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TREATMENT

GENERALMEASURES

Step 1: Discontinue or treat inciting factor (1).

Wound care for severe cases with epidermal detachment

Oral lesions should be addressed to insure maintenance of PO intake. This can include oral anesthetic solutions and antiseptic rinses.

MEDICATION

Acute EM

–Discontinuation of inciting factors and treatment of underlying disease (1) [B]

–Symptomatic treatment with oral antihistamines and topical (1)[B]

–HSV-induced EM: Most recent sources report no proven effect on the course of EM using antivirals with acute mild EM (1)[B].

–M. pneumoniae-associated EM may require antibiotics.

Mucosal membrane EM

–Consider high-potency topical corticosteroid gel, oral antiseptic, and oral anesthetic solutions if mild.

–If more severe, consider prednisone 40 to 60 mg/day with dosage tapered over 2 to 4 weeks (1)[B].

–Ophthalmology consultation is imperative for ocular involvement.

Recurrent EM

–First-line treatment with HSV-associated and idiopathic recurrent EM is antiviral prophylaxis; 12 to 24 months of prophylaxis is most effective (1) [B].

–Therapy includes acyclovir 400 mg BID, valacyclovir 500 mg BID, famciclovir 250 mg BID (1)[B].

–Second-line therapy includes dapsone (100 to 150 mg/day), azathioprine (Imuran, 100 to 150 mg/day), thalidomide (100 to 200 mg/day), tacrolimus (0.1% ointment daily), mycophenolate mofetil (CellCept, 1,000 to 1,500 mg BID), hydroxychloroquine (400 mg/day) (1)[B].

ADMISSION, INPATIENT, AND NURSING CONSIDERATIONS

Care at home

Hospitalization needed for fluid and electrolyte management in patient with

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severe mucous membrane involvement, impaired oral intake, and dehydration

IV antibiotics if secondary infection develops

ONGOING CARE

FOLLOW-UPRECOMMENDATIONS

Patient Monitoring

The disease is self-limiting.

Complications are rare, with no mortality.

DIET

As tolerated, with increased fluid intake

PATIENT EDUCATION

The disease is self-limiting. However, the recurrence risk may be 30%.

Avoid any identified etiologic agents.

PROGNOSIS

Rash evolves over 1 to 2 weeks and subsequently resolves within 2 to 6 weeks, generally without scarring or sequelae.

Following resolution, there may be some postinflammatory hyperor hypopigmentation.

COMPLICATIONS

Secondary infection

REFERENCES

1.Sokumbi O, Wetter DA. Clinical features, diagnosis, and treatment of erythema multiforme: a review for the practicing dermatologist. Int J Dermatol. 2012;51(8):889–902.

2.Rosenblatt AE, Stein SL. Cutaneous reactions to vaccinations. Clin Dermatol. 2015;33(3):327–332.

3.Levin J, Hofstra T. Recurrent erythema multiforme. JAMA. 2014;312(4):426– 427.

4.Iwai S, Sueki H, Watanabe H, et al. Distinguishing between erythema multiforme major and Stevens-Johnson syndrome/toxic epidermal necrolysis

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immunopathologically. J Dermatol. 2012;39(9):781–786.

5.Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: part I. Introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis. J Am Acad Dermatol. 2013;69(2):173.e1– 173.e13, quiz 185–186.

6.Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: part II. Prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment. J Am Acad Dermatol. 2013;69(2):187.e1–187.e16, quiz 203–204.

ADDITIONALREADING

Sola CA, Beute TC. Erythema multiforme. J Spec Oper Med. 2014;14(3):90– 92.

Wetter DA, Davis MD. Recurrent erythema multiforme: clinical characteristics, etiologic associations, and treatment in a series of 48 patients at Mayo Clinic, 2000 to 2007. J Am Acad Dermatol. 2010;62(1):45–53.

SEE ALSO

Cutaneous Drug Reactions; Dermatitis Herpetiformis; Pemphigoid Gestationis;

Stevens-Johnson Syndrome; Toxic Epidermal Necrolysis; Urticaria

CODES

ICD10

L51.9 Erythema multiforme, unspecified

L51.8 Other erythema multiforme

L51.0 Nonbullous erythema multiforme

CLINICALPEARLS

EM is diagnosed clinically by careful review of the history, thorough detailed physical exam, and by excluding other similar disorders. No lab tests are required for the diagnosis.

Typical lesions are characteristic targetoid or “iris” lesions but can include raised targetoids.

Lesions are symmetrically distributed on palms, soles, dorsum of the hands,

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and extensor surfaces of extremities and face. The oral mucosa is the most affected mucosal region in EM.

Management of EM involves determining the etiology when possible. The first step is to treat the suspected infection or discontinue the causative drug.

Complications are rare. Most cases are self-limited. However, the recurrence risk may be as high as 30%.

Recurrent cases often are secondary to HSV infection. Antiviral therapy may be beneficial.

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ERYTHEMANODOSUM

Lolwa Al-Obaid, MD Faruq Pradhan, MBBCh

Fredric D. Gordon, MD, AGAF

BASICS

DESCRIPTION

Adelayed-type hypersensitivity reaction to various antigens, or an autoimmune reaction presenting as a panniculitis (1) that affects subcutaneous fat

Clinical pattern of multiple, bilateral, erythematous, tender nodules in a typically pretibial distribution that undergo a characteristic pattern of color changes, similar to that seen in bruises. Unlike erythema induratum, the lesions of erythema nodosum (EN) do not typically ulcerate.

Occurs most commonly on the shins; less commonly on the thighs, forearms, trunk, head, or neck

Often associated with nonspecific prodrome including fever, weight loss, and arthralgia

Often idiopathic but may be associated with a number of clinical entities

Usually remits spontaneously in weeks to months without scarring, atrophy, or ulceration

Uncommon to have recurrences after initial presentation

Pregnancy Considerations

May have repeat outbreaks during pregnancy

Pediatric Considerations

Rare pediatric variant has lesions only on palms or soles, often unilateral; typically has a shorter duration in children than adults

EPIDEMIOLOGY

Incidence

1 to 5/100,000/year

Predominant age: 20 to 30 years Predominant sex: female > male (6:1) in adults

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Prevalence

Varies geographically depending on the prevalence of disorders associated with EN

Reported 1 to 5/100,000

ETIOLOGYAND PATHOPHYSIOLOGY

Idiopathic: up to 55%

Infectious: 44%. Streptococcal pharyngitis (most common), mycobacteria, mycoplasma, chlamydia, mycoplasma, coccidioidomycosis, rarely can be caused by Campylobacter spp., ricketssiae, Salmonella spp., psittacosis, syphilis

Sarcoidosis: 11–25%

Drugs: 3–10%; sulfonamides amoxicillin, oral contraceptives, bromides, azathioprine, vemurafenib

Pregnancy: 2–5%

Enteropathies: 1–4%; ulcerative colitis, Crohn disease, Behçet disease, celiac disease, diverticulitis

Rare causes: <1% (2)

–Fungal: dermatophytes, coccidioidomycosis, histoplasmosis, blastomycosis

–Viral/chlamydial: infectious mononucleosis, lymphogranuloma venereum, paravaccinia, HIV

–Malignancies: lymphoma/leukemia, sarcoma, myelodysplastic syndrome

–Sweet syndrome

RISK FACTORS

See “Etiology and Pathophysiology.”

COMMONLYASSOCIATED CONDITIONS

See “Etiology and Pathophysiology.”

DIAGNOSIS

HISTORY

Often a prodrome 1 to 3 weeks prior to onset of lesions; can consist of malaise, fever, weight loss, cough, and arthralgia

Increasingly tender nodules on the legs, usually over the shins Fever, malaise, chills, fatigue

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Headache

Can precede systemic process by weeks

PHYSICALEXAM

Lesions initially present as warm, tender, erythematous firm nodules and become fluctuant, gradually fading to resemble a bruise over 1 to 2 months (erythema contusiformis).

Typically pretibial, although can extend proximally to involve thighs or trunk (atypically can involve extensor surface of forearms)

Diameter varies from 1 to 10 cm with poor demarcation.

DIFFERENTIALDIAGNOSIS

Nodular vasculitis or erythema induratum (warm ulcerating calf nodules)

Superficial thrombophlebitis

Cellulitis

Weber-Christian disease (violaceous, scarring nodules)

Lupus panniculitis

Cutaneous polyarteritis nodosa

Sarcoidal granulomas

Cutaneous T-cell lymphoma

EN leprosum (clinically similar to EN but shows vasculitis on histopathology)

Subcutaneous infection (including staphylococcus, Sporothrix schenckii,

Nocardia brasiliensis, Mycobacterium marinum, Leishmania braziliensis)

DIAGNOSTIC TESTS & INTERPRETATION

Diagnosis is made clinically, with support of testing.

ESR or C-reactive protein (CRP): often elevated, but can be normal in up to 40% (3)[C]

CBC: mild leukocytosis (3)[C]

Urine pregnancy test (3)[C]

Throat culture, antistreptolysin O titer (3)[C]

Blood and/or stool culture, stool ova and parasites (O&P)

Tuberculin skin testing (3)[C]

Seronegative rheumatoid factor

Initial Tests (lab, imaging)

CXR for hilar adenopathy or infiltrates related to sarcoidosis or tuberculosis (3) [C]

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Diagnostic Procedures/Other

Deep-incisional or excisional skin biopsy including subcutaneous tissue; rarely necessary except in atypical cases with ulceration, duration >12 weeks, or absence of nodules overlying lower limbs (4)[C]

Test Interpretation

Septal panniculitis without vasculitis

Neutrophilic infiltrate in septa of fat tissue early in course

Actinic radial (Miescher) granulomas, consisting of collections of histiocytes around a central stellate cleft, may be seen.

Fibrosis, paraseptal granulation tissue, lymphocytes, and multinucleated giant cells predominate late in course (5).

TREATMENT

Condition usually self-limited within 1 to 2 months

All medications listed as treatment for EN are off-label uses of the medications. There are no specific FDA-approved medications.

GENERALMEASURES

Mild compression bandages and leg elevation may reduce pain. (Wet dressings, hot soaks, and topical medications are not useful.)

Discontinue potentially causative drugs.

If specific cause is identified, treatment of the condition typically leads to resolution of EN.

Indication for treatment is poorly defined in literature; hence, therapy specifically for EN is directed toward symptom management.

MEDICATION

First Line

NSAIDs:

–Ibuprofen 400 mg PO q4–6h (not to exceed 3,200 mg/day)

–Indomethacin 25 to 50 mg PO TID

–Naproxen 250 to 500 mg PO BID

Precautions

–GI upset/bleeding (avoid in Crohn or ulcerative colitis)

–Fluid retention

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–Renal insufficiency

–Dose reduction in elderly, especially those with renal disease, diabetes, or heart failure

–May mask fever

–NSAIDs can increase cardiovascular (CV) risk.

Significant possible interactions

–May blunt antihypertensive effects of diuretics and β-blockers

–NSAIDs can elevate plasma lithium levels.

–NSAIDs can cause significant elevation and prolongation of methotrexate levels.

Second Line

Potassium iodide 400 to 900 mg/day divided BID or TID × 3 to 4 weeks (for persistent lesions); need to monitor for hyperthyroidism with prolonged use; pregnancy class D (6)[B]

Corticosteroids for severe, refractory, or recurrent cases in which an infectious workup is negative. Prednisone 1 mg/kg/day for 1 to 2 weeks is the recommended dose/duration. Potential side effects include hyperglycemia, hypertension, weight gain, worsening gastroesophageal reflux disease, mood changes, bone loss, osteonecrosis, and proximal myopathy (2).

For EN related to Behçet disease, one can also consider colchicine 0.6 to 1.2 mg BID. Potential side effects include GI upset and diarrhea (7)[B].

ADMISSION, INPATIENT, AND NURSING CONSIDERATIONS

Occasionally, admission may be needed for the antecedent illness (e.g., tuberculosis).

ONGOING CARE

FOLLOW-UPRECOMMENDATIONS

Keep legs elevated.

Elastic wraps or support stockings may be helpful when patients are ambulating.

Patient Monitoring

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Monthly follow-up or as dictated by underlying disorder

DIET

No restrictions

PATIENT EDUCATION

Lesions will resolve over a few weeks to months.

Scarring is unlikely.

Joint aches and pains may persist.

<20% recur.

PROGNOSIS

Individual lesions resolve generally within 2 weeks.

Total time course of 6 to 12 weeks but may vary with underlying disease

Joint aches and pains may persist for years.

Lesions do not scar.

Recurrences: occurs over variable periods, averaging several years; seen most often in sarcoid, streptococcal infection, pregnancy, and oral contraceptive use. If medication induced, avoid recurrent exposure.

COMPLICATIONS

Vary according to underlying disease

None expected from lesions of EN

REFERENCES

1.Chowaniec M, Starba A, Wiland P. Erythema nodosum—review of the literature. Reumatologia. 2016;54(2):79–82.

2.Schwartz RA, Nervi SJ. Erythema nodosum: a sign of systemic disease. Am Fam Physician. 2007;75(5):695–700.

3.Cribier B, Caille A, Heid E, et al. Erythema nodosum and associated diseases. Astudy of 129 cases. Int J Dermatol. 1998;37(9):667–672.

4.Requena L, Yus ES. Erythema nodosum. Dermatol Clin. 2008;26(4):425– 438.

5.Sánchez Yus E, Sanz Vico MD, de Diego V. Miescher’s radial granuloma. A characteristic marker of erythema nodosum. Am J Dermatopathol. 1989;11(5):434–442.

6.Horio T, Imamura S, Danno K, et al. Potassium iodide in the treatment of erythema nodosum and nodular vasculitis. Arch Dermatol. 1981;117(1):29–

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