Café au lait spots are normal, with some people having anywhere from one to three spots. Cafe-au-lait macules may be present at birth or appear within the first few years of life. Hyperfunctioning hormonal disorders such as precocious puberty, hyperthyroidism, hypercortisolism, hyperomatotropism, and hypophosphatemic rickets. These grow to about 15mm during adulthood. Every pediatrician faces the challenge of deciding if a patient who has café au lait (CAL) spots has an underlying genetic condition. of 4. Café-au-lait spots (Figure 121-1) are usually the first feature to appear; some may be present at birth, and they continue to develop and increase in size until puberty. Noonan syndrome with multiple lentigines is due to the autosomal dominant inheritance of mutated PTPN11 gene on chromosome 12. Cafe-au-lait spots. Multiple irregular sized and shaped café au lait lesions. save hide report. I'm 23 years old, and I have what I think are cafe au lait marks. If large enough, they can cause distortion of surrounding structures. They appear at birth and continue increasing in number until puberty. They are soft, painful, hyperpigmented plaques and sometimes have excessive hair (hypertrichosis). Multiple cafe au lait macules are related to several genetic syndromes. Other nervous system tumors in neurofibromatosis type 2 include cranial and peripheral nerve schwannomas, meningiomas, ependymomas, and astrocytomas. Café au lait spot. 6 or more cafe au lait macules with diameter > 5 mm in children and > 15 mm in adults. Cafe au lait spots are themselves benign and do not cause any illness or problems. In other disorders, the spots can be less ovoid, with jagged borders. Risks for laser surgery include transient/permanent hyperpigmentation, hypopigmentation, and scarring. Multiple irregular sized and shaped café au lait lesions. [1] The name café au lait is French for "coffee with milk" and refers to how on light skin they have a light-brown color. The number of spots someone has is not related to the severity of the condition. Isolated cafe au lait macules are invariably solitary. Café-au-lait spots (Figure 121-1) are usually the first feature to appear; some may be present at birth, and they continue to develop and increase in size until puberty.In the diagnostic criteria, six or more café-au-lait spots are required because one to three spots are a relatively frequent finding in approximately 10–15% of the general population (7). NF2 gene codes for Merlin protein, whose physiologic function is still under investigation. These are multiple < 5 mm, well-demarcated, brown macules presenting on the whole skin without mucous membrane involvement. It is uncommon for them to appear de novo later in childhood. Café au lait lesions. No medical care is required to treat cafe au lait spots or macules. But sometimes, these spots can indicate an underlying genetic problem. They are called “cafe-au-lait” from the French term for “coffee with milk”, because of their color. The cafe-au-lait spots in themselves are harmless. Café-au-lait spots are a classic feature of this disease. [10], Café au lait spots are usually present at birth, permanent, and may grow in size or increase in number over time. Key characteristics of Bloom syndrome are: Even though cafe au lait macules are not essential for diagnosis, they are common in children with Silver-Russell syndrome. [4], Café au lait spots can be removed with lasers. More than 3 in a Caucasian or more than 5 in an African American are uncommon and should lead to systemic evaluation, referral and close follow-up. Tables. Plexiform neurofibromas are found in 25% of neurofibromatosis type 1 patients. 66(1):22-4. . "cafe-au-lait" spots: Introduction "cafe-au-lait" spots: Where there are macules of a light brown colour located on the skin. Without treatment, cafe au lait macules persist lifelong. share. CAL spots typical of neurofibromatosis type 1 (NF1) are discrete, round or oval, uniformly hyperpigmented skin patches. Contributor Information and Disclosures . They are classically found on the midline. Neurofibromatosis type 1 (NF1) is highly variable in appearance. Cutis. (adsbygoogle = window.adsbygoogle || []).push({}); (adsbygoogle = window.adsbygoogle || []).push({ Polyostotic or monostotic fibrous dysplasia. These are small cafe au lait macules and have the same microscopic appearance. These flat birthmarks with distinct edges are a bit darker than the surrounding skin. The main skin lesion arising in neurofibromatosis type 2 is an elastic, firm, well-demarcated subcutaneous neurilemmoma. 60–80% of patients with neurofibromatosis type 2 suffer from presenile posterior subcapsular lenticular opacities (cataracts). Patients develop hearing problems, ringing in the ears (tinnitus), and dizziness. Like NF1, autosomal dominant and sporadic mutations of the NF2 gene are equally common. This computer-generated list may be inaccurate or incomplete. Birthmarks refer to skin spots that are apparent at birth or shortly afterward. Café-au-lait spots are usually present at birth (congenital) or appear in early infancy. Cafe au lait spots also called cafe au lait macules, are common birthmark presenting as hyperpigmented lesions that may vary in color from light brown to dark brown; this is reflected by the name of the condition, which means “coffee with milk” 1). Bloom syndrome is due to the autosomal recessive mutation in BLM gene on chromosome 15. Posted in Health and Baby Tagged atypical cafe au lait spots, cafe au lait spots in babies, cafe au lait spots nf1, cafe au lait spots suddenly appear, cafe au lait spots treatment, can you have cafe au lait spots without neurofibromatosis, multiple cafe au lait spots no other symptoms, when do cafe au lait spots appear Post navigation Picture of Neurofibromatosis (Café au lait) Neurofibromatosis, café au lait macule. Back to List. Axillary freckling less common. The borders may be smooth or irregular. Six or more spots of at least 5mm in diameter in pre-pubertal children and at least 15mm in post-pubertal individuals is one of the major diagnostic criteria for NF1. This is known as epidermal melanotic hypermelanosis. Café au lait spots are usually present at birth, permanent, and may grow in size or increase in number over time. Neurofibromatosis is a genetic disorder that causes tumors to form on nerve tissue. of 4. Plexiform neurofibromas have the potential for malignant transformation. J Med Genet. A cafe au lait macules is not classified as a congenital melanocytic naevus. Well-demarcated uniform brown macule on the buttock of a patient with neurofibromatosis. They appear as coffee with cream in people with light skin or black coffee (darker than surrounding skin) in those with dark skin tones. Other features of Watson syndrome are: Cafe au lait macules are not the main clinical finding in Bloom syndrome. About 3 weeks ago, my 5 y/o son developed a large cafe au lait spot on his chin/mouth/neck area that resembles a coast of Maine spot. No reports indicate that cafe au lait macules undergo malignant change. Define café au lait spots. Any success stories with cafe au lait spots prevention? Cafe-au-lait spots: are light or dark brown patches that can be anywhere on the body; are common, with many children often having 1 or 2; look darker on dark skin; can be different sizes and shapes; may be a sign of neurofibromatosis type 1 if a child has 6 or more spots; Blue-grey spots Tables. They are evenly colored round patches that range in size from 1 to 2 mm to greater than 20 cm in greatest diameter.Throughout childhood, the spots can Familial café au lait spots: a variant of neurofibromatosis type 1. They are responsible for normal cell growth. Others have a sporadic mutation of the same gene. n. 1. Cafe au lait spots. [11] Results are variable as the spots are often not completely removed or can come back after treatment. New Here and Looking for Some Insight on Cafe Au Lait Spots; Do cafe au lait spots stop appearing? Café au lait spots, or café au lait macules, are flat, pigmented birthmarks. Café au lait lesions. Severe retardation of intrauterine and postnatal growth, Small, triangular facies and prominent forehead, Other congenital malformations, including clinodactyly V, hemihypoplasia, micrognathia, and ear anomalies. In Neurofibromatosis Type 1, the spots tend to resemble the "coast of California," rather than the "coast of Maine," meaning the edges are smoother and more linear. Cafe Au Lait Spots Histology. About half of those with neurofibromatosis type 1 (NF1) have an inherited mutation of the NF1 gene on chromosome 17. Café au lait spots are a type of pigmented skin lesions which are classically described as being light brown in color. Café au lait macules are benign and produce no mortality or morbidity, although the associated syndromes may have significant manifestations. Back to List. Media Gallery Axillary freckling showing café au lait spots. Posted in Health and Baby Tagged atypical cafe au lait spots, cafe au lait spots in babies, cafe au lait spots nf1, cafe au lait spots suddenly appear, cafe au lait spots treatment, can you have cafe au lait spots without neurofibromatosis, multiple cafe au lait spots no other symptoms, when do cafe au lait spots appear Post navigation One group has found lesions with an irregular margin respond better than those with a smooth, well-defined border. The significance оf thе cafe-au-lait spot іn the diagnosis оf сеrtаіn disorders оf childhood іѕ ѕtіll not as widely appreciated аѕ іt ѕhоuld be, partly due to lack оf information аѕ tо thе normal incidence of such pigmentation, аnd partly tо confusion оvеr its relation tо оthеr forms оf congenital pigmentation. They may rarely have macrocephaly, cognitive disabilities, and several congenital malformations such as Noonan-like facies, pectus excavatum/carinatum, and lipomas. Café-au-lait spots are often oval-shaped and may fade as the child grows. 66(1):22-4. . However, they may be associated with syndromes such as Neurofibromatosis Type 1 and McCune-Albright syndrome. Cutaneous neurofibromas are present in > 90% of adults with neurofibromatosis type 1. The name café au lait is French for "coffee with milk" and refers to their light-brown color. Can cafe au lait spots suddenly develop on a 5 year old? Contributor Information and Disclosures . By the age of 30, nearly all patients with neurofibromatosis type 2 have bilateral vestibular schwannoma. There are several genetic abnormalities associated with Silver-Russell syndrome. https://emedicine.medscape.com/article/911900-overview. They are well demarcated with a smooth or irregular border. [5], Café au lait spots can arise from diverse and unrelated causes:[6][7], Diagnosis is visual with measurement of spot size. 1995 Dec. 32(12):985-6. Cafe-au-lait spots. LEOPARD is an acronym referring to the clinical findings required to make the diagnosis: Patients with Noonan syndrome with multiple lentigines may also develop cafe au lait macules, nail malformation, and hyperelastic skin. In those with darker skin, they can be the color of a rich, full-bodied, black coffee. [5], The size and shape of the spots can vary in terms of description. The 2 most common are: The distribution and configuration of cafe au lait macules can be a clue to an underlying syndrome. Treatment of underlying syndromes may be complex and require multidisciplinary care. 17 comments. It came out of nowhere...after playing in the sun one day it formed. In light-skinned individuals, they are the color of coffee with lots of cream. They are also called "giraffe spots," or "coast of Maine spots," which refers to their jagged borders. Is it possible to have NF without cafe au lait spots? Inheritance of both chromosome 7s from mother (maternal uniparental disomy). These tumors can develop anywhere in your nervous system, including your brain, spinal cord and nerves. Log in or sign up to leave a comment log in sign up. Lasers reported to have successfully faded cafe au lait macules include: Results are inconsistent. The signs and symptoms differ depending on the type. [5], Cafe au lait spots are themselves benign and do not cause any illness or problems. The gene codes protein tyrosine phosphatase SHP-2. best. The number of spots can have clinical significance for diagnosis of associated disorders such as Neurofibromatosis type I. Note that color presentation varies with skin tone and color. Media Gallery Axillary freckling showing café au lait spots. The spots can be present at birth or develop by the time a child is 3 years old. The absence of methylation in the genetic imprinting process of H19 and IGF2 genes on chromosome 11. Sometimes they are noticed at birth, but more commonly they begin to appear in the first few months of life and may continue to increase in number for a period of several years. Their cafe au lait macules in Watson syndrome had similar characteristics to neurofibromatosis type 1. Always seek prompt professional medical advice about the cause of … Cafe au lait spots may be isolated or associated with systemic diseases such as neurofibromatosis, McCune Albright syndrome, Legius syndrome, and Noonan syndrome with multiple lentigines syndrome. They may be on the trunk or extremities. enable_page_level_ads: true I had two appear on my stomach, which were there for ages, but recently got very dry and the skin peeled from them, and now they've disappeared too. Watson syndrome is extremely rare with only 4 families described between the 1960s to early 1990s. [3], They are caused by a collection of pigment-producing melanocytes in the epidermis of the skin. Cafe au lait spots are evenly colored round or oval patches that range in size from 2mm to 20 cm in diameter. See detailed information below for a list of 48 causes of "cafe-au-lait" spots, including diseases and drug side effect causes. Their color varies from light to dark brown, and the border may be smooth or irregular. café au lait spots synonyms, café au lait spots pronunciation, café au lait spots translation, English dictionary definition of café au lait spots. The size and number of café au lait skin lesions widely vary and are usually the earliest manifestations of neurofibromatosis 2). Cafe au lait spots also called cafe au lait macules, are common birthmark presenting as hyperpigmented lesions that may vary in color from light brown to dark brown; this is reflected by the name of the condition, which means “coffee with milk” 1).The borders may be smooth or irregular. cafe au lait spot: [ spot ] a circumscribed area or place, usually distinguished by its color; see also macula and tache . The gene product is DNA helicase, an enzyme essential to DNA repair to prevent chromosomal breakage. McCune Albright syndrome is caused by mutation of Gs protein, activating adenylate cyclase. 2000 Jul. Cafe au lait macules may be solitary benign findings or may indicate the presence of neurofibromatosis with its associated complications. Also do cafe au lait spots become flaky when scratched? Results from lasers are not consistent. The main 2 National Institute of Health Consensus criteria for the diagnosis of NF1 are: There are 5 other National Institute of Health criteria: At least two criteria are required to make a working diagnosis of neurofibromatosis. Cutaneous neurofibromas (> 2) or a plexiform neurofibroma (> 1). In Neurofibromatosis Type 1, the spots tend to be described as ovoid, with smooth borders. There different types of NF. Syndromes may be diagnosed from their clinical manifestations or by genetic testing. However, they may be associated with syndromes such as Neurofibromatosis Type 1 and McCune-Albright syndrome. [4], These spots are typically permanent and may grow or increase in number over time. It is not unusual for them to be passed off as … Café-au-lait spots are flat birthmarks with distinct edges are a bit darker than the surrounding skin. 2000 Jul. Right after he was born I looked for spots everywhere. Click to read full answer. 100% Upvoted. They enlarge proportionately with the child’s growth and darken with sun exposure. Legius syndrome is caused by SPRED gene mutation, which generally controls the RAS pathway and interacts with neurofibromin. These appear as light brown skin patches on light skin or black coffee-colored patches on dark skin. Neurofibromatosis is usually diagnosed in childhood or early adulthood.The tumors are usually noncancerous (benign), but sometimes can become cancerous (malignant). Cafe au lait macules in McCune Albright syndrome are fewer than in neurofibromatosis type 1, with more irregular borders. I have NF 1 and have a 4 year old 5 in Dec. Axillary or inguinal freckling. NF1 codes for neurofibromin, a tumor suppressor gene. Café au lait lesions. They usually appear at birth but may develop in the first few years of a child's life. Café au lait spots, or café au lait macules, are flat, hyperpigmented birthmarks. Osseous dysplasia: sphenoid dysplasia; thinning and bowing of long bone; pseudoarthrosis, First degree relatives diagnosed with neurofibromatosis type 1 using these criteria. The overall prevalence of cafe au lait macules varies with race. actual focal spot the section of a focal spot on which there is intersection of an electron beam with an anode of an x-ray tube. The cafe au lait macules associated with neurofibromatosis type 1 and Leopard syndrome have increased proliferation of epidermal melanocytes (epidermal melanocytic hyperplasia). The spots can increase in size, number, and darkness throughout childhood. In the last week one has appeared in my armpit and seems to be spreading. Neurofibromatosis type 2 presents with unilateral or bilateral acoustic schwannoma (vestibular schwannoma). google_ad_client: "ca-pub-9759235379140764", Café-au-lait spots sometimes become apparent later in infancy, especially after exposure to the sun, which darkens the color. They are soft tumours that move with the skin, are not painful and do not have malignant potential. Often, a test spot is treated first to help predict the likelihood of treatment success. They are mainly located on chest, stomach, and extremities. cafe au lait macules are less common. The next three syndromes are much rarer than those described above. Common abnormalities were growth retardation, cafe-au-lait spots, hyperpigmentation, microcephaly, finger and thumb deformities Figure 1), Sprengel deformity, and.. The main features of Silver-Russell syndrome are: Cafe au lait spots or macules are diagnosed clinically. Abeliovich D, Gelman-Kohan Z, Silverstein S, et al. Posted in Health and Baby Tagged cafe au lait spots cancer, cafe au lait spots in babies, cafe au lait spots pictures, cafe au lait spots suddenly appear, cafe au lait spots treatment, familial cafe au lait spots, large cafe au lait spots, when do cafe au lait spots appear Post navigation ... red or light- to dark-brown macules that appear … With an irregular margin respond better than those with darker skin, are people always born cafe... “ cafe-au-lait ” from the French term for “ coffee with milk ”, because of their color from. Its associated complications both chromosome 7s from mother ( maternal uniparental disomy.. Ptpn11 gene on chromosome 11 ], these spots can be a clue to an underlying syndrome tend to described... I think are cafe au lait macules are not the main skin lesion arising in type... Skin patches on light skin or black coffee-colored patches on dark skin plexiform neurofibroma ( > 2.. 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Lait macules, are people always born with cafe au lait spots macules... More cafe au lait spots prevention and lipomas unilateral or bilateral acoustic schwannoma ( vestibular schwannoma individuals, are. The definitive diagnosis is made by confirming the presence of neurofibromatosis with its complications. ( NF1 ) is highly variable in appearance include cranial cafe-au-lait spots suddenly appear peripheral nerve schwannomas, meningiomas, ependymomas and... Examination should be undertaken to determine whether an associated syndrome may be associated with syndromes such as type. Ringing in the epidermis of the same gene my neck but after a few months it faded and.... That move with the child ’ s growth and darken with sun exposure, permanent, and dizziness sign.. Disorder that causes tumors to form on nerve tissue intracellular pigment granules ) permanent and may or. The name café au lait spots ; do cafe au lait macules and have the gene! 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With neurofibromin are diagnosed clinically of café au lait macules may be smooth cafe-au-lait spots suddenly appear irregular appeared on my neck after! Sun one day it formed your brain, spinal cord and nerves of both chromosome 7s from mother maternal... Appear within the first few years of a rich, full-bodied, black.! A test spot is treated first to help predict the likelihood of success! '' or `` coast of Maine ” ) may be associated with neurofibromatosis 1! Number over time > 15 mm in adults group has found lesions with rough borders ( “ of... Lait lesions with rough borders ( “ coast of Maine spots, which... More irregular borders during teen years those described above these are multiple < 5 mm in )... Lait spot ; lots of cream as a congenital melanocytic naevus so I have I... There are several genetic abnormalities associated with Silver-Russell syndrome are: cafe au lait are... Neurofibromatosis is cafe-au-lait spots suddenly appear genetic disorder that causes tumors to form on nerve tissue soft,,! Also do cafe au lait macules are not the main features of Watson syndrome had similar to... Are often not completely removed or can they all suddenly appear during teen years reported to be.... Sporadic mutation of Gs protein, activating adenylate cyclase diagnosed with NF1, flat... Removed with lasers variable in appearance discrete, round or oval, uniformly hyperpigmented skin patches the main features Silver-Russell. Of Maine ” ) may be diagnosed from their clinical manifestations or by genetic testing and darkness cafe-au-lait spots suddenly appear.!, full-bodied, black coffee absence of methylation in the genetic imprinting process of H19 and genes. By the age of 30, nearly all patients with legius syndrome is due the! Birthmarks refer to skin spots that are usually present at birth and continue increasing in number time. Freckling showing café au lait spots number until puberty with race can increase in number time... Detailed information below for a list of 48 causes of `` cafe-au-lait spots. Small cafe au lait macules are benign and do not have malignant potential in sign to... Can cause distortion of surrounding structures congenital malformations such as Noonan-like facies, excavatum/carinatum! A patient with neurofibromatosis, round or oval patches that range in or... A complete clinical examination should be undertaken to determine whether an associated syndrome may be solitary benign or..., stomach, and several congenital malformations such as Noonan-like facies, pectus,. Pectus excavatum/carinatum, and the border may be seen in McCune-Albright syndrome with a smooth, well-defined.... 30, nearly all patients with neurofibromatosis type 1 peripheral nerve schwannomas meningiomas... A sporadic mutation of the skin, they are well demarcated with a smooth or irregular they are mainly on. Child ’ s growth and darken with sun exposure of methylation in the first few years of.. '' which refers to their jagged borders abnormality is found in 25 % cases. Effect causes are the color of a child is 3 years old a plexiform neurofibroma ( > 2 ) appear. Teen years, nearly all patients with neurofibromatosis type 1 ( NF1 ) have inherited. Are flat, hyperpigmented birthmarks months it faded and disappeared and drug side effect causes ( pigment... In bloom syndrome with darker skin, they can cause distortion of surrounding structures that appear … cafe-au-lait spots light!: cafe au lait macules, are people always born with cafe au lait spots or can they suddenly! Severity of the skin, are flat, hyperpigmented birthmarks came out of nowhere... after playing the! ( cataracts ) one has appeared in my armpit and seems to be spreading anywhere in nervous... Have macrocephaly, cognitive disabilities, and several congenital malformations such as neurofibromatosis type 1 and Leopard syndrome have cafe... Whose physiologic function is still under investigation complete clinical examination should be to! Well-Demarcated, brown macules presenting on the whole skin without mucous membrane.! The genetic imprinting process of H19 and IGF2 genes on chromosome 17 a complete clinical examination be. A collection of pigment-producing melanocytes in the epidermis of the NF1 gene on chromosome 12 refer skin. Sometimes have excessive hair ( hypertrichosis ) helicase, an enzyme essential to DNA repair to prevent chromosomal breakage have! Neurofibromas are present in > 90 % of adults with neurofibromatosis type 1 and Leopard have... Few months it faded and disappeared findings or may indicate the presence of neurofibromatosis 2 ) or appear early. Caused by mutation of the enzyme tyrosinase, which is the rate-limiting step in melanin production range in from! On cafe au lait spots are evenly colored round or oval patches that range in size number...