Can you identify this sunburn-like rash?
Dermclinic
A Photo Quiz to Hone dermatologic Skills
CASE: A previously healthy 28-month-old girl presented with complaints of a rash. The rash had started 4 days earlier in her vaginal area. Her parents described the rash as severe and red initially, and they had treated it at first with diaper cream. The parents denied any fever in the child.
Three days before presentation, the father noted that the rash had a sunburned appearance and had spread to the girl’s neck and perioral area. At that time, both parents thought it was a food allergy rash, since the patient’s older brother has a history of food allergies.
Two days before presentation, the child had become irritable, with poor oral intake and worsening rash. Her parents brought her to a hospital emergency department, where the girl was treated with intravenous fluids. Later that afternoon, the rash worsened further, and blisters formed around her mouth, axillae, and vaginal area. The rash progressed to areas of desquamation on her back and genital areas.
What is this child’s condition?
A. Stevens-Johnson syndrome
B. Staphylococcal scalded skin syndrome
C. Erythema multiforme
D. Toxic epidermal necrolysis
(Answer and discussion begin on next page.)
ANSWER: B, staphylococcal scalded skin syndrome
The child was taken to her pediatrician, who diagnosed staphy-lococcal scalded skin syndrome (SSSS). The girl was sent home, and the family was advised to keep her well hydrated. The following morning, the child was seen by a dermatologist, who referred her to our hospital for evaluation and management of dehydration and worsening irritability.
PHYSICAL EXAMINATION
On admission to our facility, review of systems was negative for fever, chills, upper respiratory infection symptoms, nausea, vomiting, and diarrhea. The girl’s pulse was 118 beats per minute, respiratory rate was 22 breaths per minute, blood pressure was 94/100 mm Hg, oxygen saturation via pulse oximetry was 100% on room air, and weight was 10.5 kg (fifth percentile).
The child appeared nontoxic but uncomfortable and fussy, and was crying but consolable. HEENT examination reveled dry lips, intact mucous membranes, mucopurulent secretions bilaterally from the eyes, and normal tympanic membranes. Her neck was supple. Cardiovascular examination showed normal first and second heart sounds and regular rhythm with no murmurs, rubs, or gallops. The lungs were clear to auscultation bilaterally; the abdomen was soft, nontender, and nondistended, with no masses on palpation and normal bowel sounds. No lymphadenopathy was appreciated on palpation of lymphatics.
Skin examination showed an erythematous, macular rash over all of the body except for the lower extremities. Desquamation was noted on the back and the genital area. The rash was especially prominent on the face, trunk, axillae, vagina, and upper extremities. The Nikolsky sign (denuding of the skin at sites of gentle pressure) was present.
HOSPITAL COURSE
On admission, an intravenous normal saline fluid bolus was started, followed by maintenance intravenous fluids. Ear, eye, throat, genital, and blood cultures were obtained. The patient was given intravenous vancomycin, oral acetaminophen, intravenous morphine, and topical mupirocin ointment.
The next day, the child was seen by an ophthalmologist and a dermatologist. The ophthalmologist thought she had blepharitis secondary to SSSS and recommended erythromycin ointment on the eyelids, which then was started. The dermatologist agreed with the diagnosis of SSSS and advised that the mupirocin ointment be continued.
The ear culture grew coagulase-negative staphylococci, the eye culture was positive for Moraxella catarrhalis, and the throat culture showed heavy growth of Staphylococcus aureus. The genital culture showed normal flora, and blood cultures showed no growth. After sensitivity testing was performed, intravenous vancomycin was switched to oral trimethoprim-sulfamethoxazole.
When oral intake had become adequate and intravenous morphine was no longer required, the patient was discharged. Oral trimethoprim-sulfamethoxazole, topical mupirocin ointment, erythromycin ophthalmic ointment, and acetaminophen as needed, were continued, and the girl did well.
DISCUSSION
SSSS, also known as Ritter disease, is characterized by a blistering, desquamating rash.1 It is caused by epidermolytic or exfoliative toxins that are produced by S aureus and that target cell-to-cell adhesion molecules in desmosomes of the superficial epidermis.3 SSSS most often is seen in otherwise healthy neonates and children younger than 5 years of age because of their lack of antitoxin antibodies and poor renal clearance of toxins.1
SSSS usually originates with an S aureus infection of the conjunctivae, nares, throat, perineum, umbilicus, or blood.2 The result is a spectrum of disease, from localized bullous impetigo to widespread cutaneous involvement and systemic illness. Fever, malaise, lethargy, irritability, and tenderness of the skin usually are the first symptoms; these are followed by a diffuse scarlatiniform erythema that is most marked in the flexural and perioral areas. The erythema usually progresses to large, fragile bullae, and fissuring and crusting of the lips and around the eyes and nose often occurs. The blisters desquamate around days 2 to 5, and the Nikolsky sign is present. Healing occurs in approximately 10 to 14 days, and because of the superficial cleavage level, usually no scarring occurs.3
The diagnosis of SSSS most often is based on clinical presentation but can be confirmed by isolation of S aureus. The bullae almost always are sterile, so cultures should be obtained from all possible sources of infection and from the blood.
Differential diagnoses may include Stevens-Johnson syndrome, erythema multiforme (EM), and toxic epidermal necrolysis (TEN). Cases of Stevens-Johnson syndrome and TEN usually are characterized by a triad of mucosal lesions, target lesions, and epidermal necrosis with skin detachment.4 EM usually occurs in adults aged 20 to 40 years and presents with symmetric, round maculopapular lesions that may progress to target lesions. EM can occur in crops of lesions for 2 to 4 weeks.1 In SSSS, the cleavage level is in the granular layer of the superficial epidermis, as opposed to TEN, which cleaves deeper at the dermal-epidermal junction.2 If necessary, SSSS can be differentiated from Stevens-Johnson syndrome, TEN, and EM by skin biopsy.
The main treatment usually is antibiotic therapy with a penicillinase-resistant penicillin, first- or second-generation cephalosporin, or clindamycin, which may inhibit the toxin synthesis.2,3 Therapy should be modified if sensitivities are available or if in areas with a high incidence of clindamycin-resistant, methicillin-resistant S aureus, which was the case for our patient. Children who are older or who have with mild disease may be treated as outpatients. Infants or those with more extensive skin involvement should be hospitalized; treatment in these cases is aimed at maintaining fluid and electrolyte balance, pain management, and skin care.2
REFERENCES:
1. Habif TP. Bacterial infections. In: Clinical Dermatology: A Color Guide to Diagnosis and Therapy. 5th ed. Philadelphia, PA: Elsevier; 2010:360-362.
2. Paller AS, Mancini AJ. Bacterial, mycobacterial, and protozoal infections of the skin. In: Hurwitz Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence. 4th ed. Philadelphia, PA: Elsevier Saunders; 2011:321-347.
3. Morelli JG. Staphylococcal scalded skin syndrome (Ritter disease). In: Kliegman RM, Stanton BF, St. Geme J, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011:2302-2303.
4. Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol. 1993;129(1):92-96.