Neurofibromatosis Type 6

Other names
Types
Causes
Symptoms
Diagnostic tests
Non-pharmacological treatments (therapies & others)
Drug treatments
Dietary molecular supplements
Immunity booster / regenerative / stem-cell drugs
Procedures and why they’re done
Prevention
When to see a doctor
What to eat and what to avoid
Frequently asked questions

“NF type 6” (older name) or café-au-lait spots syndrome / familial isolated café-au-lait macules is a skin-only condition. People have several flat, light-to-dark brown patches (like “coffee with milk”) with smooth edges. There are no neurofibromas, no optic gliomas, no vestibular schwannomas, and no internal tumors that define NF1 or NF2. Doctors mainly watch the skin and the child’s growth/learning, and they check early in life for signs of NF1; many families are simply CALM-only. Genetic testing may also look for SPRED1 variants (Legius syndrome), which causes NF1-like spots without tumors. NCBI+2orpha.net+2

Neurofibromatosis type 6 (NF6) is an old label from the 1980s. It was part of the Riccardi system that tried to sort many skin and nerve findings into “types.” In that list, “type VI” meant “café-au-lait spots only.” Café-au-lait spots are flat, light-to-dark brown patches on the skin. They are harmless by themselves. If you have only multiple café-au-lait spots and none of the other signs of neurofibromatosis (no neurofibromas, no Lisch nodules, no optic glioma), some older papers called this “NF6.” Today, experts do not use this “type” system. Instead, doctors check if a person meets the modern criteria for NF1, or if they have Legius syndrome, or if they simply have isolated café-au-lait macules (CALMs) without a syndrome. NCBI+3Mahidol University+3PMC+3

Other names

People have used several names for this “café-au-lait only” picture:

NF6
Café-au-lait spots syndrome
Familial multiple café-au-lait spots
Multiple café-au-lait macules (CALMs) without other NF1 features
NF1-like pigmentary features (often later shown to be Legius syndrome, not NF1) MalaCards+2MD Edge+2

Types

Doctors no longer use the Riccardi “types” to diagnose patients. Instead, they sort patients with café-au-lait spots into these modern groups:

Neurofibromatosis type 1 (NF1) – needs specific clinical or genetic criteria (for example, ≥6 café-au-lait macules of a certain size plus another sign such as freckling in the armpit/groin, neurofibromas, eye or bone findings, a pathogenic NF1 variant, etc.). NCBI
Legius syndrome (SPRED1-related disorder) – looks like NF1 in young children because of multiple café-au-lait spots and sometimes freckling, but there are no neurofibromas or NF1-related tumors; it is due to variants in the SPRED1 gene. NCBI
Mosaic (segmental) NF1 or mosaic Legius syndrome – pigment changes (and sometimes other signs) limited to one body area due to a post-zygotic mutation; in young children who only have café-au-lait spots and freckling, mosaic NF1 is often the most likely explanation. PMC
Isolated or familial multiple café-au-lait macules (no syndrome) – some people have many café-au-lait spots and never develop other features; careful follow-up is used in early childhood. JAMA Network

A child with only café-au-lait spots does not automatically have NF1. Many children who start with café-au-lait spots alone later meet criteria for NF1; others prove to have Legius syndrome; and some remain “CALMs only.” Modern, updated criteria help doctors tell these apart, and genetic testing can confirm. PMC+1

Causes

Below are simple, one-paragraph explanations for reasons someone can have multiple café-au-lait spots without classic NF1 signs at first. Some are genetic syndromes; some are patterns like mosaicism. Doctors use the story, the exam, and—in many cases—genetic testing to tell them apart.

Isolated multiple café-au-lait macules (no syndrome). Some people, and some families, have several café-au-lait spots and stay healthy with no other features. This can be sporadic or run in families. Doctors watch over time to see if other signs appear. MD Edge
Early NF1 before other features appear. In young children, café-au-lait spots often show up first. Other NF1 signs (freckling, neurofibromas, eye or bone findings) may appear later, so doctors cannot diagnose NF1 until a second criterion appears or a pathogenic NF1 variant is found. NCBI+1
Legius syndrome (SPRED1). This condition causes multiple café-au-lait spots and sometimes armpit/groin freckling and learning issues. It does not cause neurofibromas or the tumor risks seen in NF1. Genetic testing of SPRED1 confirms it. NCBI
Mosaic (segmental) NF1. A post-zygotic NF1 change affects only part of the body, so café-au-lait spots or other signs may sit in a band or patch on one side. The rest of the body can look normal. This can mimic “NF6” if only spots are seen. PMC
Mosaic (segmental) Legius syndrome. Like mosaic NF1, but the gene is SPRED1. Pigment changes can be limited to one segment with no neurofibromas and lower tumor risk. PMC
McCune–Albright syndrome (GNAS). Irregular, “coast of Maine” café-au-lait patches with jagged edges, plus bone disease (fibrous dysplasia) and early puberty or other endocrine issues. The skin patches can be the first clue. NCBI
Noonan syndrome with multiple lentigines (formerly LEOPARD). This RAS-MAPK pathway disorder can produce many small dark macules and café-au-lait spots along with heart and facial features. NCBI
Watson syndrome (allelic to NF1). Features can include café-au-lait spots, pulmonary artery narrowing, and short stature. It overlaps the RAS-MAPK disorders. NCBI
Constitutional mismatch repair deficiency (CMMRD). Children can have many café-au-lait spots that resemble NF1, but they have biallelic DNA repair gene variants and very high cancer risks. Pigment changes in a young child may be the first sign. NCBI
Fanconi anemia. Some patients show café-au-lait spots with bone marrow and limb findings; the spots alone can lead to confusion with NF1 in early life. NCBI
Bloom syndrome. This DNA repair disorder can present with pigment changes, short stature, and high cancer risk; café-au-lait spots may be part of the picture. NCBI
Ring chromosome syndromes (e.g., ring 7). Structural chromosome changes can cause multiple pigment patches including café-au-lait spots and growth issues. NCBI
KITLG or other pigment-pathway variants. Rare variants in pigment-signaling genes can cause CALMs without a broader syndrome, sometimes running in families. NCBI
Physiologic/familial CALMs. One or two café-au-lait spots are common in the general population and do not mean disease. In some families, more spots occur but remain benign. NCBI
Post-inflammatory hyperpigmentation mimicking CALMs. A healed rash, burn, or friction area can leave flat brown patches that look like CALMs but have a different cause. Clinicians use exam details to tell them apart.
Mastocytosis (urticaria pigmentosa) confusion. Brownish macules from mast cells can be mistaken for CALMs at a glance; rubbing can cause wheal/itch (Darier sign), which CALMs do not show.
Segmental pigmentary mosaicism. Some people have a block or swath of darker skin from early development. These are not CALMs but can look similar.
Silver–Russell syndrome. Growth restriction with body asymmetry; café-au-lait spots may be present. NCBI
Tuberous sclerosis complex confusion. TSC classically shows hypopigmented “ash leaf” spots, but mixed pigment changes can confuse the picture; careful light exam (Wood’s lamp) helps. NCBI
Normal sun-darkening of existing CALMs. CALMs can darken with sun and seem to “increase,” which can worry families, even when no new disease is present. Education helps. Cleveland Clinic

Symptoms

Because “NF6” was defined as café-au-lait spots only, most people have no other symptoms at all. Still, a careful list helps parents and patients know what to watch for, because some children later meet NF1 criteria or are found to have Legius syndrome.

Flat light-brown patches from birth or early childhood. Patches can grow slowly with age and sun. They do not itch, hurt, or bleed. NCBI
Many spots (often >6) but otherwise feel fine. This is the most common story for “NF6,” and there are no bumps or nerve tumors. MD Edge
Family members with similar spots. Sometimes parents or siblings have similar pigment patches without problems. MD Edge
Armpit or groin freckles. If these appear, the picture shifts toward NF1 or Legius syndrome rather than pure “NF6.” NCBI
No skin tumors (neurofibromas). By definition of “café-au-lait only,” there are no neurofibromas. If small skin bumps appear later, a doctor reassesses for NF1. NCBI
Normal eyes at first exam. No Lisch nodules or optic pathway glioma are expected in “CALMs only.” If vision changes occur, doctors check for NF1 signs. NCBI
Normal hearing. Hearing loss points away from pigment-only patterns and raises other diagnoses (e.g., NF2), which is a different disease. NCBI
Learning or attention issues (some patients). This is more common in NF1 and can occur in Legius syndrome; it is not required in CALMs-only. NCBI
Head size slightly large (macrocephaly) in some Legius cases. If present, doctors think of SPRED1 testing. NCBI
No bone pain or deformity. CALMs alone do not cause bone problems. Bone issues suggest NF1 or McCune–Albright rather than “NF6.” NCBI
No weakness or numbness. CALMs do not affect nerves.
No seizures from CALMs themselves. If seizures occur, doctors look for other causes.
Stable general health. CALMs do not raise tumor risk by themselves; tumor risks come from the underlying syndrome (e.g., NF1), which “NF6” lacks. NCBI
Cosmetic concern. Some people feel self-conscious about the spots; counseling and, sometimes, laser can help for appearance. NCBI
Anxiety about the future. Clear education about what to watch for (and what not to fear) helps families and reduces worry. Cleveland Clinic

Diagnostic tests

Doctors confirm whether café-au-lait spots are isolated, NF1, Legius syndrome, or mosaic forms. The plan uses the physical exam first. Imaging or lab tests are used only when needed.

A) Physical examination (bedside checks)

Full skin exam in good light. Count spots, measure size (≥5 mm before puberty, ≥15 mm after puberty are NF1 size cutoffs), and note borders. Check for axillary/inguinal freckling. These details decide if NF1 criteria are met or if the pattern is CALMs-only. NCBI
Wood’s lamp exam. Blue light helps find faint spots and tells CALMs from other pigment issues (e.g., ash-leaf spots in TSC). This is quick and painless.
Eye exam (slit lamp when age-appropriate). An ophthalmologist looks for Lisch nodules or optic nerve signs. Absence supports CALMs-only or Legius; presence supports NF1. NCBI
Neurologic exam. Reflexes, strength, vision fields, and coordination are checked. CALMs alone should have a normal neuro exam.
Growth and head circumference. Macrocephaly may point to Legius; short stature or other dysmorphisms may point to a different RASopathy. NCBI
Skeletal look-over. Bowed tibia or sphenoid dysplasia suggests NF1, not CALMs-only. NCBI
Hearing screen (age-appropriate). Routine if there are concerns; pigment-only patterns should have normal hearing. Problems hint at other diagnoses (e.g., NF2). NCBI
Family history and pedigree. Who else has spots? Any tumors? This helps pick the right genetic test and the right follow-up. NCBI

B) Manual office tests and tools

Dermatoscopy of lesions. A hand scope can confirm even pigment and smooth borders typical of CALMs and exclude melanocytic lesions.
Standard photos and body map. Photos track number and size over time. This helps decide if new features appear later.
Vision screening (chart/fields) in clinic. Simple checks support or refute optic pathway concerns when formal ophthalmology is not yet possible. NCBI

C) Laboratory and pathological tests

NF1 gene testing (blood/saliva). If the clinical picture is unclear or if only pigment signs are present, molecular testing can confirm or exclude NF1. Modern criteria allow genetic diagnosis. PMC
SPRED1 gene testing (Legius). If NF1 testing is negative and the person has CALMs with/without freckling and no neurofibromas, SPRED1 testing is recommended. NCBI
Targeted panels for “NF1-like” syndromes. Some centers use RAS-MAPK pathway panels or pigment-disorder panels to look for other rare causes when NF1/SPRED1 are negative. NCBI
Testing for mosaicism (deep sequencing/skin biopsy DNA). If signs are segmental or limited to one body area, testing affected skin can reveal a mosaic NF1 or SPRED1 variant missed in blood. PMC
Histology (rarely). A skin biopsy is not needed for diagnosis, but if done for another reason, CALMs show increased melanin in basal cells; this does not prove a specific syndrome. NCBI

D) Electrodiagnostic tests

Electroretinography/visual evoked potentials (selected cases). Not routine for CALMs-only. Used only if there are visual signs suggesting optic pathway disease. In “NF6-pattern,” this is usually not needed. NCBI
Nerve conduction studies/EMG (selected cases). Not routine. Consider only if there are symptoms of neuropathy (which CALMs do not cause).

E) Imaging tests

MRI of brain/orbits (only if indicated). Imaging is not recommended for an asymptomatic child with CALMs only. MRI is used if vision changes, neurologic signs, or other NF1 features appear. NCBI
Whole-body MRI or regional MRI (rare, specialist-guided). Reserved for people with symptoms or findings that suggest internal plexiform neurofibromas—absent by definition in a pure CALMs-only pattern. NCBI

Non-pharmacological treatments (therapies & others)

For CALM-only/NF6 there’s no medicine that erases spots reliably. Care focuses on education, skin care, cosmetic options, psychosocial support, and periodic checks—especially in children to ensure we’re not missing NF1. Each item below gives description (~150 words), purpose, and mechanism in simple terms.

Reassurance & education
What it is: A clear talk about what CALMs are, what they are not, and how they behave over time. Purpose: Reduce fear and stop needless tests. How it works: Understanding that CALMs are harmless pigment patches lowers anxiety and prevents over-treatment; families also learn signs that would prompt re-evaluation (new lumps, eye issues, learning problems). Evidence shows multiple CALMs can be seen in healthy people; six or more may prompt evaluation for NF1/Legius, but many children with only CALMs never develop tumors. Cleveland Clinic+1
Dermatology follow-up in childhood
What it is: Skin and growth checks every 6–12 months in early years; less often later. Purpose: Make sure no features of NF1 appear (neurofibromas, freckling in the armpits/groin, bone changes) and track school/learning. How it works: Physical exam and history catch evolving signs early; most CALM-only children stay CALM-only. NCBI
Genetic counseling
What it is: A meeting to discuss inheritance, testing choices (NF1 vs SPRED1), and family planning. Purpose: Clarify risk for children and relatives. How it works: Counselors explain that CALM-only may run in families; if a SPRED1 variant (Legius) is found, it explains the spots and lack of tumors; if testing is negative and the exam stays normal, ongoing tumor risk remains low. NCBI
Targeted genetic testing (NF1 & SPRED1) when indicated
What it is: Blood/saliva test for variants. Purpose: Distinguish NF1 from Legius or CALM-only. How it works: Finding SPRED1 confirms Legius; a negative NF1 test with typical CALMs and no tumors supports CALM-only; this steers follow-up and avoids unnecessary tumor surveillance. NCBI
High-SPF sun protection
What it is: Daily broad-spectrum SPF 30+ and clothing. Purpose: Prevent darkening and edge contrast of patches and protect skin health. How it works: UV light increases melanin activity, making CALMs more visible; sunscreen limits that. Cleveland Clinic
Cosmetic camouflage (makeup/cover creams)
What it is: Pigment-matched concealers for spots. Purpose: Improve self-image without medical risk. How it works: Optical blending hides color differences; removable and adjustable to preference. Cleveland Clinic
Psychosocial support / counseling
What it is: Short-term therapy or support groups for body-image concerns. Purpose: Reduce teasing impact and improve confidence. How it works: Cognitive-behavioral strategies and peer support help children and adults reframe concerns about visible patches. Cleveland Clinic
School support and screening for learning/attention
What it is: Teacher awareness and optional screening. Purpose: Because Legius can be associated with ADHD/learning issues in some families, early supports help if needed. How it works: Simple classroom adjustments if issues appear; many CALM-only children need nothing beyond routine. NCBI
Laser therapy: Q-switched 1064-nm Nd:YAG
What it is: Short-pulse laser that targets melanin. Purpose: Lighten CALMs for cosmetic reasons. How it works: Selective photothermolysis breaks up pigment; multiple sessions are common; meta-analyses show meaningful clearance in many patients, with low adverse-event rates. Recurrence can occur. PMC+2BioMed Central+2
Laser therapy: picosecond devices (755/1064 nm)
What it is: Ultra-short pulses. Purpose: Similar to above, sometimes with fewer side effects. How it works: Even shorter pulses may fragment pigment more efficiently; recent studies show comparable efficacy with potentially fewer complications. PubMed+1
Patch-testing before full laser
What it is: Treat a small area first. Purpose: Predict response and reduce risk of hypo/hyperpigmentation. How it works: If the test spot heals well with lightening, larger areas are treated. PubMed
Alternative lasers (ruby 694 nm, alexandrite 755 nm, 532-nm)
What it is: Other pigment lasers when Nd:YAG/picosecond are unsuitable. Purpose: Cosmetic lightening. How it works: Melanin-selective wavelengths; outcomes vary by skin type, border shape, and lesion depth; historical data show mixed clearance and some recurrence. Wiley Online Library+1
Gentle skin care routine
What it is: Fragrance-free cleansers and moisturizers. Purpose: Maintain healthy barrier and even tone. How it works: Reduces irritation and post-inflammatory hyperpigmentation risk around treated areas. Medscape
Photography-based monitoring
What it is: Yearly standardized photos. Purpose: Track changes objectively. How it works: Helps families and clinicians see stability over time and avoid over-interpreting normal growth-related changes. Cleveland Clinic
Sun-smart clothing & shade planning
What it is: UPF fabrics, hats, and avoiding peak UV. Purpose: Keeps color contrast down and protects skin. How it works: Less UV → less pigment stimulation. Cleveland Clinic
Expectant management (do nothing)
What it is: Choosing not to treat cosmetically. Purpose: Avoid procedures when spots don’t bother the person. How it works: CALMs are benign; many people simply accept them. Cleveland Clinic
Address misinformation
What it is: Provide vetted info (Cleveland Clinic, MedGen/Orphanet, GeneReviews). Purpose: Prevent unnecessary fear of “cancer risk.” How it works: Accurate resources clarify that CALM-only lacks NF1 tumor spectrum. Cleveland Clinic+2NCBI+2
Pediatric ophthalmology once if NF1 concern exists
What it is: Eye exam in early childhood only if NF1 features suspected. Purpose: Screen for NF1 complications (not expected in CALM-only). How it works: Baseline check reassures families when normal. NCBI
Shared decision-making for cosmetic lasers
What it is: Discuss benefits, recurrence, costs. Purpose: Align expectations. How it works: Evidence shows good but variable clearance; multiple sessions often needed; recurrence ~13% in pooled data. Medscape
Referral to a neurocutaneous clinic if uncertainty
What it is: Multidisciplinary review when the diagnosis is unclear. Purpose: Distinguish NF1, Legius, segmental NF, or CALM-only. How it works: Combined dermatology/genetics/neurology input reduces mislabeling. PubMed

Drug treatments

Key fact: There are no FDA-approved drugs to treat café-au-lait macules themselves. Topical lightening agents (like hydroquinone or tretinoin) may be used off-label for other pigment disorders and work variably on CALMs; evidence for CALM clearance is limited, and lasers outperform creams. Below I list the only FDA-approved systemic drugs relevant to the NF spectrum (both for NF1 plexiform neurofibromas, not for CALMs/NF6), followed by a brief note on commonly discussed topical options (off-label). I will not invent “NF6 drugs,” because none exist. Medscape

1) Selumetinib (KOSELUGO)
Class: MEK1/2 inhibitor. Who/when: FDA-approved (April 2020) for children ≥2 years with NF1 and symptomatic, inoperable plexiform neurofibromas. Dose/time: Label-based pediatric dosing twice daily on an empty stomach; dose by body surface area with specific adjustments for toxicity. Purpose: Shrink/discourage growth of plexiform neurofibromas; not for CALMs. How it works: Blocks MEK in the RAS/MAPK pathway overactive in NF1, slowing tumor cell signaling. Side effects: GI upset, rash, elevated creatine phosphokinase, cardiomyopathy risk, ocular events; monitoring is specified in the label. Evidence: FDA label and approval notice. FDA Access Data+1

2) Mirdametinib (GOMEKLI)
Class: MEK1/2 inhibitor. Who/when: FDA-approved Feb 11, 2025 for adults and children ≥2 years with NF1 who have symptomatic plexiform neurofibromas not amenable to complete resection. Dose/time: Label provides weight-based capsules and tablets for oral suspension on an intermittent schedule; details in FDA labeling. Purpose: Reduce PN size/symptoms; not for CALMs. Mechanism: MEK inhibition in RAS/MAPK pathway. Side effects: Similar MEK-class effects—rash, diarrhea, edema, CPK elevations, ocular and cardiac risks; label outlines monitoring. Evidence: FDA approval announcement and labeling. FDA Access Data+3U.S. Food and Drug Administration+3FDA Access Data+3

Topical “lightening” agents (off-label for CALMs; variable benefit):

Hydroquinone 4%: Tyrosinase inhibition reduces melanin production; evidence in CALMs is inconsistent; avoid in young children and long-term continuous use; lasers tend to work better for CALMs. Medscape
Topical retinoids (tretinoin/adapalene): Increase cell turnover; sometimes used for pigment blending but CALMs often persist; irritation common. Medscape
Azelaic acid, kojic acid, vitamin C: Mild pigment-modulating effects in melasma/post-inflammatory hyperpigmentation; no strong CALM-specific evidence; may be tried cautiously in adults. Medscape

Because you requested “20 drug treatments,” I’m being deliberately conservative: beyond selumetinib and mirdametinib (for NF1-PN, not CALMs), there are no FDA-approved drugs for “NF6/CALM-only.” Listing 18 more “drugs” would be misleading or off-label without CALM-specific evidence. The safest, evidence-based plan for CALMs is non-drug care and laser options above. FDA Access Data+1

Dietary molecular supplements

Important: There are no supplements proven to fade café-au-lait macules or treat “NF6.” The points below are general skin-health or safety tips; none are CALM cures. Please discuss any supplement with your clinician, especially for children.

Vitamin D (if deficient) – supports general health; correct only when blood tests show deficiency; not a CALM treatment. Cleveland Clinic
Omega-3 fatty acids – may help skin barrier and inflammation moderation; no CALM-specific effect proven. Cleveland Clinic
Vitamin C (oral) – antioxidant; helps overall skin health; topical forms are sometimes used for pigment, but data for CALMs are lacking. Medscape
Zinc (if low) – supports healing/immune function; correct deficiency only. Cleveland Clinic
Protein-adequate diet – supports skin turnover after laser; no effect on CALMs alone. Medscape
Probiotics – general skin–gut interest but no evidence for CALMs. Cleveland Clinic
Green-tea extract (EGCG) – antioxidant; topical uses studied for hyperpigmentation generally, not CALMs. Medscape
Niacinamide (oral/ topical) – barrier and pigment-transfer effects in melasma; not proven for CALMs. Medscape
Avoid megadose herbal “skin lighteners” – safety concerns; no evidence for CALMs. Medscape
Hydration + low-irritant diet pattern – supports skin tolerance if doing laser; no CALM lightening by itself. Medscape

Immunity booster / regenerative / stem-cell drugs

There are no approved immunity-boosting, regenerative, or stem-cell drugs for CALMs/NF6. Promoting such treatments would be misleading and potentially unsafe. Management should stick to evidence-based options above (observation, sun care, lasers) and FDA-approved MEK inhibitors only for NF1 plexiform neurofibromas—which is a different indication than CALMs. FDA Access Data+1

Procedures and why they’re done

Q-switched 1064-nm Nd:YAG laser – Most widely supported option to lighten CALMs; chosen for its melanin targeting and safety profile; done in multiple sessions. PMC+1
Picosecond 755/1064-nm laser – Alternative with comparable lightening and possibly fewer side effects in some studies. PubMed+1
Test spot (patch test) before full treatment – Predicts response and reduces pigmentary risks. PubMed
Fractional/adjunct lasers in selected cases – Sometimes used if standard lasers fail, but evidence is limited; decisions individualized. BioMed Central
Surgical excision – Rarely used and generally discouraged for flat, large CALMs because it causes scars larger than the spot; considered only if a lesion is atypical and biopsy is needed to confirm diagnosis. Medscape

Prevention

You can’t prevent being born with CALMs; they’re genetic/constitutional. What you can do is protect skin to keep contrast down. Cleveland Clinic
Daily sunscreen (SPF 30+) on exposed areas. Cleveland Clinic
UPF clothing/hat outdoors. Cleveland Clinic
Avoid tanning/UV lamps. Cleveland Clinic
Gentle skin care—avoid harsh scrubs that inflame skin. Medscape
Plan laser after summer to reduce post-treatment pigment shifts. Medscape
Photograph spots yearly to track stability. Cleveland Clinic
Seek evaluation if new lumps, vision issues, or learning problems appear—to re-check for NF1 features. NCBI
Use camouflage cosmetics if spots bother you; safe and reversible. Cleveland Clinic
Rely on reputable sources (GeneReviews/Orphanet/MedGen) to avoid misinformation. NCBI+2orpha.net+2

When to see a doctor

A child has six or more café-au-lait spots, especially if new ones appear quickly. Cleveland Clinic
Any new lumps, skinfold freckling, bone pain/deformity, vision problems, or learning/attention concerns. NCBI
If you’re considering laser treatment and want guidance on device choice and expectations. Medscape
If you want genetic counseling/testing to clarify Legius vs NF1 vs CALM-only. NCBI

What to eat and what to avoid

There’s no diet that removes CALMs. These tips support overall skin health and safe laser recovery:

Balanced diet with lean protein to support healing if you have a procedure. Medscape
Plenty of fruits/vegetables (antioxidants) for general skin wellness. Medscape
Hydration to maintain skin barrier. Medscape
Vitamin D adequacy if deficient (check first). Cleveland Clinic
Avoid megadose “skin-lightening” supplements—unproven and risky. Medscape
Limit alcohol before/after laser sessions to reduce swelling/bruising risk. Medscape
Stop smoking to improve skin healing if you undergo procedures. Medscape
Avoid irritating spices/very hot drinks immediately after laser to limit flushing. Medscape
Use sunscreen regardless of diet—food cannot replace UV protection. Cleveland Clinic
Discuss any herbs (e.g., ginkgo, high-dose fish oil) with your doctor before laser since they can increase bruising. Medscape

Frequently asked questions

Is “NF type 6” the same as NF1?
No. “NF6” is an outdated label for CALM-only; NF1 includes tumors like plexiform neurofibromas, which CALM-only lacks. NCBI
Could CALM-only actually be Legius syndrome?
Yes—Legius can look very similar (multiple CALMs ± freckling) but no NF1 tumors; SPRED1 testing can confirm. NCBI
Do café-au-lait spots mean cancer?
No. The spots are benign pigment patches. Evaluation is needed mainly when there are many spots or other NF1 signs. Cleveland Clinic
Can creams remove CALMs?
Usually not. Lightening creams have inconsistent results; lasers work better when cosmetic treatment is desired. Medscape
Which laser has the best evidence?
Q-switched 1064-nm Nd:YAG has strong support; picosecond devices are comparable and may have fewer side effects. PMC+1
Do spots come back after laser?
They can; meta-analysis suggests recurrence around 13% overall; multiple sessions are typical. Medscape
Are there medicines that shrink CALMs?
No medicines reliably remove CALMs. Systemic MEK inhibitors (selumetinib, mirdametinib) are for NF1 plexiform neurofibromas, not CALMs. FDA Access Data+1
When should a child with CALMs get genetic testing?
Consider testing if there are many spots, family history, or other features suggesting NF1 or Legius; your clinician will guide timing. NCBI
Can sun make CALMs worse?
Sun doesn’t create them but darkens contrast; sunscreen and clothing help. Cleveland Clinic
Are CALMs birthmarks?
Yes—many are present at birth or appear early; some people have a few with no disease at all. Cleveland Clinic
Do CALMs affect learning?
CALMs alone do not; Legius can be linked with ADHD/learning issues for some, so screening is reasonable if concerns arise. NCBI
Is segmental (localized) presentation possible?
Yes, segmental NF and mosaic patterns exist; experts evaluate distribution and other signs to categorize correctly. PubMed
What’s the first FDA-approved drug for NF1 PN in kids?
Selumetinib (KOSELUGO) in 2020. FDA Access Data
Is there an NF1 PN drug for adults, too?
Yes. Mirdametinib (GOMEKLI) was approved in Feb 2025 for adults and children with NF1-PN not amenable to complete resection. U.S. Food and Drug Administration
Bottom line for “NF6/CALM-only”?
It’s a skin-only, usually benign condition. Focus on sun care, reassurance, and laser if you want cosmetic lightening; watch children for any NF1 features over time. NCBI+1

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