A five year old boy presents with shoulder pain and a rash around his lips and on his neck
What could this be?
Our patient had staphylococcal scalded skin syndrome. He developed skin sloughing as shown below.
Scalded skin syndrome usually occurs in children under the age of six. It is also called Ritter’s disease. It is caused by a type of staph aureus which produces one of two exotoxins. The exotoxin targets desmoglein 1 in the top layer of epidermis resulting in exfoliation of the skin.
The disease usually follows a localized infection in the upper repiratory tract, ears, conjunctiva or umbilical stump. Children are at greater risk because of their immature renal system although It has also been reported in immunosuppressed adults. Topical antibiotics alone are not effective and cefazoin, nafcillin or oxacillin should be administered. Vancomycin should be administered if the patient has had recent health care exposure.
The Nikolsky sign is present and the upper layer of skin peels away with slight pressure in scalded skin syndrome. It is also present in Stevens Johnson syndrome and pemphigus vulgasis. It is not present in bullous pemphigoid.
DISEASES THAT CAN MIMIC SCALDED SKIN SYNDROME
Bullous Impetigo can look very similar to scalded skin syndrome but the exotoxins remain localized to the site of the infection and systemic infection does not occur. Staph and strep can cause it and often honey colored scabs form.
Impetigo is a localized infection
Scarlet fever- is caused by Group A strep and while it presents with erythema and a rash on the cheeks , axillae or trunk , bullae do not develop. Mucous membranes are involved and it begins with a sore throat
the strawberry tongue of scarlet fever
Kawasaki’s disease-causes inflammation in small to medium sized blood vessels. The cause is thought to be an abnormal immune response to infection. Desquamation of the tips of the fingers and toes may occur in the third week of illness.
Stevens Johnson syndrome/Toxic epidermal necrolysis is a spectrum of severity from 10% of body surface area to > 60% involvement. It is also a disease in which the skin exfoliates but there is necrosis of the entire epidermal layer instead of just the upper epidermis. There is often mucous membrane involvement with TEN which does not occur in scalded skin syndrome. The disease is often caused drug-induced with drugs such as sulfonamides, pyrazones, and antiepileptics being the most frequent triggers. There are genetically susceptible populations to TEN such as the Han Chinese who develop SJS/TEN from carbamazepine.
Toxic shock syndrome- fever, diffuse macular erythroderma, hypotension and multisystem involvement are necessary for the diagnosis. Desquamation occurs 1-2 weeks after the onset of the rash. It can be caused by either staph or strep. The majority of cases occur in women during menstruation mostly associated with tampon use which presumably cause small abrasions of the vaginal mucosa.
Our patient was treated with a cephalosporin and recovered uneventfully.
Chang L, Weng K, Yan J, et al. Desquamation in Kawasaki Disease. Children 2021 8(5), 317; https://doi.org/10.3390/children 8050317Moss C, Gupta E. The nikolsky sign in staphylococcal scalded skin syndrome. Arch Dis Child 79(3):290
Nikolski P. Materiali Kuchenigu o pemphigus foliaceus(doctoral thesis) Kiev. 1896.
Tiwari P, Panik R, Chandy A. Toxic epidermal necrolysis: an update. Asian Pac J Trop Dis 2013 Apr;3(2):85-92.
Gordon E. Treatment of scarlet fever with streptococcus antitoxin. 5 February 1927. JAMA The Journal of the American medical Association 88(6):382