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erythema multiforme
Self-limited mucocutaneous reaction.
Etiology:
1) systemic infection (~50%) [5]
a) Herpes simplex (especially recurrent erythema multiforme minor)
b) Mycoplasma pneumoniae
c) Yersinia enterocolitica
2) sensitivity to pharmacologic agent (~50%) [5]
a) NSAIDs
- phenylbutazone
- salicylates
b) anticonvulsants
- barbiturates
- phenytoin
c) sulfonamides
d) antibotics
- penicillins
- tetracyclines
d) thiazides
e) chlorpropamide
f) codeine
g) sulfones
h) allopurinol
3) pregnancy
4) food allergy
5) X-rays
6) cancer
7) connective tissue disease
8) inflammatory bowel disease
Epidemiology:
1) adolescents & young adults
2) more frequent in males than females
Pathology:
1) dermal blood vessel involvement
2) extravasation of erythrocytes with resultant purpura
3) perivascular mononuclear infiltrate
4) edema of the upper dermis
5) eosinophilic necrosis of keratinocytes with subepidermal bulla formation
6) toxic epidermal necrolysis (TEN) in severe cases
Clinical manifestations:
1) mucous membrane manifestations (70%)
- mucosal erosion
- painful
- lips, oropharynx, nasal, conjunctival, vulvar, anal
- sudden appearance
- pharyngitis
- vulvitis & balanitis may interfere with micturition
- cheilitis & stomatitis may interfere with eating
2) skin manifestations
- erythematous plaques on skin &/or mucous membranes
- sudden appearance
- sharply demarcated
- symmetric distribution
- "target lesions" (3 zones) resulting from papule or vesicle formation in the center of a plaque
- resolution without scarring
- lesions may last up to 2 weeks
- predilection for dorsum of hands, palms & soles, feet, face, elbows, knees, penis (50%) & vulva
- pruritus
- positive Nikolsky sign in more severe forms
3) other manifestations
a) systemic symptoms in more severe forms
- mild fever
- arthralgias
- myalgias
- malaise
b) eye manifestations
- anterior uveitis
- corneal ulcers
- conjunctivitis
4) often recurrent
5) severe life-threatening variant
- necrotizing tracheobronchitis
- meningitis
- renal tubular necrosis
- Stevens-Johnson syndrome -> toxic epidermal necrolysis
* images [7,8,10]
Differential diagnosis:
1) acute erythematous plaques
a) drug eruption
b) psoriasis
c) secondary syphilis
d) urticaria
2) acute oral erosions
a) Herpes
b) pemphigus
c) lupus erythematosus
3) annular lesions
a) erythema migrans of Lyme disease
b) herald patch of Pityriasis rosea
c) Tinea corporis
Complications:
- secondary bacterial infection which may result in scarring especially on mucous membranes of eyes & urethra
- not fatal [5]
Management:
1) supportive care
2) acyclovir for control of Herpes simplex
a) does not shorten duration of symptoms
b) chronic suppressive therapy may diminish recurrence
3) treatment & prevention of bacterial infection
- azithromycin for pneumonia due to Mycoplasma pneumoniae ' - penicillin for secondary syphilis
4) prednisone 50-80 mg PO QD in divided doses, rapidly tapered
a) no controlled studies
b) reserve for severely ill patients
c) not effective; do not use [5]
Interactions
disease interactions
Related
acyclovir (ACV, Zovirax, Sitavig)
erythema nodosum (septal panniculitis)
Herpes simplex (HSV) or Herpes hominis
Nikolsky's sign
pemphigus
prednisone (Deltasone, Orasone, Liquid Pred, Meticortin, Rayos)
psoriasis
Stevens-Johnson syndrome (SJS)
syphilis
systemic lupus erythematosus
toxic (bullous) epidermal necrolysis (Lyell syndrome, TEN)
urticaria (hives)
General
vesiculobullous dermatitis
References
- Harrison's Principles of Internal Medicine, 13th ed.
Companion Handbook, Isselbacher et al (eds), McGraw-Hill
Inc. NY, 1995, pg 829-39
- DeGowin & DeGowin's Diagnostic Examination, 6th edition,
RL DeGowin (ed), McGraw Hill, NY 1994, pg 883
- Color Atlas and Synopsis of Clinical Dermatology, Common
and Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY,
1997, pg 332-35
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 169
- Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15,
16, 17, 18. American College of Physicians, Philadelphia 1998, 2006,
2009, 2012, 2015, 2018.
- Medical Knowledge Self Assessment Program (MKSAP) 19
Board Basics. An Enhancement to MKSAP19.
American College of Physicians, Philadelphia 2022
- Scully C, Bagan J.
Oral mucosal diseases: erythema multiforme.
Br J Oral Maxillofac Surg. 2008 Mar;46(2):90-5. Epub 2007 Sep 4.
PMID: 17767983
- Plaza JA, James WD (images)
Medscape: Erythema Multiforme
http://emedicine.medscape.com/article/1122915-overview
- DermNet NZ. Erythema multiforme (images)
http://dermnetnz.org/reactions/erythema-multiforme.html
- Stoopler ET, Houston AM, Chmieliauskaite M, Sollecito TP.
Erythema Multiforme.
J Emerg Med. 2015 Dec;49(6):e197-8.
PMID: 26281815
- Puerta-Pena M, Velasco-Tamariz V.
Images in Clinical Medicine.
Herpes-Associated Erythema Multiforme.
N Engl J Med 2021; 384:1848. May 13
PMID: 33979491
https://www.nejm.org/doi/full/10.1056/NEJMicm2033090