ulnar nerve, 2 cases) or median nerve, 1 case), hip/lower limb (i.e. sciatic nerve, 1 case), and lumbar spine/pelvis/thigh (i.e. the obturator nerve, 1 case). Individual cases of NF have been reported to occur in the lower female genital tract, bladder, prostate, tongue, and parotid gland. In some cases, NF tumors have  regressed after incisional biopsy, i.e. a biopsy that takes a small part of the diseased tissue.The cranial fasciitis variant of NF occurs in the soft and hard cranial tissues of the outer layers of the skull. Patients with this variant are more commonly males than females and almost exclusively between 3 weeks and 6 years of age. They typically present with a tumor in areas of the head that lay directly over the temporal or parietal bones. Individual cases have been reported to occur in the lower jaw, frontonasal region, anterior nasal spine (i.e. nasal process of the maxilla), and the orbit (i.e. eye socket)-maxilla area. Characteristically, the tumor is rapidly enlarging, non-painful, rarely regressing without treatment, and potentially expanding into the skulls interior.In a review of 50 cases, the intravascular fasciitis variant of NF occurred in individuals aged 6 months to 66 years (median age 27 years) with male and females being equally affected (52%:48%). Individuals with this variant commonly presented with a blood vessel-localized tumor of the head and neck area (34% of cases), lower extremity (32% of cases), upper extremity (20% of cases), or trunk (14% of cases).
Painful genital ulcerations usually develop around the anus, vulva, or scrotum and cause scarring in 75 percent of the patients. Additionally, patients may present with erythema nodosum, cutaneous pustular vasculitis, and lesions similar to pyoderma gangrenosum. Eyes
Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20 percent of cases. Ocular involvement can be in the form of posterior uveitis, anterior uveitis, or retinal vasculitis. Anterior uveitis presents with painful eyes, conjuctival redness, hypopyon, and decreased visual acuity, while posterior uveitis presents with painless decreased visual acuity and visual field floaters. A rare form of ocular (eye) involvement in this syndrome is retinal vasculitis which presents with painless decrease of vision with the possibility of floaters or visual field defects.Optic nerve involvement in Behçets disease is rare, typically presenting as progressive optic atrophy and visual loss. However, cases of acute optic neuropathy (specifically anterior ischemic optic neuropathy) have also been reported to occur. Optic nerve atrophy has been identified as the most common cause of visual impairment. Behçets disease may result in primary or secondary optic nerve involvement. Papilledema as a result of dural sinus thrombosis and atrophy resulting from retinal disease, have been characterized as secondary causes of optic nerve atrophy in Behçets disease.Signs and symptoms of acute optic neuropathy include painless loss of vision which may affect either one or both eyes, reduced visual acuity, reduced color vision, relative afferent pupillary defect, central scotoma, swollen optic disc, macular edema, or retrobulbar pain.