Looking for the diagnosis beneath the edema

Mucosal pathology
Facial dermatosis

Dr. Emmanuelle Bourrat

Saint-Louis Hospital

The clinical case
Now it's your turn!
Co-prescription and advice

Clinical case presentation

An adolescent with sickle cell anemia and no other known history was regularly hospitalized for hyperalgesic vaso-occlusive crises. He reported the almost systematic occurrence of a painless but highly deforming upper labial edema during his hospitalizations.

This edema lasted a few days and disappeared spontaneously. It was not influenced by any particular food intake. There were no other localizations (eyelids, hands or feet, oral or ENT mucosa), no superficial urticaria and no concomitant abdominal pain.

The young patient, recently fitted with well-supported orthodontic braces, denied any self-inflicted trauma. However, there was a large fibrinous, polycyclic ulceration of the inner mucosa of the upper lip, with little pain.

A review of the patient’s history showed that each vaso-occlusive crisis prompted a prescription for painkillers of levels 1 to 3, including a nonsteroidal anti-inflammatory drug.

Your turn

What is your diagnosis?

Select 1 answer(s) from the following choices:

Herpes recurrence

Bradykinin-mediated angioedema

Histamine-mediated angioedema due to food allergy

Non-allergic angioedema due to NSAIDs

Wrong answer!

Good answer!

Selected diagnosis

Systematic viral sampling of the ulceration found on the mucosal surface of the edematous lip revealed the presence of herpes simplex virus type 1, supporting the diagnosis of purely mucosal labial herpes recurrence responsible for perilesional edema at each vaso-occlusive crisis.

Herpes recurrences in this young man could be explained by a concomitant viral or bacterial infection (itself responsible for the vaso-occlusive crisis) or by the stress of pain and hospitalization.

Explanation of wrong answers

Bradykinin-mediated angioedema: this recurrent labial edema should raise the possibility of hereditary angioedema (but no family history, and the strictly labial distribution was not suggestive) or acquired angioedema (but no context of blood disorder or autoimmunity). An antigenic and functional C1 inhibitor assay was checked and found to be normal.
Histamine-mediated angioedema due to food allergy: the onset of this edema long after any food intake and its duration of more than 24 hours rule out a food allergy.
Non-allergic angioedema due to nonsteroidal anti-inflammatory drugs: this hypothesis was put forward because the anti-cyclooxygenase-1 activity of these molecules increases leukotriene synthesis and can trigger angioedema, often labial, in predisposed subjects.

Treatment

In theory, preventive or curative treatment with valacyclovir could be proposed to eliminate or limit the aesthetic and functional impact of herpes recurrence.

Message from the expert

Any lesion of the oral or genital mucosa with no obvious clinical diagnosis should be sampled for HSV, as the expression of herpes recurrence is sometimes atypical (ulceration, edema, vegetative lesions).

References:

^{[Recurrent labial herpes. How to treat and prevent it best].}
^{Nikkels AF, Piérard GE.Rev Med Liege. 2006 May-Jun;61(5-6):442-7}