Nevus Comedonicus Syndrome (NCS) (2024)

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Nevus Comedonicus Syndrome (NCS) (1)By Dr. Liji Thomas, MDReviewed by Afsaneh Khetrapal, BSc

Nevus comedonicus syndrome is one of the epidermal nevus syndromes, but is extremely rare. There is no gender or racial difference in the prevalence of this condition. The prevalence ranges from 1 in 45,000 - 100 000. Nevus comedonicus syndrome (NCS) was described under this name in 1978. It consists of the following findings:

  • Nevus comedonicus
  • Ipsilateral cataract
  • Skeletal defects
  • Brain defects
  • Trichilemmal cysts

Spreading Blackheads! Dilated Pore of Winer

Clinical Manifestations

Nevus Comedonicus

The nevus was described in 1895 under the term “comedo nevus.” The typical lesion is an overgrowth of clumps of papules, usually with a central black firm center. These are morphologically blackheads, later evolving into inflammatory acne. For this reason, later lesions may appear nodular, pustular or abscessed. Significant scar formation is a natural post-inflammatory sequel of these lesions.

The lesions are formed by obstruction of the hair follicle canal, leading to the accumulation of black keratinized material within dilated follicular openings arranged close together. This material is not easily taken out. In most cases the lesions follow a linear or band-like pattern, but occasionally Blaschko lines are followed, or no pattern is recognizable.

While the condition presents any time between the time of birth and middle age, the most common time of its appearance is at birth (in half the cases) or at least before the age of 10 years. The most common site is in the face and neck. The lesion is usually free of any symptoms. It has various patterns of distribution. In a very few cases, multiple follicular basal cell carcinomas have been reported.

Extracutaneous Manifestations

Non-cutaneous anomalies are reported including:

  • Skeletal: absence of fifth digit, rudimentary toe, scoliosis, polysyndactyly, bone hypertrophy, bone cyst, and vitamin D-resistant rickets are some of the bone defects that have been reported so far.
  • Dental: oligodontia has been noted in one case.
  • Glandular: eccrine spiradenoma, follicular basal cell nevus, and hidradenoma are sometimes seen.
  • Central nervous system: microcephaly, brain malformations including agenesis of the corpus callosum, Sturge-Weber syndrome.
  • Ocular: cataract, usually ipsilateral, but rarely bilateral.
  • Trichilemmal cysts: these are cysts that form from hair follicles usually on the scalp, and are filled with keratin.

Diagnosis and Treatment

Conservative treatment of nevus comedonicus is given first, especially if there is inflammation or irritation. Treatment is with emollients, topical steroids and keratolytic lotions. Retinoids alone or in combination with topical steroids have been tried with mixed success. Newer drugs include FGFR inhibitors and IL-1α inhibitors, as well as tazarotene and calcipotriene.

Nevus comedonicus may require removal for cosmetic reasons or if severe inflammation sets in. surgical excision, shave excision, comedo extraction, or dermabrasion are some of the modalities adopted. Large lesions may require proper drainage, transplantation to fill the defect, or other measures for better cosmetic outcomes. Laser treatment using Er-YAG or pulsed carbon dioxide are also being tested for this indication.

References

Further Reading

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Last Updated: Feb 27, 2019

Nevus Comedonicus Syndrome (NCS) (3)

Written by

Dr. Liji Thomas

Dr. Liji Thomas is an OB-GYN, who graduated from the Government Medical College, University of Calicut, Kerala, in 2001. Liji practiced as a full-time consultant in obstetrics/gynecology in a private hospital for a few years following her graduation. She has counseled hundreds of patients facing issues from pregnancy-related problems and infertility, and has been in charge of over 2,000 deliveries, striving always to achieve a normal delivery rather than operative.

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Nevus Comedonicus Syndrome (NCS) (4)

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Nevus Comedonicus Syndrome (NCS) (2024)

FAQs

Nevus Comedonicus Syndrome (NCS)? ›

Nevus comedonicus (NC) is characterized by a localized collection of dilated follicles containing keratin. Some authors have split this entity into NC consisting of comedo-like dilated follicular orifices and “acne nevus” which produces sebum and may be complicated by inflammation and scarring.

What are the complications of nevus comedonicus? ›

Persons with inflammatory nevus comedonicus can develop cysts, recurrent bacterial infections, fistulae, and abscesses, and these may subsequently heal with scarring. Treat these lesions with appropriate antibiotics or surgical drainage. Infections may be recurrent.

How rare is nevus comedonicus? ›

Nevus comedonicus syndrome is one of the epidermal nevus syndromes, but is extremely rare. There is no gender or racial difference in the prevalence of this condition. The prevalence ranges from 1 in 45,000 - 100 000.

What is inside nevus comedonicus? ›

Nevus comedonicus (NC), first described by Kofmann in 1895, is a rare condition considered to be a type of epidermal nevus. Nevus comedonicus presents as grouped, dilated follicular openings with dark keratin plugs.

What is a rare blackhead condition? ›

Rare conditions

Nevus comedonicus or comedo nevus is a benign hamartoma (birthmark) of the pilosebaceous unit around the oil-producing gland in the skin. It has widened open hair follicles with dark keratin plugs that resemble comedones, but they are not actually comedones.

How do you treat nevus Comedonicus syndrome? ›

As nevus comedonicus is an asymptomatic benign condition, no treatment is required. Surgical excision of small lesions can be curative and should be considered in consultation with a dermatologic/plastic surgeon. Incomplete excision may result in recurrence of the lesion. Laser surgery has been tried.

What are the side effects of nevus sebaceous syndrome? ›

Nevus sebaceous syndrome can result in eye tumours and the skull may be asymmetrical. Characteristic associated neurological features include: Developmental delay. Epileptic seizures, especially infantile spasms.

Is nevus a genetic disorder? ›

With basal cell nevus syndrome, the first mutation is inherited from either the mother or the father. This happens in 70% to 80% of cases. In 20% to 30% of cases, the first mutation is not inherited. It arises for the first time (de novo) in the affected person.

Does a nevus go away? ›

Congenital melanocytic nevi do not go away with time. Some congenital melanocytic nevi may get lighter in color over the first few years of life.

What is the prognosis of nevus? ›

The prognosis associated with any single melanocytic nevus is favorable because these lesions are benign neoplasms with no potential for malignant behavior, unless evolution of melanoma occurs.

What is a birthmark that looks like a blackhead? ›

A comedo naevus (comedo nevus), also known as naevus comedonicus, is a rare, benign, cutaneous anomaly consisting of grouped, dilated follicular openings containing soft, dark keratin that resemble comedones.

What does nevus sebaceous look like? ›

A nevus sebaceous will appear as a yellow-orange patch of skin that is hairless and smooth. It's circ*mscribed, meaning that it has well-defined borders, and is likely to appear either oval or linear in shape. Typically, there will be just one patch of skin affected, and it may look like a plaque.

What is epidermal nevus syndrome? ›

Epidermal nevus syndromes (ENSs) are a group of rare complex disorders characterized by the presence of skin lesions known as epidermal nevi associated with additional extra-cutaneous abnormalities, most often affecting the brain, eye and skeletal systems.

What are the tiny worms with black heads? ›

During early development stages, budworm larvae are creme-colored with a distinct black head. Their body color changes to green as they develop and the head capsule is brown during the last development stage (Fig.

When I popped a pimple, a rock came out.? ›

This phenomenon is called a "pore of Winer," and it's an actual thing. It's just a fancy term for a very big blackhead or whitehead. So now not only do we have to fear those nasty things, but these giant, raised rock pimples can cause a lot of trouble. It certainly did for this patient.

When I squeeze my nose, pus comes out.? ›

What Is the White Stuff That Comes Out When You Squeeze Your Nose Pores? The white stuff that comes out of your pores like thin strings when you squeeze your nose is called a sebaceous filament. It's mostly made up of sebum (oil that your skin produces) and dead skin cells.

What are the complications of nevus? ›

Large congenital melanocytic nevi are associated with an increased risk for developing cutaneous melanoma, leptomeningeal melanoma, neurocutaneous melanocytosis, malformations of the brain, and, rarely, rhabdomyosarcoma and liposarcoma.

What are the complications of birthmark removal? ›

What to expect after each treatment?
  • There may be redness or discoloration, swelling, bruising, or irritation for 24 hours after laser therapy to remove a birthmark.
  • Keep the area clean and avoid the sun.
  • You may experience peeling around the treated area.
  • The area should be healed within a week.
May 3, 2021

What are the complications of nevus of the eye? ›

However, sometimes nevi under the center of the retina (the macula) can cause blurred vision. When a nevus causes degeneration or dysfunction of the overlying RPE, fluid may accumulate under the retina or abnormal blood vessels (choroidal neovascularization) may develop and bleed or leak fluid.

What are the complications of acne keloidalis nuchae? ›

Keloidlike plaques, scarring alopecia, chronic draining sinuses, and bacterial infection are complications of acne keloidalis nuchae (AKN).

References

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