Dermatologic emergencies

Chapter 64 Dermatologic emergencies

1. “Dermatologic emergencies” sounds like an oxymoron. Are there dermatologic emergencies?

Yes. Several groups of diseases in dermatology are emergencies. In some, the skin is the primary organ affected (e.g., pemphigus vulgaris) and, in others, the cutaneous manifestations are an important diagnostic finding of a severe underlying condition (e.g., meningococcemia). Rapid recognition and diagnosis of dermatologic emergencies are important because these conditions are often acutely lethal but can be treated successfully if the diagnosis is made early in the disease course.

Vesiculobullous disorders and drug reactions

3. How does toxic epidermal necrolysis differ from the Stevens-Johnson syndrome or erythema multiforme major?

Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome are commonly confused entities, in part because many clinicians use the two terms interchangeably. Because these two diseases have significantly different prognoses and treatments, it is important to differentiate between them (Table 64-1). The diseases can usually be distinguished by their clinical presentation (Fig. 64-1), histologic findings, and course.

Table 64-1. Clinicopathologic Features of Toxic Epidermal Necrolysis (TEN) versus Stevens-Johnson Syndrome (SJS)

	TEN	SJS
Maximal intensity	1–3 days	7–15 days
Skin pain	Severe	Minimal
Mucosal involvement	Mild	Severe
Lesional pattern	Diffuse erythema, desquamation	Annular and targetoid lesions
Skin histology	Few inflammatory cells	Numerous inflammatory cells
Prognosis	Poor	Excellent

Figure 64-1. A, Stevens-Johnson syndrome demonstrating typical mucosal inflammation of the mouth, lips, and conjunctiva. B, Fatal case of captopril-induced toxic epidermal necrolysis showing violaceous discoloration with sheets of epidermis peeling away from the skin.

(Courtesy of James E. Fitzpatrick, MD.)

The relationship between TEN and Stevens-Johnson syndrome is one of the great controversies in dermatology. Some in vitro research suggests that they are different diseases based on pathogenic mechanisms, but some authorities regard TEN as a more severe form of Stevens-Johnson syndrome. It is universally accepted that Stevens-Johnson syndrome is a more severe form of erythema multiforme.

Wolf R, Orion E, Marcos B, Matz H: Life-threatening acute adverse cutaneous drug reactions, Clin Dermatol 23:171–181, 2005.

4. How do you treat TEN?

Patients with TEN should be treated as burn patients, with supportive care to maintain fluid balance, avoid infection, and prevent adult respiratory distress syndrome (ARDS). In essence, TEN patients should be treated as severe burn victims. There are in vitro data to suggest that this entity may be the result of direct toxicity of drugs, or their metabolites, to skin cells. Anecdotal reports suggest that systemic corticosteroids are actually detrimental to TEN patients, although others continue to advocate their use. Initial anecdotal reports suggested that intravenous immunoglobulin therapy improved survival; however, this is controversial because other reports have not demonstrated efficacy.

5. How do you treat Stevens-Johnson syndrome?

Stevens-Johnson syndrome appears to be immunologically mediated and responds to systemic corticosteroids. Patients with severe symptoms, especially involvement of the oral mucosa, which interferes with eating and fluid intake, may be treated with a short trial of systemic corticosteroids. Stevens-Johnson syndrome is usually a self-limited disease with a 3-week course, and milder cases may not require systemic corticosteroids. Some authorities consider the use of systemic corticosteroids in this condition as controversial, because there are no good prospective, controlled studies evaluating this treatment. Therefore, an empirical 3- to 4-day trial of high-dose systemic steroids should be considered. If there is no clinical improvement at that time, the treatment should be discontinued.

6. What is pemphigus vulgaris?

Pemphigus vulgaris is a superficial blistering disease that typically affects middle-aged individuals (Fig. 64-2). It often presents initially with mouth ulcerations (60% of cases) but can involve blistering on areas above the waist. Pemphigus vulgaris may present acutely and, in severe cases, may resemble TEN or Stevens-Johnson syndrome. Early diagnosis is important because this condition is usually fatal if untreated, and current therapies are effective.