Plasma Cell Granuloma of the Jaw and the Infratemporal Fossa: A Clinical Case.

Plasma cell granuloma or inflammatory pseudotumor (IPT) is diagnosed by a process of elimination. The precise etiology is unknown, although it can occur after a bout of periodontal infection. This report describes the various stages of progression for this ailment. A 49-year-old woman with no noteworthy medical history presented with a recurrent periodontal abscess accompanied by progressive and severe destruction of the right maxilla. There was invasion of the infratemporal fossa and very tight trismus. Histologic examination indicated a reactive plasma cell granuloma. IPT is an entity recognized by the World Health Organization. A triggering infectious or inflammatory factor is often present. In the maxilla, progression is very aggressive. Treatment relies on corticotherapy, with or without radiotherapy, and administration of cyclosporine.

[Oral diseases in auto-immune polyendocrine syndrome type 1]. / Polyendocrinopathies auto-immunes de type 1 et pathologies buccales.

Auto-immune polyendocrine syndrome type 1 (APS1) also called Auto-immune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED) is a rare monogenic childhood-onset auto-immune disease. This autosomal recessive disorder is caused by mutations in the auto-immune regulator (AIRE) gene, and leads to autoimmunity targeting peripheral tissues. There is a wide variability in clinical phenotypes in patients with APSI, with auto-immune endocrine and non-endocrine disorders, and chronic mucocutaneous candidiasis. These patients suffer from oral diseases such as dental enamel hypoplasia and candidiasis. Both are frequently described, and in recent series, enamel hypoplasia and candidiasis are even the most frequent components of APS1 together with hypoparathyroidism. Both often occur during childhood (before 5 years old for canrdidiasis, and before 15 years old for enamel hypoplasia). Oral candidiasis is recurrent all life long, could become resistant to azole antifungal after years of treatment, and be carcinogenic, leading to severe oral squamous cell carcinoma. Oral components of APS1 should be diagnosed and rigorously treated. Dental enamel hypoplasia and/or recurrent oral candidiasis in association with auto-immune diseases in a young child should prompt APS1 diagnosis.