[Skip to Content]
[Skip to Content Landing]

Desquamatory Lesions of the Upper Aerodigestive Tract Mucosa

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A man 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.

Please finish quiz first before checking answer.

You answered correctly!

Read the answer below and download your certificate.

You answered incorrectly.

Read the discussion below and retake the quiz.

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.1 Multiforme 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.

Survey Complete!

Sign in to take quiz and track your certificates

Buy This Activity

JN Learning™ is the home for CME and MOC from the JAMA Network. Search by specialty or US state and earn AMA PRA Category 1 CME Credit™ from articles, audio, Clinical Challenges and more. Learn more about CME/MOC

Article Information

Corresponding Author: Jérôme René Lechien, MD, PhD, MSc, Department of Otorhinolaryngology–Head and Neck Surgery, CHU de Lille, Lille, France 59000 (jerome.lechien@umons.ac.be).

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
1.
Assier  H, Bastuji-Garin  S, Revuz  J, Roujeau  JC.  Erythema multiforme with mucous membrane involvement and Stevens-Johnson syndrome are clinically different disorders with distinct causes.  Arch Dermatol. 1995;131(5):539-543. doi:10.1001/archderm.1995.01690170041005PubMedGoogle ScholarCrossref
2.
Amode  R, Ingen-Housz-Oro  S, Ortonne  N,  et al.  Clinical and histologic features of Mycoplasma pneumoniae-related erythema multiforme: a single-center series of 33 cases compared with 100 cases induced by other causes.  J Am Acad Dermatol. 2018;79(1):110-117. doi:10.1016/j.jaad.2018.03.013PubMedGoogle ScholarCrossref
3.
Wetter  DA, Camilleri  MJ.  Clinical, etiologic, and histopathologic features of Stevens-Johnson syndrome during an 8-year period at Mayo Clinic.  Mayo Clin Proc. 2010;85(2):131-138. doi:10.4065/mcp.2009.0379PubMedGoogle ScholarCrossref
4.
de Risi-Pugliese  T, Sbidian  E, Ingen-Housz-Oro  S, Le Cleach  L.  Interventions for erythema multiforme: a systematic review.  J Eur Acad Dermatol Venereol. 2019;33(5):842-849. doi:10.1111/jdv.15447PubMedGoogle ScholarCrossref
5.
Gupta  SS, Sabharwal  N, Patti  R, Kupfer  Y.  Allopurinol-induced Stevens-Johnson syndrome.  Am J Med Sci. 2019;357(4):348-351. doi:10.1016/j.amjms.2018.11.018PubMedGoogle ScholarCrossref
If you are not a JN Learning subscriber, you can either:
Subscribe to JN Learning for one year
Buy this activity
jn-learning_Modal_LoginSubscribe_Purchase
If you are not a JN Learning subscriber, you can either:
Subscribe to JN Learning for one year
Buy this activity
jn-learning_Modal_LoginSubscribe_Purchase
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right

Name Your Search

Save Search
With a personal account, you can:
  • Track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
jn-learning_Modal_SaveSearch_NoAccess_Purchase

Lookup An Activity

or

My Saved Searches

You currently have no searches saved.

With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Topics
State Requirements