Endgames Case Review

The diagnosis is in the rings

BMJ 2017; 359 doi: https://doi.org/10.1136/bmj.j3817 (Published 05 October 2017) Cite this as: BMJ 2017;359:j3817
  1. Saskia Ingen-Housz-Oro, dermatologist1 2 5,
  2. Nicolas Ortonne, professor of pathology3 4,
  3. Olivier Chosidow, professor of dermatology1 2 3 5
  1. 1Department of dermatology, AP-HP, Henri Mondor Hospital, Créteil, France
  2. 2EA 7379-EpiDermE, Créteil, France
  3. 3University Paris-Est Val de Marne Créteil, UPEC, France
  4. 4Department of pathology, AP-HP, Henri Mondor Hospital, Créteil, France
  5. 5Referral centre for severe cutaneous adverse reactions, Créteil, France
  1. Correspondence to Saskia Ingen-Housz-Oro saskia.oro{at}aphp.fr

A 62 year old man reported a history of recurrent oral ulcers, sometimes with laryngitis, and conjunctivitis. He consulted his doctor in 2011 for acute onset fever (39°C), odynophagia, and laryngitis lasting two days, and was prescribed ibuprofen and clarithromycin. Two days later, conjunctivitis, oral mucous membrane erosions, and cutaneous lesions appeared. The patient was hospitalised on suspicion of Stevens-Johnson syndrome.

Dermatological examination showed several target lesions on the trunk, lower limbs, and scrotum (fig 1, 2), conjunctivitis (fig 3), and diffuse erosions of the mucous membranes involving the mouth (palate, tongue, buccal mucosa, and lips) (fig 4), and glans. Examination of ear, nose, and throat showed diffuse nasal erosion, crusts, and epiglottal erosion. The patient had no dysuria, pain with defecation, or pulmonary symptoms. His general condition was poor, with fever, fatigue, and difficulty eating.

Fig 1 Disseminated target lesions

Fig 2 Eye involvement with conjunctivitis

Fig 3 Lip and tongue erosions

Fig 4 Lesions with three rings

Blood examination showed normal blood cell counts, increased C reactive protein (160 mg/L), negative herpes simplex virus culture from the mouth and skin, and negative HIV, herpes simplex virus, Mycoplasma pneumoniae, and Chlamydia pneumoniae serologies. Skin biopsy showed a dense lichenoid lymphocytic infiltrate with necrotic keratinocytes in the basal layer. Direct and indirect immunofluorescence tests were both negative.

Questions

  • 1. What is the diagnosis and what clinical findings suggest this?

  • 2. What differential diagnosis needs to be excluded before deciding whether the patient can use ibuprofen and clarithromycin in the future?

  • 3. When should patients with this condition be referred to the dermatologist and what is the treatment?

Answers

1. What is the diagnosis and what clinical findings suggest this?

Short answer

A diagnosis of erythema multiforme major …

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