A 45-year-old man comes to the office after sudden onset of a skin rash and oral lesions with fever. The patient is unable to eat or drink due to pain in his mouth and throat. His wife says that he had a headache, malaise, and joint pain prior to developing the skin lesions. The patient was in generally good health other than an episode of sinusitis for which he was prescribed trimethoprim-sulfamethoxazole 5 days ago. His temperature is 38.3 C (101 F), blood pressure is 110/80 mm Hg, pulse is 92/min, and respirations are 14/min. On examination, conjunctivae in both eyes are inflamed. The patient has extensive erosions involving the oral mucosa. There is also an erythematous, desquamating rash with scattered involvement of the trunk and proximal thighs, as shown in the image below. Which of the following is the most likely diagnosis?

Question

A 45-year-old man comes to the office after sudden onset of a skin rash and oral lesions with fever.  The patient is unable to eat or drink due to pain in his mouth and throat.  His wife says that he had a headache, malaise, and joint pain prior to developing the skin lesions.  The patient was in generally good health other than an episode of sinusitis for which he was prescribed trimethoprim-sulfamethoxazole 5 days ago.  His temperature is 38.3 C (101 F), blood pressure is 110/80 mm Hg, pulse is 92/min, and respirations are 14/min.  On examination, conjunctivae in both eyes are inflamed.  The patient has extensive erosions involving the oral mucosa.  There is also an erythematous, desquamating rash with scattered involvement of the trunk and proximal thighs, as shown in the image below.  Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

11ec25c8_d38d_9178_bc81_13450aedf409_MD0003_00 Stevens-Johnson syndrome (SJS) is a serious inflammatory reaction to drugs or certain infections (eg, Mycoplasma pneumoniae). Clinical features include coalescing erythematous macules, bullae, desquamation, and mucositis. Systemic signs are common and may include fever, tachycardia, hypotension, altered level of consciousness, seizures, and coma. By convention, SJS denotes <10% of body surface area involvement, >30% is designated as toxic epidermal necrolysis (TEN), and 10%-30% is SJS/TEN overlap. mucositis toxic epidermal necrolysis (TEN) The diagnosis of SJS is based on the typical mucocutaneous lesions, systemic signs, and exposure to a likely causative medication. Treatment is supportive, with aggressive fluid support and wound care similar to that for burns. Secondary infections are common, and fatalities may occur despite treatment. (Choice A) Erythema multiforme (EM) is a self-limited illness characterized by an acute erythematous rash and usually occurs after herpes simplex infection. EM may cause mucosal lesions similar to those of SJS, but the predominant skin lesions are typically targetoid plaques favoring the distal extremities rather than desquamating bullae; systemic symptoms are not as prominent as in SJS. targetoid plaques (Choice B) Impetigo is a staphylococcal or streptococcal skin infection characterized by red macules and papular lesions with honey-colored crusts. It is most common in children. Impetigo (Choice C) Pemphigus vulgaris is caused by autoantibodies to desmosomes. Like SJS, it can cause mucosal lesions and desquamating bullae. However, systemic signs (eg, fever) are less prominent, and the course is typically more chronic, with oral lesions appearing weeks to months prior to skin symptoms. (Choice D) Staphylococcal scalded skin syndrome is usually seen in children age <6. It is a syndrome of acute exfoliation caused by toxins produced by Staphylococcus aureus. (Choice F) Toxic shock syndrome is an inflammatory response caused by a staphylococcal exotoxin and presents with fever, rash, hypotension, constitutional symptoms, and multiorgan injury. The rash is characterized by diffuse erythema resembling sunburn, with desquamation involving the palms and soles. Educational objective: Stevens-Johnson syndrome is an inflammatory reaction to drugs or certain infections and is characterized by coalescing erythematous macules, bullae, desquamation, and mucositis. Systemic signs include fever, hemodynamic instability, and altered level of consciousness. References: The current understanding of Stevens-Johnson syndrome and toxic epidermal necrolysis. (http://www.ncbi.nlm.nih.gov/pubmed/22014021)

A 45-Year-Old Man Comes to the Office After Sudden Onset