Pigmented Epithelioid Melanocytoma

**Semantic type:** Neoplastic Process

**Definition:** A rare cutaneous melanocytic neoplasm that presents as a slow growing pigmented nodule or papule. It is characterized by the proliferation of heavily pigmented epithelioid and dendritic cells, predominantly in the dermis. PRKAR1A gene inactivation is present in the majority of cases. It can spread to regional lymph nodes, but distant metastases are rare.

**Synonyms:** - PEM - Skin Epithelioid Blue Nevus - Skin Pigmented Epithelioid Melanocytoma