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Abstract:

The diagnosis of ectodermosis erosiva pluriorificialis was made in a 16-year-old boy who had suffered from recurrent oral aphthosis and progressive illness with high fever, anorexia and malaise for more than nine years. This form of erythema multiforme confined to mucosal surfaces, is extremely rare. Due to the atypical clinical course of the disease, it lasted several years before the definitive diagnosis was made. The differential diagnosis of recurrent aphthous stomatitis, viral aphthosis, systemic disease, mucusal pemphigoid, pemphigus vulgaris and erythema multiforme, is discussed.