Milk coffee spots Milk coffee spots (Café au lait spots) are hyperpigmented lesions that range in color from light brown to dark brown and may have smooth or irregular borders. Café au lait spots are highly variable in size and number and are usually an early manifestation of neurofibromatosis. Skin defects develop more rapidly in young children and may become larger and more pronounced by age 2 years. Ninety-five percent of patients with type I neurofibromatosis present with café au lait spots. Others, such as McCune-Albright syndrome, tuberous sclerosis, and Fanconi anemia, may also be seen. Milk-like café au lait spots are mainly increased melanin deposits, and melanocytes are significantly increased in patients with type I neurofibromatosis. 2. Diagnostic criteria of type I neurofibromatosis: 1) Milk coffee spots with maximum diameter greater than 5mm and number of 6 or more before puberty, and milk coffee spots with maximum diameter greater than 15mm after puberty. 2) Two or more neurofibromas of any type or 1 plexiform neurofibroma 3) Freckles in axillary or inguinal area 4) Optic glioma 5) Two or more Lish nodules (iris 6) Obvious bony defects, such as pterygoid dysplasia or thinning of the periosteum of the long bones, with or without pseudarthrosis Differentiation between type I neurofibromatosis and type II neurofibromatosis: Type II neurofibromatosis, also known as central neurofibromatosis, is combined with auditory neuroma and is commonly seen in middle-aged patients, whereas type I neurofibromatosis is mostly seen in pediatric patients. Treatment: Milk café au lait does not require treatment. Further testing is needed when the coffee spot is combined with neurofibromatosis or other disorders, such as scoliosis.