Dermatology Quiz and Case Discussion
- Anthony J. Mancini, MD
- Head, Dermatology, Ann & Robert H. Lurie Children's Hospital of Chicago; Professor of Pediatrics and Dermatology, Northwestern University Feinberg School of Medicine
- Disclosure: Dr. Mancini has no industry relationships to disclose and does not refer to products that are still investigational or not labeled for the use in discussion.
- Sapna Patel Vaghani, MD
- Former Fellow, Pediatric Dermatology, Ann & Robert H. Lurie Children’s Hospital of Chicago
- Disclosure: Dr. Vaghani has no industry relationships to disclose and does not refer to products that are still investigational or not labeled for the use in discussion.
Other Disclosure Information
At the conclusion of this activity, participants will be able to:
- Recognize the disorder described in the vignette and shown in the photographs
- Describe the clinical features and causes of the condition
- Discuss the approach to diagnosis and treatment
Estimated time to complete:
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- Correctly answer at least 70% of questions on the quiz and answer evaluation questions.
A previously healthy 3-year-old girl presents with mild rhinorrhea and decreased oral intake, as well as periorbital and perioral desquamation and fissuring (Figures 1, 2), with superficial erosions and sheets of desquamation on the neck, axillary vaults (Figure 3), and upper trunk. The left neck has a focal hemorrhagic plaque with surrounding heme crust. Her left chest has multiple pinpoint-to-1 mm clear vesicles. The labia majorae and gluteal crease (Figure 4) have superficial desquamation, but all mucosal surfaces are clear, including her conjunctivae. Her pediatrician recently started her on ibuprofen, sulfacetamide eye drops, and oral Bactrim.
1. What is the most likely diagnosis?
A. Kawasaki disease
B. Toxic epidermal necrolysis
C. Staphylococcal scalded skin syndrome
D. Stevens-Johnson syndrome
2. Diagnosis is best confirmed by:
A. Gram stain and culture of pyogenic areas
B. Punch biopsy
C. Antistreptolysin O (ASO) titer
D. Blood culture
3. The most likely causative agent is:
A. Herpes simplex virus
C. Mycoplasma pneumonia
D. Staphylococcus aureus
Answers: 1C, 2A, 3D
Staphylococcal scalded skin syndrome (SSSS), previously known as pemphigus neonatorum or Ritter disease in newborns, describes a spectrum of superficial blistering skin eruptions caused by the epidermolytic toxin-producing Staphylococcus aureus. It primarily occurs in neonates and young children, with 62% of cases occurring before the age of 2 years. Adult cases are typically seen in the setting of immunosuppression, renal failure, malignancy, or diabetes.
The pathophysiology of SSSS is well described and stems from the production of epidermolytic (or exfoliative) toxins (ET), which are made by roughly 5% of all S. aureus organisms. Two main serotypes of toxin, ETA and ETB, affect humans and target desmoglein 1 (DG-1), an important cell-to-cell adhesion molecule found within the stratum granulosum. This is the same structure that is targeted in the autoimmune blistering disease, pemphigus foliaceous. Cleavage within this level of the epidermis results in the superficial desquamation seen in both entities. The relative quantity of DG-1 decreases with age, which partially explains the increased frequency of SSSS in neonates and young children. In addition, the immature renal function in this age group results in decreased glomerular clearance of the toxin and also likely contributes to more extensive disease in younger patients.
SSSS usually starts as a localized infection of the conjunctivae, nares, perioral region, perineum, or umbilicus. Other infections, such as septic arthritis, endocarditis, and pneumonia can occasionally serve as a nidus. Malaise, fever, irritability, and skin tenderness may be present; however, many patients are non-toxic in appearance. The toxins are spread via the hematogenous route and act at remote sites, causing erythema that progresses to large and superficial fragile bullae that easily rupture, leaving behind tender and denuded skin. Blisters progress with application of gentle pressure to the edge of the bulla, known as the Nikolsky sign. Subsequent desquamation, more pronounced in flexural areas, is typically sheet-like, and separation of crust in the perioral areas often leaves behind classic radial fissures. If extensive, the denudation can lead to significant fluid loss, electrolyte imbalance, and difficulties with thermoregulation. Patients are at risk for secondary infection and sepsis.
The clinical presentation typically leads to the diagnosis; however, other entities to consider are toxic epidermal necrolysis (TEN), nutritional deficiency, epidermolysis bullosa and burns. TEN is a drug-induced exfoliative process that involves full thickness necrosis of the epidermis and which is associated with a high mortality rate. In TEN, however, mucosal involvement is prominent, helping to distinguish it from SSSS. In the neonate, bullous ichthyosis is another diagnostic consideration. Bullous impetigo is also caused by staphylococcal exfoliative toxin; however, in this entity, the toxin is confined to the focal areas of skin infection and bacteria can be cultured from blister contents.
Isolation of S. aureus confirms the diagnosis of SSSS, but the majority of blisters and denuded areas are sterile. The best yield for gram stain and culture is from pyogenic, not exfoliative, areas of involvement. A polymerase chain reaction (PCR) serum test for the toxin is available, but the cost and logistical difficulties of this study are often prohibitive. Although blood cultures are usually positive in adult cases, they are typically negative in children.
Treatment hinges upon eradication of the toxin-producing S. aureus, and consideration should be given for the rapidly increasing rates of community-acquired methicillin resistant S. aureus (CA-MRSA). Depending on sensitivities, a penicillinase resistant penicillin, 1st or 2nd generation cephalosporin, or clindamycin are appropriate antibiotics. Clindamycin is often used for its ability to inhibit bacterial ribosomal production of the ET. In cases of MRSA, parenteral vancomycin may be indicated. For neonates and children with severe infection, hospitalization is mandatory, with close attention to fluid and electrolyte status. Older children who are non-toxic can often be treated in the ambulatory setting, with close clinical follow-up. In the future, neutralizing antibodies that prevent the ETs from binding to DG-1 may become available.
For Further Reading:
- Amagai M, Yamaguchi T, Hanakawa Y, Nishifuji K, Sugai M, Stanley JR. Staphylococcal exfoliative toxin B specifically cleaves desmoglein 1. J Invest Dermatol. May 2002;118(5):845-850.
- Gemmell CG: Staphylococcal scalded skin syndrome. J Med Microbiol 1995;43:318-327.
- Hubiche T, Bes M, Roudiere L, Langlaude F, Etienne J, Del Giudice P. Mild staphylococcal scalded skin syndrome: an underdiagnosed clinical disorder. Br J Dermatol. Jan 2012;166(1):213-215.
- Paller AS, Mancini AJ. Bacterial, Mycobacterial, & Protozoal Infections of the Skin. In: Bonnett C, Gabbedy R, Mortimer A, eds. Hurwitz Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence. Edinburgh: Elsevier Saunders; 2011:331-333.
- Patel GK, Finlay AY. Staphylococcal scalded skin syndrome: diagnosis and management. Am J Clin Dermatol. 2003;4(3):165-175.