involved include those of the extremities, including the what?
About 75% of patients experience a dermatitis that can vary from what? 3
- wrists,
- the fingers,
- the elbows,
- the knees, and
- the ankles,
with 70% of patients experiencing a migratory polyarthralgia of 1-3 joints and the remaining 30% having involvement of more than 3 joints. - macular/papular to
- vesicular/pustular to
- necrotic or hemorrhagic erythema.
Labs of Gonococcemia? 3
- CBC
- ESR
- Culture
Gonococcemia treatment?
6
- Hospitalization with IV antibiotics
- Empiric tx with Rocephin
- PCN-G
- Augmentin
- Doxycycline and
- Azithromycin
