An 18-month-old girl is brought to the office by her parents due to a rash and blisters that started this morning. Yesterday, the child was very fussy, particularly when her parents picked her up. The patient also had a fever that responded well to ibuprofen. This morning, the parents noted erythema and peeling skin around her mouth, axillae, and inguinal areas. Medical history is significant for otitis media 6 months ago. Vaccinations are up to date, and the patient takes no daily medications. Temperature is 39 C (102.2 F), blood pressure is 89/52 mm Hg, pulse is 145/min, and respirations are 28/min. Examination reveals an irritable child who cries with any attempt to move her. Superficial flaccid bullae and an erythematous rash are diffusely distributed over the body. Slight focal pressure to the back results in sloughing of the top layer of skin with underlying erythema. The face appears edematous, with perioral crusting. A single pustule is noted under the left naris, but no mucosal lesions are seen. Which of the following is the most likely diagnosis in this patient?

Question

An 18-month-old girl is brought to the office by her parents due to a rash and blisters that started this morning.  Yesterday, the child was very fussy, particularly when her parents picked her up.  The patient also had a fever that responded well to ibuprofen.  This morning, the parents noted erythema and peeling skin around her mouth, axillae, and inguinal areas.  Medical history is significant for otitis media 6 months ago.  Vaccinations are up to date, and the patient takes no daily medications.  Temperature is 39 C (102.2 F), blood pressure is 89/52 mm Hg, pulse is 145/min, and respirations are 28/min.  Examination reveals an irritable child who cries with any attempt to move her.  Superficial flaccid bullae and an erythematous rash are diffusely distributed over the body.  Slight focal pressure to the back results in sloughing of the top layer of skin with underlying erythema.  The face appears edematous, with perioral crusting.  A single pustule is noted under the left naris, but no mucosal lesions are seen.  Which of the following is the most likely diagnosis in this patient?

Quizplus · Accepted Answer

11ec25c8_d64c_d93f_bc81_9db8cdbcdba5_MD0003_00 Staphylococcal scalded skin syndrome (SSSS) is a blistering skin condition most commonly found in infants and young children. The pathogenesis involves infection with certain strains of Staphylococcus aureus that produce exfoliative toxins, which disrupt keratinocyte adhesion in the superficial epidermis. Clinical presentation begins with a prodrome of fever, irritability, and skin tenderness. Generalized erythema is then followed by flaccid blisters/bullae located predominantly in flexural areas exposed to mechanical pressure (eg, axillae, groin). Although perioral crusting can occur, mucosal surfaces are typically spared. Gentle pressure on the skin surface causes detachment of a superficial layer of skin (ie, Nikolsky sign), and epidermal shedding exposes underlying erythematous skin with a scalded appearance. In neonates, the source of infection is often the umbilicus or circumcision site, whereas in older children, there may be nasopharyngeal colonization or a primary skin lesion (eg, pustule), as seen in this patient. Cultures from the blood and potential infectious sources are obtained; however, the diagnosis is generally clinical, and intact bullae are usually sterile. Treatment involves antistaphylococcal antibiotics (eg, nafcillin, vancomycin) and supportive wound care for denuded areas. The mortality rate is low in children, and symptoms typically resolve in 1-2 weeks. (Choice A) Bullous impetigo is a localized form of SSSS also caused by exfoliative toxin-producing S aureus. Patients typically appear well, with blisters confined to the primary area of infection. In addition, when ruptured, the blisters leave a distinct honey-colored crust, which is not seen in this patient. Bullous impetigo (Choice B) Erysipelas is a bacterial infection (eg, group A Streptococcus) of the dermis that presents with a well-demarcated, warm, tender area of erythema. Overlying bullae can develop in severe cases, and patients often have constitutional symptoms (eg, fever, chills). However, diffuse rash and shedding do not occur. Erysipelas (Choice C) Erythema multiforme is a self-limited reaction to certain infections (eg, herpes simplex virus) that manifests as targetoid lesions. The lesions may have central bullae, but Nikolsky sign is negative. Erythema multiforme (Choice D) Scarlet fever, caused by erythrogenic toxin-producing strains of group A Streptococcus, presents with a fine, pink, sandpaper-like rash following an episode of pharyngitis in children. The rash is prominent in flexural areas and often desquamates but does not cause blistering or a positive Nikolsky sign. Scarlet fever (Choice F) Toxic epidermal necrolysis manifests as extensive (>30% body surface area) skin blistering and erosion; it is typically triggered by a medication. Nikolsky sign is positive; however, mucous membrane involvement is expected with this condition but is not seen in this patient. Toxic epidermal necrolysis Educational objective: Staphylococcal scalded skin syndrome is caused by exfoliative toxin-producing strains of Staphylococcus aureus and typically occurs in infants and young children. A prodrome of fever, irritability, and skin tenderness is followed by generalized erythema, superficial flaccid blisters, and epidermal shedding with light pressure (ie, Nikolsky sign). References: Staphylococcal scalded skin syndrome: diagnosis and management in children and adults. (http://www.ncbi.nlm.nih.gov/pubmed/24841497)

An 18-Month-Old Girl Is Brought to the Office by Her