Staphylococcal Scaled Skin Syndrome

Introduction

Definition

It is a syndrome consisting of skin blistering disorder caused due to exfoliative toxins A & B of Staphylococcus aureus.

Age groups affected: It affects primarily neonates, infants, and young children with occasional reported cases in adults.^1,2 The generalized form in the neonates is known as "Ritter's Disease". This often happens due to colonization of the child during the passage through the maternal vaginal flora.¹

Symptoms & Signs

The symptoms may range from fever, erythema initially with the formation of large superficial localized blisters to generalized exfoliation of the whole body.^1,2

The localized form, which is the mildest form of the disease, is known as Bullous Impetigo, measles pemphigoid, or bullous varicella. They form large superficial thin formed bullae which rupture and leave denuded skin behind after oozing fluid which varies from thin, serous liquid to purulent pus. They are more common around the extremities in the older children and in the periumbilical area in the neonates.¹

In the generalized form, the whole skin is involved often leaving the mucous membranes. They present with generalized fever, malaise, feeding difficulties, and irritability which is followed by a rash starting on the head and spreading to the whole body in days. This leads to the formation of the multiple blisters all over the body which ruptures leaving large areas of denuded skin. They also have temperature irregulation and very often have secondary bacterial infection leading to hypotension, tachycardia, and respiratory distress.¹

The ghastly appearance makes the patient and the parents extremely anxious and tense.²

Unusual presentations have been reported like recurrent episodes in neonates especially very low birth weight babies.³

Diffential Diagnosis

Bullous impetigo, chemical burns, drug or viral-induced toxic epidermal necrolysis, epidermolysis bullosa, bullous mastocytosis and neonatal pemphigus.³

Diagnosis

S. aureus isolated from the skin lesions and blood samples & the exfoliative toxin can also be identified by various methods like the PCR, radioimmunoassay, etc.^2,3 A biopsy of the blister is one of the most definitive diagnostic tests in SSSS.¹ However, this is not always possible due to the limited availability of the tests and the time-consuming factor. So, the diagnosis is often clinical and confirmation is made after a favorable response to anti-staphylococcal medications.^1,2

Treatment

It responds very well to antibiotic therapy.³ Any secondary symptoms can be treated symptomatically.¹ Regimens used are cloxacillin plus tobramycin and erythromycin.¹ Other oral antibiotics effective against penicillin-resistant staphylococcus can be used as well. Neonates should be isolated and the skin lesion must be treated with care like burn wounds. Nasal carriers must be identified by swabs and treated to prevent future outbreaks.¹