Scalded skin syndrome is a skin disease characterized by the abrupt onset of diffuse superficial desquamating blisters caused by exfoliative toxins released by staphylococcus aureus.

Five months after being struck by lightning, a 55-year-old male presented with a 2-day history of an erythematous rash. The patient noted his skin had been excessively dry since the lightning strike, and he recently added a new essential oil to his bath. His medical history includes liver failure, asthma, environmental allergies, and the lightning strike about five months ago. A review of systems revealed intermittent fevers, joint pains, generalized skin dryness, facial swelling, and abdominal pain. Pertinent laboratory findings included an elevated white blood cell count (27.25K/uL), elevated procalcitonin count (6.26ng/mL), elevated erythrocyte sedimentation rate, (84mm/Hr), elevated lactate (4.52mmol/L), and a positive blood culture for staphylococcus aureus. Physical examination revealed sloughing of skin with erythema, superficial ulcers, and edema around the neck, bilateral hands, and upper chest, with greenish weeping in the inframammary, axillary, and inguinal folds. Additionally, bullae formation was noted with multiple ulcers of bilateral feet, with erythema extending up to the thighs. Biopsy revealed diffuse parakeratosis with focal intracorneal splitting/bullae, as well as intense neutrophilic inflammation within the epidermis, within the intracorneal blister, and superficial dermis. Additionally, the biopsy revealed spongiosis of the epidermis with minimal apoptotic keratinocytes. The patient was diagnosed with Staphylococcal scalded skin syndrome (SSS). 

SSS is a skin disease characterized by the abrupt onset of diffuse superficial desquamating blisters caused by exfoliative toxins released by staphylococcus aureus. Although it is fairly common in children, its epidemiology is not as well-known in adults. It is relatively rare for adults to develop SSS except in cases where they are immunocompromised or have other comorbidities such as significant kidney diseases. This is because immunocompetent adults have antibodies against the exotoxins and are sufficient for renal clearance of the toxins¹. Our patient was in liver failure, and this could have hindered his ability to adequately clear the initial staphylococcal aureus infection².Adults typically begin with the same symptoms as children including irritability and fever before developing bullae-type blisters within 48 hours of onset of symptoms. These blisters will have a positive Nikolsky sign, or rupture of the blister when pressure is applied, which is a helpful clinical differentiator of dermatological diseases.^1,3

Skin biopsy is typically not required for diagnosis but can help to rule out differentials such as Steven Johnson Syndrome which is more common in adults than staphylococcal SSS, although clinical history can also usually rule this out¹. Biopsy typically shows cleavage beneath the stratum corneum in staphylococcal SSS, whereas the stratum corneum is not involved in Steven Johnson Syndrome⁴. Our patient’s biopsy revealed inflammation with intracorneal and epidermal involvement, which is also characteristic of staphylococcal SSS. Keratinized skin is targeted by staphylococcus aureus and therefore is also helpful in ruling out SJS⁴.

First-line treatment for staphylococcal scalded skin syndrome in adults is a penicillin or a first- or second-generation cephalosporin. Vancomycin should be considered for methicillin-resistant staphylococcus aureus infections.  Additionally, supportive care with IV fluids is critical to manage fluid loss along with electrolyte replenishment as the exotoxins are excreted through the kidneys. Wound care must be included in care to prevent secondary infections. Prognosis in adults is typically much worse (60% mortality rate) than in children even with prompt treatment, due to the comorbidities associated with contracting staphylococcal SSS¹.

 CAPTION: Figure. The left, Patient's neck showing sloughing of skin with erythema and edema. Right, histopathologic examination of excised tissue showing diffuse parakeratosis with focal intracorneal splitting/bullae, intense neutrophilic inflammation within the epidermis, within the intracorneal blister, and superficial dermis, and spongiosis of the epidermis with minimal apoptotic keratinocytes (hematoxylin-eosin, original magnification x 10).

About the authors

Rachel Parks, resident, Department of Internal Medicine, Section of Dermatology, Louisiana State University Health Shreveport, Shreveport, LA.

Elise Thibodeaux, medical student, Department of Internal Medicine, Section of Dermatology, Louisiana State University Health Shreveport, Shreveport, LA.Bohdan Zoshchuk, MD, Department of Internal Medicine, Section of Pathology, Louisiana State University Health Shreveport, Shreveport, LA. Christopher Haas, MD, Department of Internal Medicine, Section of Dermatology, Louisiana State University Health Shreveport, Shreveport, LA.

REFERENCES

1. Brazel M, Desai A, Are A, Motaparthi K. Staphylococcal Scalded Skin Syndrome and Bullous Impetigo. Medicina (Kaunas). 2021 Oct 24;57(11). 
2. Kang CI, Song JH, Ko KS, Chung DR, Peck KR. Clinical significance of Staphylococcus aureus infection in patients with chronic liver diseases. Liver Int. 2010 Oct;30(9):1333-8. 
3. Channual J, Wu JJ. The Nikolskiy Sign. Arch Dermatol. 2008;144(9):1140. 
4. Nguyen QD, Vu MN, Hebert AA. Recognizing and Managing Staphylococcal Scalded Skin Syndrome in the Emergency Department. Pediatr Emerg Care. 2022 Mar 1;38(3):133-135.