(BQ) Part 2 book "Dermatology for the USMLE" has contents: Melanocytic skin disorders, premalignant and malignant skin disorders, selected bacterial infections, selected fungal infections, sexually transmitted infections (stis),. and other contents. | C h a p t e r 11 Inherited Skin Disorders Table . Neurofibromatoses Diagnostic Criteria Neurofibromatosis Type 1 (NF-1) * 1 ) ≥ 6 café-au-lait spots (hyperpigmented macules) || ≥ 5 mm in diameter in pre-pubertal children || ≥ 15 mm in diameter in post-pubertal children Neurofibromatosis Type 2 (NF-2) ** 1 ) Bilateral vestibular schwannomas 2 ) > 2 axillary or inguinal freckles 2 ) A first-degree relative with NF-2 3 ) ≥ 2 typical neurofibromas or one plexiform neurofibroma 4 ) Optic nerve glioma 5 ) ≥ 2 iris hamartomas (Lisch nodules) AND Unilateral vestibular schwannoma OR Any two of: Meningioma, schwannoma, glioma, neurofibroma, posterior subcapsular lenticular opacities 3 ) Unilateral vestibular schwannoma 6 ) Sphenoid dysplasia or typical long-bone abnormalities, such as pseudarthrosis AND Any two of: Meningioma, schwannoma, glioma, neurofibroma, posterior subcapsular lenticular opacities 4 ) Multiple meningiomas AND 7 ) First-degree relative with NF-1 Unilateral vestibular schwannoma OR Any two of: Schwannoma, glioma, neurofibroma, cataract * Clinical diagnosis of NF-1 requires that an individual present with at least 2 of 7 of the above-mentioned criteria. ** Clinical diagnosis of NF2 requires that an individual present with at least 1 of the 4 clinical scenarios mentioned above. 1. Neurofibromatoses zzGeneral: Autosomal dominant group of genetic disorders that affect bones, soft tissue, skin and nervous system. Classified into neurofibromatosis type 1 (NF-1) and neurofibromatosis type 2 (NF-2). || Neurofibromatosis type 1 (NF-1): Also known as peripheral NF or von Recklinghausen disease, fairly common neurocutaneous disorder occurring in 1:3000 births. It is caused by NF-1 gene mutation on chromosome 17 leading to decreased production of the tumor suppressor protein neurofibromin. NF-1 is associated with: pheochromocytomas, Chiari type-1 malformation and gastrointestinal stromal tumors (GIST). || Neurofibromatosis type 2 (NF-2): Also known
