Aaron Bruce, DO, FAOCD, FACMS Dermatology and Mohs … · Moisten the skin first, bath or shower....

Aaron Bruce, DO, FAOCD, FACMS

Dermatology and Mohs Micrographic Surgery

Montana Skin Cancer and Dermatology Center, PC

Bozeman, MT

Emergency: An acute illness with an almost immediate risk to life or long term health.

Focusing on common presentations in non-pregnant adults.

Practical take home points will be emphasized.

Copious images!

RED- Erythroderma

SWOLLEN- Angioedema

SJS-TEN

Vascular

Infectious

65 year old male presents with acute onset erythema and scale over 98% of his body. Extremities are edematous. BP 95/68, Temp 100.7, tachycardic and shivering. Mucosa are normal. History did reveal a life long history of Atopic Dermatitis.

Also called exfoliative dermatitis

Defined as generalized redness or scaling

Vesicles and pustules are usually absent

May present with acute hair loss

Mucosal surfaces are spared

Erythroderma is not a specific diagnosis, but a clinical manifestation.

50% cases have underlying dermatosis

Psoriasis, Atopic, Chronic Actinic, Seb Derm, PRP, Contact

Drug eruption (20%)

Anti-epileptics, Allopurinol, PCN, Sulfonamides, Vanc, CCB, Cimetidine, Dapsone, Gold, Lithium, Quinidine.

Malignancy (11%)

CTCL (Sezary) or Paraneoplastic

Idiopathic (17%)

Reported to have overall Mortality approaching 20%

Most will need to be admitted

Cease any possible contributing agents or unnecessary meds

Aggressive supportive care and fluid regulation

Treat any underlying or contributing conditions/infections

Most end up getting systemic steroids but utilizing wet wraps, faster and safer resolution can be obtained.

Moisten the skin first, bath or shower. 5+ minutes, warm but not too hot.

Gently pat down with towels and but in general keep patient a little wet.

Quickly Coat all involved skin with steroid ointment. Liberally grease them down tip to tale.

Soak several towels in warm water. Wring out and place on the bed.

The patient then lies down on the bed and you proceed to cover them with warm damp towels. Tuck all the towels around the patient so most of the surface area makes contact. Then put several dry towels and blanket over to keep warm.

Leave for 2-3 hours. Repeat 2x a day.

Impressive improvement will be noticed in 12-24 hours.

Triamcinolone 0.1% OINTMENT 454gm tub.

Face and genitals: 0.25% OINTMENT

Buy your nurse a latte!

Similar treatment can treat severe stasis dermatitis in 48-72 hours.

Severe stasis dermatitis

TAC 0.1% ointment applied under

saran wrap occlusion and covered

with socks or Coban/Ace wraps done

nightly.

RED- Erythroderma

SWOLLEN- Angioedema

SJS-TEN

Vascular

Infectious

Paraneoplastic

A 40 year old diabetic female was started on an ACE inhibitor about a week ago for hypertension.

She calls your office stating her lips, cheeks and left hand are swollen. She also complains of abdominal pain. Your astute phone nurse mentions the patients voice was more soft spoken than usual.

Now most common exogenous cause of angioedema seen in emergency rooms

Usually has no associated urticaria

Due to increased bradykinin levels secondary to inhibition of kinin degradation

Can cause dramatic swelling of tongue, pharynx, or larynx – may require intubation or tracheostomy acutely

Airway is more commonly involved with ACE Induced angioedema vs other causes

Angioedema develops in 0.1% to 0.5% of those receiving the drug

Onset from 1st week of use to 2-3 years of use

Symptoms resolve within 24-48 hours of cessation of drug

Most commonly seen with captopril and enalopril, but described with all ACE inhibitors

Genetic factors may be important

Subjects with a history of angioedema from other causes are more susceptible to ACE-induced angioedemaSlater JAMA 1988

Face and lips most commonly involved but laryngeal edema reported

Risk factors include obesity, prior endotracheal intubation and face and neck surgery

ACE inhibitors will trigger attacks in those with HAE, so avoid in these patients

Jain Chest 1992

Management

Stop drug and use other classes of antihypertensive agents

ALL ACE inhibitors are to be avoided

Management of angioedema depends on site of involvement – securing the airway by intubation may be necessary

ACE receptor antagonists are generally considered to be safe

Johnson SP, Jacobsen J, Monster TBM et al. Am. J.Med.118:1428-1429, 2005

DIFFERENTIAL DIAGNOSIS OF ACUTE ANGIOEDEMA

IgE mediated allergic reactions to food, drugs, venoms etc.

Histamine-releasing drugs (e.g. opioids, RCM), pseudoallergens in food

Adverse reactions to certain medications: NSAIDs, ACE-inhibitors

Chronic urticaria with angioedema

Idiopathic or exercise induced anaphylaxis

C1-Esterase Inhibitor deficiency (hereditary or acquired)

Gleich syndrome: recurrent angioedema and high eosinophil counts of unknown etiology

Angioedema in hypereosinophilic syndrome

Etiology:

Often idiopathic

Medications

angiotensin-converting- enzyme inhibitor in 10-25% of cases

Penicillin

NSAID

Allergens (foods, radiographic contrast media)

Physical agents (cold, vibration, etc)

C1 esterase inhibitor deficiency: hereditary vs associated with autoimmune disorder or malignancy

Management Airway management

Antihistamines

Cool compresses

Avoid triggers

For pts with C1 esterase inhibitor deficiency:

Acute management vs short term vs long term prophylaxis: androgens (danazol and stanozolol), C1 esterase inhibitor concentrate, antifibrinolytics, icatibant (selective antagoist of bradykinin B2 receptor)

RED- Erythroderma

SWOLLEN- Angioedema

SJS-TEN

Vascular

Infectious

• 44 year old male reports worsening rash over the last 2 days with a 5 day history of sore throat, fever, myalgias.

– Has not taken antibiotics but has been using ibuprofen.

28

Pathophysiology: Drug induced mucocutaneous reaction

Culprit medications: Sulfonamides, anticonvulsants, allopurinol, NSAIDs. Usually given 1-3 weeks before onset

Genetic susceptibility

SJS and TEN are continuum SJS: BSA < 10%

SJS/TEN overlap: BSA 10-30%

TEN: BSA > 30%

• Prodromal Symptoms

– Malaise, rhinitis, sore throat, body aches, and fever.

– Followed by the abrupt development of a macular rash that may or may not appear as target lesions.

– Mucous membrane involvement may precede rash in TEN

– Macular exanthem usually starts centrally and then spreads to the extremities

• Bullae form within the rash and large sheets of epidermis separate from the dermis.

– Involved skin is exquisitely tender to palpation.

– Nikolsky Sign (+)

30

• Mucous membrane involvement develops

• Conjunctival and corneal involvement may lead to permanent scarring and blindness.

– Full thickness of epidermis involved

• Mortality rate 15-20%

– as high as 50% in elderly patients

• Life-threatening metabolic derangements, sepsis, respiratory failure, and gastrointestinal hemorrhage may occur and are compounded by underlying comorbidities.

33

SJS

35

Bullous SJS/TEN

36

Supportive Care Discontinue offending agent, fluid replacement, electrolyte

balance, ophthalmologic assessment and aggressive infection control

Systemic corticosteroids remain controversial no benefit and associated with increased mortality secondary to

infection

Admission to a Burn ICU Fluid replacement

Per Parkland Formula with Ringer’s Lactate

Goal UOP 0.5cc/kg/hr

Wound Care (no Silvadine = Sulfa)

Infection control

IVIG per local protocol Fas ligand (FasL) expression hypothesized as the “death ligand”

induced for abnormal apoptosis of epidermal cells

in SJS/TEN patients

Pooled human immunoglobulin contain anti-Fas antibodies that have been shown in vitro to impede apoptosis when pre-incubated with keratinocytes

37

1. The hair-bearing scalp is spared even in severe disease.

2. Neutropenia is associated with a poor prognosis.

3. Cross reactions within anticonvulsants are common. The first 8 weeks of treatment have the highest risk of TEN and previous anticonvulsant therapy portends a tenfold increased risk.

4. Patients with previous SJS/TEN to one medication class are higher risk for developing SJS/TEN to other medication classes.

38

RED- Erythroderma

SWOLLEN- Angioedema

SJS-TEN

Vascular

Infectious

Calciphylaxis

Vasculitis (PAN)

Purpura fulminans/DIC

Arterial thrombosis

54 y/o female on HD presented with tender, lumpy, indurated plaques on abdomen and lateral thighs.

Mottled skin ulcerates, exposing necrotic SC adipose tissue

Necrosis progresses and despite intensive wound care, ptdies of sepsis

Painful skin lesions with superficial violaceous nodules on tips of digits, ankles, thighs or buttocks

Progressed to hemorrhagic eruptions with ischemic necrosis

Bilaterally symmetrical, superficial, maintained persistent distal pulses

Pathogenic Factors Uremic milieu + high Ca

Ca content of skin noted to be high in HD pts and higher when dialysate Ca concentrations of 4.0 meq/L. Lowering dialysate Ca improved CUA in some pts. Also avoiding ca based po4 binders

Presence of high PTH levels

Primary: calcium salts accumulate in media of small arteries lumen narrows

• Secondary Lesion

• Infarcts of the subcut tissue and skin comprise the secondary lesions, responsible for initial clinical manifestations of the syndrome.

• Before skin ulcerations, tissue ischemia leads to hard lumpy and/or plaque-like charac to the SC tissue.

Primary lesion alone not sufficient to initiate infarction ie, thrombosis or reduced perfusion contribute

Thrombi often found in primary lesios

Preexisting disturbances of coagulation are relevant to the pathogenesis: protein c/s and cryofibrinogen

Also iron dextran suggested to aggravate or initiate the secondary lesions

Consistent localization of secondary lesions in body areas of greatest adiposity

Women deposit more of their fat in SC than internal adipose stored

Bleyer, et al AJKD 1998 identified obesity and low serum alb as highly predictive of CUA

Risk of calciphylaxis increased with increased weight

1-4% of ESRD population

Increasing prevalence suspected due to incr use of vit d analogues and calcium based p04 binders, in addition to increased reporting• Whites > Blacks

• Females > Males 3:1

• Obesity confers risk

Mortality: 60-80% Sepsis from infected/necrotic skin lesions

Higher mortality in proximal dz than acral dz

Systemic Vasculitis

Warfarin Necrosis

Lupus Panniculitis

Polyarteritis of SLE

HSP

Pancreatic panniculitis

Weber-Christian Syndrome: panniculitis sometimes with necrotic ulcers

Acquired protein C deficiency

Normalization of Ca x P product

Aggressive wound care with debridement of necrotic tissue and systemic Abx therapy

HD with low dialysate Ca concentration no greater than 2.5 meq/L

Avoid Ca containing po4 binders

Local injections in adipose areas where lesions usu develop should be avoided

Other potential triggers: blood products, corticosteroids, immunosuppressant should be avoided

RED- Erythroderma

SWOLLEN- Angioedema

SJS-TEN

Vascular

Infectious

Meningococcemia

Rickettsioses/RMSF

Staph scalded skin and TSS

Necrotizing Fasciitis

Etiology Neisseria meningitides (gram neg diplococcus) spread by respiratory

route

Often seen in young adults and children

Risk factor: asplenia, immunoglobulin or terminal complement deficiencies

Dermatologic findings Abrupt onset of maculopapular or petechial eruption on acral surface,

trunk or lower extremities -> progression to purpura in hours

Angular edge with “gun metal gray” center

+/- mucosal involvement

Clinical presentation Flu like symptoms: fever, chills, malaise

DIC, shock, death

61 y/o male who regularly hikes and gardens presents to the ER with Fever, weakness, and myalgias. He did report tick exposure, but can’t remember exactly when. No rash was seen on exam. He seems somewhat confused, which his wife says is not baseline.

“A second peak incidence of disease occurs in

males over age 60. This is in contrast to the constant

rate of disease seen in females after age 14. Absence

of the typical skin rash (“spotless”), atypical features,

and greater severity characterize RMSF in the el-

derly.”

Clinical presentation Triad: fever, headache and rash (only in 60%)

Can have variety of organ involvement (cardiogenic shock, hepatic failure, renal failure, meningismus and DIC)

Mortality is 30-70% if untreated vs 3-7% if treated

Dermatologic findings Purpuric macules and papules

Starts on the wrists and ankles within 2 weeks-> spread to palms, soles-> to trunk and face

Over 2-4 days, the skin will become hemorrhagic and petechial

May have eschar at site of bite

“Spotless” RMSF occurs approx 10%

of the time

First starts on wrists and ankles

Greatest odds of survival if treatment started within 5 days of symptoms. Most diagnostic tests remain negative for at least 7 days. Thus, best diagnosed on clinical grounds and treated empirically.

Helpful signs, thrombocytopenia, hyponatremia, elevated LFTs

History of exposure high grass, dogs etc… may help as most don’t recall a tick exposure.

Treatment is Doxycycline first line and Chloramphenicol as distant 2nd line

Confirmatory test however is skin biopsy of rash with the appropriate immunostains, or the gold standard, indirect immunofluorescence assay with R. rickettsii antigen on two paired serum samples. (IgM and IgG)

RED- Erythroderma

SWOLLEN- Angioedema

SJS-TEN

Vascular

Infectious