Coccygeal polypoid eccrine nevus associated with imperforate anus and unilateral multicystic kidney dysplasia

Eric Frouin (1) , Benjamin Rivière (2) , Olivier Maillet (3) , Marjolaine Willems (4) , Nicolas Kalfa (3, 5) , Valérie Costes (2, 6) , Didier Bessis (2, 5)

1 CHU de Poitiers [La Milétrie] - Centre hospitalier universitaire de Poitiers = Poitiers University Hospital
2 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
3 Département Chirurgie Pédiatrique [CHRU Montpellier]
4 Département de génétique médicale, maladies rares et médecine personnalisée [CHRU Montpellier]
5 UM - Université de Montpellier
6 Pathogénèse et contrôle des infections chroniques (PCCI)

Eccrine nevi are rare hamartomas characterized by an increase in the number or size of eccrine glands. A polypoid form located in the coccygeal area has been described in a few cases and termed coccygeal polypoid eccrine nevus (CPEN). No association with internal malformations was reported in any of these cases. We describe herein a case of CPEN associated with imperforate anus and unilateral multicystic kidney dysplasia. We review the clinical and pathological characteristics of CPENs and discuss the differential diagnoses.

Mots clés: coccygeal eccrine polypoid nevus; imperforate anus; multicystic kidney dysplasia

Domaines: Dermatologie; Médecine humaine et pathologie

Eric Frouin, Benjamin Rivière, Olivier Maillet, Marjolaine Willems, Nicolas Kalfa, et al.. Coccygeal polypoid eccrine nevus associated with imperforate anus and unilateral multicystic kidney dysplasia. Journal of Cutaneous Pathology, 2016, 43 (8), pp.697 - 701. ⟨10.1111/cup.12718⟩. ⟨hal-01871908⟩