Remember that erythema multiforme (EM) may be a sign of underlying hypersensitivity and may progress to Stevens-Johnson Syndrome (SJS), which can require aggressive fluid and skin care
Remember that erythema multiforme (EM) may be a sign of underlying hypersensitivity and may progress to Stevens-Johnson Syndrome (SJS), which can require aggressive fluid and skin care: Excerpt from Avoiding Common Pediatric Errors
Author:
Madan Dharmar, MD
What to Do - Interpret the Data
EM represents a syndrome of inflammatory skin eruptions that can manifest as a mild self-limiting form or as a life-threatening condition requiring
aggressive lifesaving and critical care therapies.
EM-minor, or the mild form of EM, is characterized by the occurrence
of characteristic skin lesions, which are described as target lesions, iris lesions, or iris erythema. The skin lesions follow a symmetric distribution and
commonly seen on the extensor surfaces of the extremities, back of hands,
feet, palms, and soles. The trunk and head are usually spared. Cutaneous
symptoms consist of itching, stinging, and burning sensations; occasionally,
general symptoms, such as mild fever and chill, are present.
The "erythema multiforme disease spectrum" comprises four distinct,
severe, clinical subvariants: (a) bullous erythema multiforme (bullous-EM),
(b) SJS, (c) SJS–toxic epidermal necrolysis (TEN)-overlap syndrome, and
(d) TEN.
EM-major or bullous EM, in addition to the characteristic target lesions
in symmetric distribution, consists of atypical, raised target lesions with
some degree of moderate epidermal detachment involving <10% of the
body surface.
In SJS, the cutaneous lesions are more extensive, with flat-atypical
targets and macules that predominate over classic target lesions, and predominately in the trunk and face. When epidermal necrolysis becomes a
dominant feature, with involvement of up to 10% of the body surface,
EM-major merges into SJS. Blistering of individual skin lesions as well as
erosions/detachment become delineating features. Moreover, these patients
develop severe and extensive mucosal lesions of the oral, conjunctival, and
genital mucous membranes, which then determine the course of the disease.
These lesions are painful and consist of desquamation, erosions, and superficial ulcerations. This disease is often complicated by extensive percutaneous
loss of electrolytes and fluids, and there is a high risk for scarring and milia
and synechia formation, especially on the eyes, upper gastrointestinal tract,
trachea, and genitalia. Patients are also at risk of serious bacterial infections
from their erosions.
In SJS/TEN-overlap, patients have widespread macules or flat targets
with 10% to 30% of the body surface area involved. TEN presents similarly to SJS, but involves at least 30% of body surface. EM, SJS, SJS/TEN
overlap, and TEN not only have similar clinical features but also are caused
by similar exposure to drugs or infection. The pathophysiological mechanisms in the diverse variants of the EM/SJS/TEN disease spectrum are
only partially understood. The immunopathologic pattern of early lesions
suggests a cell-mediated cytotoxic reaction against the epidermal cells with
the epidermis infiltrated by activated lymphocytes, mainly CD8 cells, and
macrophages. An immune reaction against drug-reactive metabolites produced in excess may be responsible. Because infiltrating cells are present
in only moderate numbers, it is unlikely that these cells are the principal
cause of epidermal necrosis. Cytokines, released by activated mononuclear
cells and keratinocytes, may contribute to local cell death, fever, and malaise.
The diagnosis of all of these disorders is primarily clinical, based on the
characteristic lesion on the skin and mucous membranes. Skin biopsy is also
helpful in making the diagnosis; it shows full thickness epidermal necrosis,
which is characteristic of the diseases.
Early recognition of the disease, immediate withdrawal of any potential
causative agent, and initiation of intravenous fluid replacement is essential
in the management of this disease. All patients must be examined to access
the extent of detachment of the epidermis and for an imbalance of fluid,
protein, and electrolyte homeostasis on a daily basis. Considering the massive loss of fluid via denuded skin, fluid resuscitation should be initiated
immediately after hospitalization, similar to burn victims. Any disturbance
should be adequately treated with electrolyte solutions, blood products (e.g.,
erythrocytes, fresh frozen plasma), and albumin. Surgical débridement and
whirlpool therapy are recommended to remove the necrotic epidermis. Regular ophthalmic examination and ophthalmic care using artificial tears or
lubricating ointment is recommended.
The management of SJS involves aggressive fluid and electrolyte management, nutritional support, pain control, and necessary precautions to
avoid super infections similar to the treatment for burn patients. Treatment
with corticosteroids is controversial because it might aggravate the condition or increase risk of secondary infections. Some consider the use of early
intravenous immunoglobulin to be of benefit, although to date, there has not
been a randomized control trial demonstrating its efficacy.
Suggested Readings
Bastuji-Garin S, Rzany B, Stern RS, et al. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol.
1993;129(1):92–96.
Marvin JA, Heimbach DM, Engrav LH, et al. Improved treatment of the Stevens-Johnson
syndrome. Arch Surg. 1984;119(5):601–605.
Roujeau JC, Stern RS. Severe adverse cutaneous reactions to drugs. N Engl J Med.
1994;(331):1272–1285.
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Book Source Details
- Book Title: Avoiding Common Pediatric Errors
- Author(s): Anthony D Slonim MD, DrPH; Lisa Marcucci MD
- Year of Publication: 2008
- Copyright Details: Avoiding Common Pediatric Errors, Copyright © 2008 Lippincott Williams & Wilkins.
More About Stevens-Johnson Syndrome
More Medical Textbooks Online about Stevens-Johnson Syndrome
Review other book chapters online related to Stevens-Johnson Syndrome:
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- Erythema
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- Stomatitis
- "The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter" (2000)
- [ read ]
- Erythema Multiforme
- "The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter" (2000)
- [ read ]
- Erythema
- "Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series" (2007)
- [ read ]
- Erythema
- "Signs & Symptoms: A 2-in-1 Reference for Nurses" (2007)
- [ read ]
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Avoiding Common Pediatric Errors
Authors: Anthony D Slonim MD, DrPH; Lisa Marcucci MD
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7489-6
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