SSSS

This is an interesting case from Ipoh, Malaysia. A large group practice like FMI will see a child with Staphyloccal Scalded Skin Syndrome occasionally. Dermatologist Henry Foong sent the pictures and the history.

HPI: This 11 mo old infant presented with symmetrical and bilateral dry scaly eruptions around the eyes and mouth for 3 days. Apparently it started with periorbital eruptions after taking some chocolates. The mother had thought it was allergy to chocolates. He has seen at least one pediatrician and a general practitioner before consulting us. There was a history of asthma. 3 other siblings had impetigo recently.

On examination, he appeared fretful. He had a low grade fever temp 37.4 degC. Marked diffuse scaling and erythema on both perioral and periorbital areas were noted bilaterally and symmetrically. The neck and axilla were similarly affected. Diffuse erythema and flushing was noted on the trunk.

Clinical Photos:

Note: The child appears irritable and uncomfortable with exudations and crusting around the mouth and eyes. Skin globally is erythematous. Healing with appropriate antibiotics is complete within 14 days.
Diagnosis: Clinically he has staphylococcal scalded skin syndrome

Lab: TWBC 12,000 N50 L36 E1 M11
Culture was negative.

Course: He was admitted to the ward and treated with IV fluids, IV cefuroxime 150mg tid and topical moisturisers. He recovered well and discharged 3 days later. Plan to do an ASOT on follow up as culture was unhelpful.

Reason(s) Presented: For interest. This is a classic case; not sure why culture was negative except that patient may have had antibiotics before it was taken.

Reference: Patel GK. Treatment of staphylococcal scalded skin syndrome. Expert Rev Anti Infect Ther. 2004 Aug;2(4):575-87.
Humans are a natural reservoir for Staphylococcal aureus. Colonization begins soon after birth and predisposes to infection. S. aureus is one of the most common causes of skin infection, giving rise to folliculitis, furunculosis, carbuncles, ecthyma, impetigo, cellulitis and abscesses. In addition, S. aureus may cause a number of toxin-mediated life-threatening diseases, including staphylococcal scalded skin syndrome (SSSS). Epidermolytic toxins released by certain S. aureus strains cause SSSS by cleaving the epidermal cell adhesion molecule, desmogelin-1, resulting in superficial skin erosion. Recent experiments have revealed similarities in the pathophysiology of SSSS and pemphigus foliaceus, an autoimmune disorder that is characterized by antibodies targeting the same epidermal attachment protein. SSSS typically affects neonates and infants but may also occur in predisposed adults. It is painful and distressing for the patient and parents, although most cases respond to antibiotic treatment. Mortality is low in infants but can be as high as 67% in adults, and is dependent on the extent of skin involvement and the comorbid state. Thus, the management of adults who develop SSSS remains a major therapeutic challenge. The antibody response against the toxins neutralizes their effect and prevents recurrence or limits the effects to the area of infection, which is known as bullous impetigo.