Case
Aman in his 30s was referred to the department of Otorhinolaryngology–Head & Neck Surgery for a medical history of severe dysphagia and dysphonia. The patient reported fever and rhinorrhea in the few days preceding the emergency consultation. He did not take medication over the past 3 months. There was no relevant family medical history. At the clinical examination, the patient had several macules and desquamatory lesions on the mucosa of the oral cavity, lips, and penis, and he had hemorrhage lesions of the eye sclera (Figure). The skin of the patient was unremarkable. The nasofibroscopy results showed generalized desquamatory lesions of the upper aerodigestive tract mucosa affecting the laryngeal and vocal fold mucosa (Figure). There was no dyspnea and the airway was clear. The patient revealed that he had a similar but less severe clinical event 5 years previously. At that time, no diagnosis was found and the lesions disappeared following treatment with corticosteroids. Dermatopathologic analysis, biology, serology, and biopsy were performed.
Figure.
Lesions of eye, oral, and upper aerodigestive tract mucosa. Desquamatory injuries were found on vocal folds and ventricular bands (A); iris (B); lips and oral mucosa (C); and epiglottis (D). There was a moderate laryngeal mucosa hypertrophy related to the inflammatory reaction.
What Is Your Diagnosis?
- Bullous pemphigus
- Toxic epidermal necrolysis (Lyell syndrome)
- Multiforme erythema
- Stevens-Johnson syndrome
Discussion
Diagnosis
C. Multiforme erythema
The polymerase chain reaction (PCR) analysis revealed a positive infection result for Mycoplasma pneumoniae. The diagnosis of a multiforme erythema (ME) limited to genital, head and neck, and eye mucosa was based on the PCR analysis, the clinical picture, and the dermatopathologic analysis, which mainly found epidermal necrosis, spongiosis, and parakeratosis. The patient received intravenous antibiotics and corticosteroids. The ME progressively disappeared throughout the 2 weeks of therapy. The follow-up of the patient was unremarkable (6 months).
Multiforme erythema is a very rare disease with an unknown incidence. It is often confused with Stevens-Johnson syndrome (SJS) although these 2 conditions were well separated in the 1990s.1Multiforme erythema is characterized by acral cutaneous typical (3 rings) or atypical (2 rings) elevated targets, whereas SJS is characterized by generalized or localized (trunk) purpuric erythematous macules, flat atypical targets, with epidermal detachment.1 There may be an involvement of upper aerodigestive tract mucosa in both diseases, occurring in conjunction with ocular (iris) hemorrhages and genitourinary lesions. The cutaneous and mucosal lesions appear in groups and usually evolve from macules to bullae, which then form pseudomembranes. On dermatopathologic analysis, both diseases are characterized by dense dermal infiltrate, necrotic keratinocytes, red blood cell extravasation, pigment incontinence, and parakeratosis.2,3 The differential diagnosis between SJS and ME is made through the skin lesion pattern and the causal factor. In most cases, SJS is drug-induced whereas ME is mainly owing to infection (30%-90%; herpes simplex virus or M. pneumoniae).4 From a dermatologic point of view, major ME is limited to 1% to 2% of the body surface area (BSA) and SJS involves less than 10% of BSA.5 Because there was no skin involvement in the present case, the differential diagnosis between SJS and EM was particularly difficult and based on the causal factor identification.
Among the other main differential diagnoses, we may point to bullous pemphigus and toxic epidermal necrolysis (TEN). Toxic epidermal necrolysis is the most important diagnosis to exclude because this disease is still lethal in many cases. The skin lesions of TEN are usually predominant (involving >30% of BSA) and rapidly evolve. Although there may be associated upper aerodigestive tract, eye, and genital injuries, the diagnosis is clinical and the cause is the intake of some drugs (eg, sulfonamides, anti-inflammatories).
Pemphigus is an autoimmune blistering disease of the skin and the mucosa characterized by circulating autoantibodies directed to the keratinocyte cell surface (pemphigus vulgaris and pemphigus foliaceus) or basement membrane zone (bullous pemphigus). Similar to ME, SJS, and TEN, pemphigus may be associated with upper aerodigestive tract, eye, and genital lesions. The biopsy confirms the diagnosis and the autoantibodies are identified in both tissues (biopsy) and blood through enzyme-linked immunosorbent assay.
Although an incomplete form of ME is still exceptional, physicians should consider the diagnosis in patients with desquamatory upper aerodigestive tract lesions associated with both iris and genital injuries. When cutaneous involvement is lacking, the identification of the causal factor is important for the diagnosis.
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Article Information
Published Online: August 1, 2019. doi:10.1001/jamaoto.2019.1990
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
References
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