Derm emergencies— detecting early signs of trouble

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Applied Evidence

Derm emergencies— detecting early signs of trouble

J Fam Pract. 2012 February;61(2):71-78

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References

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17. Wang S, Best BM, Burns JC. Periungual desquamation in patients with Kawasaki disease. Pediatr Infect Dis J. 2009;28:538-539.

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CORRESPONDENCE Stephen A. Martin, MD, EdM, Barre Family Health Center, 151 Worcester Road, Barre, MA 01005; stephen.martin@umassmemorial.org

Desquamation/blistering: Act quickly when it’s widespread

Although desquamation can be seen in benign skin conditions, widespread desquamation with or without bullae requires careful evaluation and a rapid response. Separation, either at the dermal-epidermal junction or intraepidermally, raises the specter of 2 emergent conditions: the Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) spectrum and staphylococcal scalded skin syndrome (SSSS). Mucosal involvement is another red flag, if the patient appears ill and the desquamation is progressing rapidly. Conjunctival involvement, in particular, is associated with greater morbidity, and a consult with an ophthalmologist is prude

FAST TRACK

Desquamation with conjunctival involvement, in particular, is associated with greater morbidity, and a consultation with an ophthalmologist is prudent.

Rapid progression is a hallmark of SJS/TEN. Desquamation that’s widespread and rapidly progressive in a patient with fever, hypotension, and end-organ involvement is suggestive of SJS/TEN (FIGURE 3). Medications, including allopurinol, antimicrobials, and antiepileptics, are frequent culprits.^10,11

Nonsteroidal anti-inflammatory drugs (NSAIDs) have also been linked to SJS/TEN.¹⁰ Given their widespread use (1-2 per million users per week), however, the likelihood of NSAIDs leading to SJS/TEN is exceedingly low.¹²

Signs and symptoms of SJS/TEN may include target lesions with dusky centers, erythroderma, or significant pain without any visible skin abnormality, typically accompanied by fever and malaise. Widespread sloughing of the skin may be seen within several hours.¹¹

Admission to an intensive care unit—preferably a burn unit—is suggested for aggressive fluid resuscitation and management of shock and end-organ dysfunction. Intravenous immunoglobulin G (IVIG) and steroids are often used, although there is little consensus as to the most effective treatment.¹³ Mortality from TEN approaches 50%.¹³

SSSS can present at any age. Newborns often present with SSSS during their first week of life: Widespread erythema is quickly followed by fragile blisters, which may have already ruptured by the time the infant receives medical attention. Mucosal surfaces are not typically involved. Nikolsky’s sign (separation of the upper epidermis with gentle pressure) is a classic finding.

Infants with SSSS are frequently irritable, suggesting that the skin may be painful. Cultures from unruptured bullae will be negative as the blisters represent a cutaneous reaction to an infection, rather than a skin infection, but blood, urine, and nasopharynx cultures may be positive. Systemic treatment with nafcillin or oxacillin should be initiated, and supportive skin care provided.^14,15 Clindamycin or vancomycin should be used in parts of the country in which methicillin-resistant Staphylococcus aureus is prevalent. In very young infants, the outcome of SSSS is generally favorable. Not so with adults.

Because mature kidneys have a greater ability to excrete exfoliative toxins, SSSS primarily affects adults with significant comorbidities—and has a much poorer prognosis.¹⁶ You may also see chronic autoimmune bullous diseases, such as bullous pemphigoid and pemphigus vulgaris, with widespread desquamation and blistering, in the adult population. Untreated, the secondary infection and electrolyte disturbances from fluid loss associated with pemphigus vulgaris can be fatal.

Desquamation is a late finding in Kawasaki disease. Desquamation is often cited as a potential skin finding in children with Kawasaki disease (KD) (FIGURE 4), but usually not until the convalescent stage.¹⁷ (Desquamation may also appear during the recovery period of several other infections, including scarlet fever and TSS.) IVIG can prevent coronary aneurysm, the major complication of KD, but only if it is administered during the acute phase of the illness. Therefore, early diagnosis of KD (TABLE 2)¹⁸—before desquamation occurs—is critical.¹⁹

FIGURE 3
Desquamation, full-thickness epidermal necrosis on the upper back

Erythroderma and widespread denudation on the upper back of a patient who was given a diagnosis of toxic epidermal necrolysis.

FIGURE 4
Desquamation on a young patient

Desquamation associated with Kawasaki disease (shown on the hand of a child) usually occurs during the convalescent stage.

TABLE 2
Diagnostic criteria for Kawasaki disease

Fever for ≥5 days, and 4 out of 5 criteria (required):
Nonexudative bilateral conjunctivitis Mucosal fissuring, injection, or strawberry tongue Extremity erythema or edema Polymorphous rash Unilateral cervical lymphadenopathy (>1.5 cm diameter)
Supporting findings:
Thrombocytosis and other evidence of inflammation (elevated ESR, CRP) Sterile pyuria Transaminitis Hyponatremia
CRP, C-reactive protein; ESR, erythrocyte sedimentation rate. Source: Kawasaki Disease Research Committee. Pediatr Int. 2005.¹⁸

Skin pain is always a red flag

Widespread skin pain should always be taken seriously, as it is rarely associated with minor dermatoses.²⁰ Infectious cellulitis is the most likely diagnosis of a painful erythematous skin lesion. Patients with cellulitis do not usually have erythroderma, as the affected area tends to be very localized.

Cellulitis may be over- or undertreated. Once cellulitis has been diagnosed, the next thing to consider is severity. A recent retrospective study found that misclassification of skin and soft-tissue infections may result in both significant overtreatment of mild soft-tissue infections and dangerous undertreatment of severe infections, with consequent morbidity.²¹