Phakomatous Choristoma Key Points Phakomatous choristoma is a very rare congenital lesion It is believed to arise early in the embryogenesis of the eye during the optic vesicle stage as…

Pemphigus Vulgaris Key Points Pemphigus vulgaris (PV) is an autoimmune disease in which autoantibodies are directed against desmosomal adhesion molecules resulting in blistering in the suprabasal layers of the cutaneous…

Oculodermal Melanocytosis Key Points Oculodermal melanocytosis is a benign congenital anomalous pigmentation of deep facial skin and ocular tissues It is a dermal melanocytic hamartoma that presents with unilateral or…

Ocular Cicatricial Pemphigoid Key Points Mucous membrane pemphigoid describes a heterogeneous group of autoimmune disorders characterized by chronic inflammatory, mucous membrane blistering involving the ocular, oral, genital, nasopharyngeal, esophageal, or…

Nevus Sebaceous of Jadassohn Key Points Nevus sebaceous of Jadassohn (NSJ) is a congenital hamartoma of the pilosebaceous follicular unit The pathogenesis of NSJ is unknown, but a developmental arrest…

Nodular Fasciitis Key Points Nodular fasciitis is a rare benign reactive lesion characterized by a proliferation of fibroblasts and myofibroblasts that involve subcutaneous tissue, skeletal muscles, and fascia It is…

Nevus Flammeus Key Points Nevus flammeus or port-wine stain (PWS) is a congenital, low-flow vascular malformation of the skin that represents a progressive ectasia of the superficial cutaneous vascular plexus…

Neurothekeoma Key Points Neurothekeoma includes several lesions previously reported as nerve sheath myxoma or cellular neurothekeoma While some studies have questioned the relationship between these two entities, other authors have…

Neurofibroma, Plexiform Key Points Neurofibromatosis type 1 is an inherited multisystem neurocutaneous disorder The disease results from mutations in the NF1 gene that results in the loss of neurofibromin function…

Neurofibroma, Solitary Key Points Neurofibromas are tumors that arise from nonmyelinated Schwann cells and perineural fibroblasts Isolated neurofibromas are benign, slowly growing, unencapsulated neoplasms that usually arise from cutaneous nerves,…