Café-Au-Lait Spots

Other names
Types
Causes
Symptoms
Diagnostic tests
Non-pharmacological treatments
Drug treatments
Dietary molecular supplements
Immunity booster / regenerative / stem-cell drugs
Procedures & surgeries
Prevention tips
When to see a doctor
What to eat & what to avoid
FAQs

Café-au-lait spots (CALMs) are flat, light- to dark-brown skin patches. They look like milk mixed with coffee. They can be round or oval. Edges are smooth or slightly irregular. The skin feels normal. There is no scale, pain, or itch in most cases. Many people have one or two spots and are healthy. When a person has several spots (often six or more), especially if they appear in early childhood, doctors think about a genetic condition, most commonly neurofibromatosis type 1 (NF1) or Legius syndrome. Some other rare conditions can also cause many spots. DermNet®+2DermNet®+2

Café-au-lait spots are flat, light-to-dark brown skin patches with smooth or sometimes slightly irregular borders. They are common birthmarks and usually appear at birth or in early childhood. Having one to three is very common and harmless. When a person has many (classically six or more) spots—especially larger than 5 mm in children or 15 mm in adults—doctors look carefully for genetic conditions like neurofibromatosis type 1 (NF1) or Legius syndrome, or less often McCune-Albright syndrome (MAS). CALMs themselves are benign and do not turn into cancer; treatment is not medically required and is mainly cosmetic (laser) if desired. Medscape+2DermNet®+2

CALMs are usually present at birth or show up in the first years of life. They may get darker with sun exposure and become easier to see over time. They can increase in number during childhood. The spots themselves are harmless, but multiple spots can be a clue to an underlying syndrome. Early recognition helps doctors check the child’s eyes, nerves, learning, growth, bones, and other organs for problems linked to that syndrome. DermNet®+2DermNet®+2

Other names

Café-au-lait macules; café-au-lait spots; CALMs; café-au-lait patches; pigmented birthmarks (brown macules).

Types

1) Isolated (physiologic) CALMs. One or a few spots in an otherwise healthy person. No other findings. Common in the general population. Cleveland Clinic

2) Multiple CALMs with NF1. Many CALMs plus other NF1 features (e.g., armpit/groin freckling, neurofibromas, Lisch nodules in the eyes, bone changes, learning or behavior issues). NF1 diagnostic criteria were updated in 2021 to better separate NF1 from look-alike conditions. Nature+1

3) Multiple CALMs with Legius syndrome. Many CALMs with or without armpit/groin freckling, often without neurofibromas or NF1-type tumors. May have macrocephaly and learning/attention problems. Caused by SPRED1 variants. NCBI+1

4) Multiple CALMs with other syndromes. Less common, but includes McCune-Albright syndrome, constitutional mismatch repair deficiency (CMMRD), and a few rare genetic syndromes (see “Causes”). DermNet®+2jaadcasereports.org+2

Causes

1) Normal/isolated pattern. Some people just have a few CALMs and nothing else. This is a normal variant and often needs no testing. Cleveland Clinic

2) Neurofibromatosis type 1 (NF1). A common genetic condition caused by NF1 gene variants. Many CALMs are an early sign. Other findings include axillary/inguinal freckling, Lisch nodules, neurofibromas, learning issues, bone changes, and optic pathway glioma. Diagnostic criteria were revised in 2021. Nature+1

3) Mosaic NF1. The NF1 change occurs after conception, so only part of the body is affected. CALMs may be grouped on one side or segment. Features can be milder or patchy compared to classic NF1. ScienceDirect

4) Legius syndrome. Looks like NF1 in young children because of multiple CALMs and sometimes freckling, but lacks neurofibromas and NF1-type tumors. Often includes macrocephaly and learning/attention problems. Caused by SPRED1 variants. NCBI

5) McCune-Albright syndrome (MAS). Caused by mosaic GNAS variants. CALMs are often few, large, and have irregular “coast of Maine” borders. MAS often includes fibrous dysplasia of bone and early puberty or other hormonal problems. jaadcasereports.org

6) Constitutional mismatch repair deficiency (CMMRD). A childhood cancer-predisposition syndrome due to biallelic variants in PMS2, MSH6, MLH1, MSH2. Skin signs can look like NF1: multiple CALMs (often fewer but larger), sometimes hypopigmented patches and freckling. Needs urgent genetic counseling and surveillance. MedlinePlus+1

7) Noonan spectrum disorders (RASopathies). Some children with Noonan-like features can have CALMs, especially in conditions that overlap with NF1 or SPRED1 changes. NCBI

8) Watson syndrome. An NF1-related condition with pulmonic stenosis, short stature, and multiple CALMs. It lies within the NF1 spectrum. NCBI

9) Fanconi anemia (some cases). A rare bone-marrow failure syndrome where skin pigment changes, including CALMs, may occur. Recognition matters because of cancer and hematologic risks. (Dermatology and hematology references describe pigmentary changes in Fanconi anemia.) jpedhc.org

10) Bloom syndrome (some cases). A DNA-repair disorder with growth and cancer risks; pigment changes and café-au-lait–like macules are reported. jpedhc.org

11) Silver-Russell syndrome (some cases). A growth disorder where mottled pigmentation and CALMs may be noted. jpedhc.org

12) Tuberous sclerosis complex (rare CALM-like patches). TSC primarily causes hypomelanotic macules (“ash leaf” spots), but CALM-like patches can be described in some reports—important mainly for the broad differential. jpedhc.org

13) NF1/Noonan overlap. Some individuals meet features of both NF1 and Noonan; CALMs are common in this overlap. NCBI

14) Segmental (mosaic) pigmentary disorders. Post-zygotic pigment mosaicism can produce unilateral or block-like CALMs without a systemic syndrome. DermNet®

15) Postinflammatory hyperpigmentation mistaken for CALM. Healed rashes or eczema can leave brown patches that look like CALMs but have a history of inflammation. A careful exam separates them. DermNet®

16) Lentigines syndromes confusion. LEOPARD/Noonan-with-multiple-lentigines has numerous small dark freckles (lentigines), not true CALMs, but families sometimes mix the terms; experts check carefully. ScienceDirect

17) Mastocytosis look-alikes. Urticaria pigmentosa lesions can be brown macules that mimic CALMs; rubbing them can trigger a hive (Darier sign), which CALMs do not do. DermNet®

18) Medication or endocrine pigmentation look-alikes. Diffuse or patchy darkening (e.g., Addison disease, some drugs) may be mistaken for CALMs on first glance; pattern and history clarify. Cleveland Clinic

19) Other rare genodermatoses. A few very rare genetic skin disorders list CALMs among many signs; genetics teams sort these out when common causes are excluded. jpedhc.org

20) Unknown cause. Occasionally multiple CALMs are present and no syndrome is found even after testing. Ongoing follow-up is still advised in childhood. PubMed

Symptoms

1) Brown patches on the skin. Usually flat, smooth-edged, and painless. Color may darken with sun. Often first seen in infancy or early childhood. DermNet®

2) More than six spots. A practical “red flag” for a genetic cause like NF1 or Legius, especially if they are large for age (≥5 mm before puberty, ≥15 mm after). Nature

3) Armpit/groin freckling. Tiny dark spots in skin folds suggest NF1 or Legius when CALMs are present. Nature+1

4) Soft skin bumps (neurofibromas). Appear later in NF1, usually not in Legius syndrome. PubMed

5) Eye changes. Lisch nodules (benign iris bumps) suggest NF1; routine eye checks help. PubMed

6) Learning or behavior issues. Some children with NF1 or Legius have learning difficulties or ADHD. Early school support helps. NCBI+1

7) Head size differences. Macrocephaly is common in Legius syndrome and can appear in NF1. NCBI

8) Vision problems. Rarely, NF1 causes optic pathway glioma with visual changes. Any vision complaint needs urgent eye review. PubMed

9) Bone concerns. Bowed tibia, scoliosis, or skull/long-bone changes can occur in NF1; large, irregular CALMs with bone pain or fractures suggest McCune-Albright. PubMed+1

10) Early puberty or hormone changes. Seen in McCune-Albright. jaadcasereports.org

11) Headaches or seizures. Not from CALMs themselves, but may arise in syndromes linked to CALMs; evaluation depends on the full picture. PubMed

12) Blood pressure issues. Children with NF1 should have yearly BP checks because of rare renal artery or adrenal causes of hypertension. NF Midwest

13) Hearing or balance symptoms. These are not typical for CALMs but may be evaluated if the history suggests another condition; clinicians individualize testing. ScienceDirect

14) Growth concerns. Short stature or asymmetric growth can point to specific syndromes. jpedhc.org

15) Family history of CALMs or NF1-like features. Many conditions are inherited, so family patterns matter. PubMed

Diagnostic tests

A) Physical examination (bedside assessment)

1) Full skin exam with measurement of each spot. The doctor counts CALMs, measures diameter, and notes edge shape and distribution (generalized vs segmental). Consistent documentation over time helps. DermNet®

2) Wood’s lamp exam. A UV light makes faint patches easier to see on light skin and helps separate CALMs from other pigment conditions. DermNet®

3) Eye exam with slit-lamp. Checks for Lisch nodules and other ocular signs of NF1; recommended in suspected NF1. PubMed

4) Growth and head-size charting. Plots height, weight, and head circumference; macrocephaly supports Legius/NF1; short stature or asymmetry suggests other syndromes. NCBI+1

5) Neurologic and learning/behavior screen. Looks for tone, reflexes, attention, and school performance issues that can occur in NF1/Legius. PubMed

6) Blood pressure measurement. Annual BP checks are recommended for children with NF1. NF Midwest

7) Skeletal screening in clinic. Spine check for scoliosis (forward bend test), limb asymmetry, and tibial bowing; guides imaging if needed. PubMed

8) Darier sign test (rubbing a lesion). Helps distinguish mastocytosis look-alikes: urtication suggests mastocytosis, not a CALM. DermNet®

B) Manual or simple office tests

9) Diascopy (glass slide pressure). Blanching helps differentiate vascular/brown lesions; CALMs do not blanch away. DermNet®

10) Visual acuity charting and color vision. Simple eye tests detect vision loss that could point to optic pathway glioma in NF1. PubMed

11) Hearing screening. Performed if symptoms or family history suggest broader neurocutaneous disease; helps triage for audiology. ScienceDirect

12) Developmental screening tools. Short questionnaires check for language, motor, or attention issues in NF1/Legius and guide referrals. PubMed

C) Laboratory and pathological tests

13) NF1 gene testing. Looks for pathogenic variants in NF1 to confirm NF1 or mosaic NF1 when clinical signs are unclear—especially important under the 2021 criteria. Nature

14) SPRED1 gene testing. Confirms Legius syndrome when CALMs with/without freckling exist but there are no NF1-type tumors. NCBI

15) Mismatch repair gene testing (PMS2/MSH6/MLH1/MSH2). Evaluates CMMRD when a child has CALMs with additional red flags (e.g., family history, early cancers, consanguinity). Nature

16) Targeted tissue testing for GNAS (if MAS suspected). Blood tests often miss mosaic GNAS variants; affected tissue (bone/skin) may be needed. jaadcasereports.org

17) Skin biopsy (rarely needed). CALMs are usually diagnosed clinically; biopsy is reserved for uncertain cases or to rule out look-alikes. DermNet®

D) Electrodiagnostic tests (used selectively, based on symptoms)

18) Electroencephalogram (EEG). If seizures occur in a syndrome linked to CALMs, EEG helps classify and guide therapy. PubMed

19) Visual evoked potentials (VEPs). If vision symptoms or optic pathway concerns arise in NF1, VEPs can support assessment along with eye exams and imaging. PubMed

20) Brainstem auditory evoked responses (BAER). If hearing or brainstem pathway concerns exist, BAER can help—ordered case-by-case. ScienceDirect

E) Imaging tests (ordered according to findings)

21) MRI of the brain/orbits. Not done routinely for CALMs, but used if there are NF1 red flags (vision change, neurologic signs) to assess optic pathway glioma or other issues. PubMed

22) Skeletal X-rays or MRI. Follow up on clinic findings like tibial bowing or scoliosis in NF1, or bone pain/deformity in suspected McCune-Albright. PubMed+1

23) Bone scans/CT (selected cases). For extensive fibrous dysplasia in MAS, imaging helps map bone involvement and plan care. jaadcasereports.org

24) Abdominal imaging (only if indicated). Used for specific NF1 complications (e.g., suspected pheochromocytoma in an older child/adult with hypertension or symptoms). NF Midwest

Non-pharmacological treatments

Reality check: CALMs are safe birthmarks. Treatment is optional and cosmetic. No cream has proven to permanently erase CALMs; lasers can help some people, with variable clearance and recurrence. DermNet®+1

Education & reassurance (they’re benign). Purpose: reduce fear. Mechanism: informed decision-making reduces unnecessary procedures. DermNet®
Sun protection daily (shade, clothing, broad-spectrum SPF ≥ 30, reapply 2-hourly outdoors). Purpose: prevent darkening/contrast. Mechanism: blocks UV that deepens pigment. American Academy of Dermatology+1
Cosmetic camouflage (makeup/tinted sunscreen). Purpose: immediate concealment. Mechanism: optical blending. AAFP
Q-switched Nd:YAG (1064 nm) laser. Purpose: lighten CALMs. Mechanism: selective photothermolysis of melanosomes. Evidence suggests favorable clearance in many patients. PMC+1
Q-switched alexandrite (755 nm). Alternative wavelength; some series show good outcomes. Wiley Online Library
Q-switched ruby (694 nm). Another historical option with variable results. DermNet®
Picosecond lasers (e.g., 730 nm titanium sapphire). Newer platforms with promising case-series data. Wiley Online Library
Er:YAG or fractional resurfacing (selected cases). Purpose: texture-sparing pigment disruption; careful selection needed. ScienceDirect
Test spot + staged treatments. Purpose: predict response/side-effects, especially in darker skin. Mechanism: minimizes PIH risk. PMC
Strict post-laser photoprotection. Purpose: cut rebound pigmentation. Mechanism: prevents UV-driven melanogenesis. AAFP
Psychological support/counseling if appearance causes distress. Purpose: QoL. NCBI
School/work advocacy (anti-bullying support). Purpose: social functioning. NCBI
Regular NF1/Legius follow-up when criteria are met. Purpose: early detection of complications. The Lancet
Ophthalmology surveillance (NF1). Purpose: detect optic pathway glioma/Lisch nodules. DermNet®
Endocrine evaluation (suspected MAS). Purpose: treat precocious puberty/thyroid issues. NCBI
Audiology/ENT review (suspected NF2). Purpose: catch vestibular schwannomas early. DermNet®
Body-image coaching/dermatology photography to track change and reduce anxiety. Purpose: objective monitoring. NCBI
Patch test avoidance of irritants in treated skin (reduce PIH risk). Purpose: protect barrier during laser series. AAFP
Shared decision aids (photos, expected sessions, recurrence risk). Purpose: informed consent. PMC
Genetic counseling for family planning when a syndrome is confirmed. Purpose: inheritance risk discussion. NCBI

Drug treatments

Key point: There are no FDA-approved medications specifically to remove or lighten café-au-lait macules. Cosmetic bleaching agents (e.g., hydroquinone) have not shown consistent, durable benefit for CALMs, and guidelines emphasize lasers if treatment is desired. For NF1-related plexiform neurofibromas (a different tumor problem), the MEK inhibitor selumetinib is FDA-approved—not to treat CALMs, but to shrink inoperable plexiform neurofibromas in children ≥2 years. FDA Access Data+3DermNet®+3YMAWS+3

Because your brief asks for “20 drug treatments (from accessdata.fda.gov) for this disease,” the evidence base simply does not support such a list for CALMs. Listing drugs would be misleading. The one relevant FDA label to be aware of in this space is selumetinib (KOSELUGO®) for NF1 plexiform neurofibromas; again, it does not lighten café-au-lait macules. FDA Access Data

If you still want a drug section, the only responsible way is to explain why drugs are not indicated, outline off-label agents tried historically (e.g., hydroquinone, tretinoin, triple-combination creams) and clearly state their limitations for CALMs so readers are not misled. AAFP

Dietary molecular supplements

There is no supplement proven to erase CALMs. Some oral agents offer modest photoprotection (may reduce UV-induced darkening), which can help keep spots from appearing darker relative to surrounding skin. Summaries below reflect evidence for photoprotection, not CALM clearance:

Polypodium leucotomos (PLE) — 240–480 mg/day in studies; antioxidant/anti-inflammatory; increases minimal erythema dose; generally well-tolerated. PMC+1
Carotenoids (β-carotene, lycopene, lutein/zeaxanthin) — dietary or supplements; integrate into skin and absorb UV; gradual effect over weeks. Wiley Online Library
Green tea polyphenols — catechins reduce UV-mediated oxidative stress; adjunct only. OAE Publishing
Pomegranate extract — polyphenols with UV-modulating properties; small trials only. OAE Publishing
Resveratrol — antioxidant/NRF2-linked pathways; limited human data. OAE Publishing
Silymarin — plant flavonolignans with photoprotective signals; adjunctive. OAE Publishing
Vitamin C (ascorbic acid) — cofactor/antioxidant; supports collagen and reduces oxidative stress; best as diet + topical, not as a sole oral agent. Frontiers
Vitamin E (tocopherols) — lipid antioxidant; often combined with C; modest effect. Frontiers
Niacinamide — supports barrier/reduces UV immunosuppression; oral data are mixed; stronger evidence topically. Frontiers
Combined photoprotective formulas (e.g., PLE + polyphenols + vitamins) show small RCT signals for higher UV dose before redness. MDPI

Editorial caution for readers: supplements do not replace sunscreen/clothing; effects are additive at best. Liebert Online

Immunity booster / regenerative / stem-cell drugs

There are no immune-boosting, regenerative, or stem-cell drugs approved or supported by evidence to treat CALMs. Recommending such products would be unsafe and misleading. The correct, ethical advice is avoid these claims and focus on sun protection and, if desired, laser with realistic expectations. DermNet®

Procedures & surgeries

Q-switched laser series (Nd:YAG / alexandrite / ruby) — outpatient sessions; risk of temporary or permanent hypo/hyperpigmentation; clearance varies; recurrence can occur. PMC+1
Picosecond laser — newer option; early data promising, still evolving. Wiley Online Library
Er:YAG / fractional resurfacing (selected cases) — less common; operator-dependent. ScienceDirect
Surgical excision — rarely considered (small, discrete lesions in high-impact areas); leaves a line scar; not first-line. AAFP
No procedure — the right choice for many; document and monitor if syndromic signs appear later. DermNet®

Prevention tips

Daily broad-spectrum SPF ≥ 30; reapply every 2 hours outdoors. American Academy of Dermatology+1
UPF clothing, wide-brim hat, and shade, especially 10 am–4 pm. American Cancer Society
Do not chase extreme SPF numbers; technique and reapplication matter more. American Academy of Dermatology
Avoid DIY “bleaching” or unregulated products. Verywell Health
Plan lasers outside peak-sun seasons when possible. AAFP
Patch-test cosmetics after laser to reduce irritation/PIH. AAFP
Healthy photoprotection diet (antioxidant-rich foods) as adjunct. Frontiers
Keep records/photos to avoid unnecessary repeat procedures. NCBI
Family screening and counseling if NF1/Legius/MAS suspected. The Lancet
Follow evidence, not myths (anti-sunscreen misinformation is harmful). The Washington Post

When to see a doctor

You count six or more CALMs (>5 mm child; >15 mm adult). DermNet®
New freckling in armpits/groin or soft nodules appear. DermNet®
Vision changes, eye pain, or rapid head growth in a child. DermNet®
Signs of early puberty or other endocrine issues. NCBI
Bone pain/deformity (bowing) or unexplained fractures. DermNet®
Hearing loss, tinnitus, or imbalance. DermNet®
Significant anxiety or bullying related to appearance (ask for support). NCBI

What to eat & what to avoid

Eat: colorful fruits/vegetables (polyphenols, carotenoids), nuts/seeds (vitamin E), oily fish (omega-3s). These support skin health and may add slight photoprotection alongside sunscreen. Wiley Online Library+1
Avoid/limit: tanning and intentional sunburns; rely on food + sunscreen rather than unproven “skin-lightening” diets; avoid online “bleaching” concoctions (risk of burns/PIP). Verywell Health
Hydrate and maintain a balanced diet—helpful for recovery after procedures and overall skin function. Frontiers

FAQs

Do CALMs turn into cancer? No—they are benign. DermNet®
How many spots are “concerning”? ≥6 (size criteria apply) → get evaluated. DermNet®
Can creams lighten CALMs? Not reliably; lasers are the main option if you want treatment. DermNet®
Which laser works best? Evidence favors Q-switched 1064 nm Nd:YAG in many studies, but results vary. PMC
Will spots come back after laser? Recurrence can happen; rates vary by series and lesion pattern. PMC
Are lasers safe for darker skin? Possible with expert settings and strict sun care; test spots help reduce PIH risk. PubMed
Is there a pill to remove CALMs? No. No FDA-approved drug removes CALMs. DermNet®
What about selumetinib? It’s for NF1 plexiform neurofibromas, not CALMs. FDA Access Data
Do CALMs mean I have NF1? Not by themselves; doctors look at the whole picture (freckling, neurofibromas, eye/bone signs, family history, genetics). ScienceDirect
Can diet or supplements erase CALMs? No; at best, some supplements add photoprotection as an adjunct. Liebert Online
Should children be tested? If they meet or approach criteria, clinicians may do genetic testing and structured follow-up. The Lancet
How often is follow-up in NF1? ERN GENTURIS suggests adults at least every 3 years (with additional checks as needed). The Lancet
Can CALMs be prevented? You can’t prevent the birthmarks, but sun protection stops them from looking darker. American Academy of Dermatology
Are irregular borders always bad? In MAS, CALMs often have irregular, midline borders—this pattern guides evaluation. MedlinePlus
Is it okay to do nothing? Yes. For most people, no treatment is the right choice. DermNet®

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.