Diagnose on Sight: Diffuse Desquamating Dermatitis

Case: An 84 year old female presents with five days of a diffuse rash. She had a seizure and was started on phenytoin 2 weeks ago. Her mouth, labia, and medial canthi are involved. There are scattered areas of desquamation comprising less than a tenth of her total body surface area. She is tachycardic and febrile. Her complete blood count differential is normal. What is the diagnosis?

Poll Results

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Stevens-Johnson Syndrome

Explanation

Stevens–Johnson syndrome (SJS) is a severe mucocutaneous disorder often caused by a hypersensitivity reaction to a medication. SJS and toxic epidermal necrolysis (TEN) are considered bookends of a spectrum of severe cutaneous drug reactions, differing only by their extent of skin detachment.¹

SJS is characterized by fever, widespread blistering exanthema and mucous membrane erosions with epidermal detachment on less than 10% of the body surface area. TEN has >30% epidermal detachment.² Several medications are associated with SJS/TEN:^1,3

Allopurinol
Aminopenicillins
Carbamazepine
Cephalosporins
NSAIDs
Oxicam-type
Phenobarbital
Phenytoin
Quinolones
Sulfonamide-containing medications

Rarely, SJS/TEN can be related to an infectious etiology like Mycoplasma pneumonia.^2,3

Prompt withdrawal of the offending agent and supportive care are the mainstays of treatment. Corticosteroids are widely used. Although its effectiveness remains controversial, high-dose intravenous immunoglobulin (IVIG) has more recently been used to treat SJS/TEN.⁴

SJS/TEN by the numbers³

90 – percentage of patients that have mucosal lesions
3 weeks – the typical length of the hospitalization
∞ – occurs in all ages, all races, and both sexes
5 – percentage of patients who report no preceding drug use

Master Clinician Bedside Pearls⁵

Paul Juhn, MD FAAEM
Content Director
Hippo Education Inc.
Clinical Assistant Professor of Emergency Medicine
University of California San Francisco – San Francisco General Hospital
Twitter: @jhunlikefun

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Harr T, French L. Toxic epidermal necrolysis and Stevens-Johnson syndrome. Orphanet J Rare Dis. 2010;5:39. [PubMed]

Nozaki Y, Fujita H, Okada R, Kou K, Aihara M. Non-drug-induced Stevens-Johnson syndrome successfully treated with high-dose i.v. immunoglobulin. J Dermatol. 2015;42(4):439-440. [PubMed]

Roujeau J, Stern R. Severe adverse cutaneous reactions to drugs. N Engl J Med. 1994;331(19):1272-1285. [PubMed]

Lee H, Lim Y, Thirumoorthy T, Pang S. The role of intravenous immunoglobulin in toxic epidermal necrolysis: a retrospective analysis of 64 patients managed in a specialized centre. Br J Dermatol. 2013;169(6):1304-1309. [PubMed]

The Annals of Emergency Medicine – Decoding the Drug Eruption – It is Hard to Breathe When Your Vocal Cords Don’t Work! EM:RAP. http://www.emrap.org/episode/february2015/theannalsof. Published February 2015.

Author information

Jeff Riddell, MD

Assistant Professor of Clinical Emergency Medicine
Co-Director, Medical Education Fellowship
LA County + USC Emergency Medicine Residency, Department of Emergency Medicine
Keck School of Medicine of the University of Southern California

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