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:
What is the pathophysiology of nevus comedonicus?
Nevus comedonicus appears to be a subset of epidermal nevus. Somatic mutation has been proposed as the etiology of both. Some cases of familial lesions indicate more complex etiopathogenesis. Nevus comedonicus has been linked to a somatic mutation in fibroblast growth factor-2 receptor (FGFR-2).
What is a comedo naevus?
It is also known as ‘comedone naevus’ and ‘ nevus comedonicus’. A comedo naevus is an unusual type of epidermal naevus, or birthmark (a pilosebaceous hamartoma ), in which there is a localised collection of dilated follicles filled with keratin. It is more accurately termed follicular keratotic naevus, as there are no true comedones.
Does nevus comedonicus cause hidradenitis suppurativa?
One case report has been seen in a patient with nevus comedonicus complicated with lichen striatus and linear morphea. A case has been reported in which hidradenitis suppurativa was seen possibly in association with nevus comedonicus. What is the Evidence?
What are the treatment options for nevus comedonicus (NVC)?
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.
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What causes nevus comedonicus?
Nevus comedonicus has been linked to a somatic mutation in fibroblast growth factor-2 receptor (FGFR-2). Several other diseases have been associated with this gene, including Apert's syndrome, chondrodysplasia, and craniosynostosis syndromes.
How do you treat nevus comedonicus?
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.
Is nevus comedonicus rare?
Nevus comedonicus syndrome is a rare syndrome with extracutaneous manifestations. Commonly involved sites for comedo nevus include face, neck, upper arm, chest and abdomen in a grouped, band-like lesions, or linear pattern along lines of Blaschko.
Can nevus comedonicus be removed?
Uncomplicated nevus comedonicus can be treated with topical keratolytics, diode, erbium laser, and ultrapulse CO2 laser. Surgical excision can be performed to ensure complete removal and nonrecurrence.
How many people have nevus comedonicus?
Nevus comedonicus is a rare problem with an estimated occurrence of 1 case in every 45,000–100,000 individuals [2, 6].
How do you get rid of sebaceous nevus?
For nevus sebaceous treatment, the time of resection is controversial, but most investigators suggest that surgical excision is the treatment of choice2. However, removing nevus sebaceous by surgical excision leaves linear scar. To minimize scar, there were several other treatment options include CO2 laser treatment.
Is nevus comedonicus hereditary?
Selected Hereditary Diseases Epidermal nevi vary according to their predominant component and include nevus sebaceus (sebaceous glands), nevus comedonicus (hair follicles) and verrucous nevus (keratinocytes).
How common is nevus Depigmentosus?
The term nevus depigmentosus, however, is a misnomer, because the lesion is hypopigmented but not depigmented. The reported prevalence of nevus depigmentosus varies from 0.4% to 3%.
How are comedones formed?
Comedones form when excess oil and dead skin cells block the oil-producing glands in the skin. This causes the affected pores to bulge outward, creating the bumps. Several factors can contribute to the development of comedonal acne.
What causes spreading blackheads?
Open comedones, or blackheads, develop near the pore opening. Comedones are especially common during the teenage years but they can happen at any age. Hormone changes can be a contributing factor. Oily skin care products and humidity can also make it worse.
What is epidermal nevus?
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 causes spreading blackheads?
Open comedones, or blackheads, develop near the pore opening. Comedones are especially common during the teenage years but they can happen at any age. Hormone changes can be a contributing factor. Oily skin care products and humidity can also make it worse.
How common is nevus Depigmentosus?
The term nevus depigmentosus, however, is a misnomer, because the lesion is hypopigmented but not depigmented. The reported prevalence of nevus depigmentosus varies from 0.4% to 3%.
Is nevus Comedonicus hereditary?
Selected Hereditary Diseases Epidermal nevi vary according to their predominant component and include nevus sebaceus (sebaceous glands), nevus comedonicus (hair follicles) and verrucous nevus (keratinocytes).
What is epidermal nevus?
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 is the differential diagnosis of nevus comedonicus?
The differential diagnosis of nevus comedonicus includes nevus sebaceous, acne neonatorum, and, in older patients, comedonal acne. Although the lesions usually occur as a sporadic finding, multiple or extensive lesions may be associated with abnormalities in other organ systems as part of the ‘nevus comedonicus type’ of epidermal nevus syndrome. 108,159 Management of nevus comedonicus is challenging. Topical retinoids, ammonium lactate lotion, or topical antibiotics may each be useful for some patients. Pore strips have been reported useful for removal of the keratin plugs. 160 However, most medical therapies are ineffective, and the definitive therapy for cosmetically significant lesions is surgical excision.
What is a Nevus unius lateris?
Nevus unius lateris: Extensive unilateral plaque with abrupt midline demarcation, usually on the trunk.
What is epithelial nevus?
Some authors use the term ‘epithelial nevus’ or ‘epidermal nevus’ as a group generic term to cover malformations of adnexal epithelium, as well as those involving the epidermis alone (see below). 1,2 The term ‘epidermal nevus’ is used here in a restricted sense and does not include organoid, sebaceous, eccrine, and pilar nevi. These are considered with the appendageal tumors in Chapter 33. An exception has been made for nevus comedonicus, an abnormality of the infundibulum of the hair follicle. It is considered here because its histological appearance suggests an abnormality of the epidermis, rather than of appendages. Furthermore, the report of the coexistence of nevus comedonicus and an epidermal nevus suggests that the two entities are closely related.3 The nevus comedonicus syndrome is regarded as a variant of the epidermal nevus syndrome; it has also been grouped with the organoid nevus (see p. 806 ).
What are the different types of nevi?
Epidermal nevi vary according to their predominant component and include nevus sebaceus (sebaceous glands), nevus comedonicus (hair follicles) and verrucous nevus (keratinocytes). Multiple other mucocutaneous findings may occur simultaneously with the epidermal nevus. Central nervous, ocular, and musculoskeletal systems are predominantly affected. However, a wide range of abnormalities may occur within each of these systems, and occasionally other organ systems may be involved. ENS occurs sporadically. ENS should be considered in any patient with extensive epidermal nevi or epidermal nevi associated with systemic abnormalities. The possibility that an epidermal nevus is a component of another syndrome, such as Proteus, CHILD (congenital hemidysplasia with ichthyosiform nevus and limb defects), or phakomatosis pigmentovascularis, should be considered. Patients suspected of having ENS should have a thorough physical evaluation with special attention to the musculoskeletal, neurologic, ocular, and cardiovascular systems. Management should be multidisciplinary, depending on the findings of the physical examination.
What is a NC?
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.
Is Nevus comedonicus inherited?
Nevus come donicus is a sporadic disorder and is not inherited. The pathogenesis is believed to involve somatic mosaicism.116,135 A mutation in the fibroblast growth factor receptor 2 ( FGFR2) gene detected in a nevus comedonicus suggests that this may represent a mosaic form of Apert syndrome. 138
Is nevus comedonicus a skeletal disorder?
In most cases there are no extracutaneous manifestations. Rarely, a nevus comedonicus is associated with central nervous system, skeletal , and ocular abnormalities.136 In these cases, unilateral cataract and skeletal abnormalities are found on the same side of the body as the nevus. 136 The nevus comedonicus syndrome is considered a variant or subtype of the epidermal nevus syndrome. 105,116,136
What is the treatment for nevus comedonicus?
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.
What is a nevus?
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.
What drugs are used to remove Nevus comedonicus?
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.
When does a lesion appear?
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.
Is Nevus comedonicus rare?
Nevus Comedonicus Syndrome (NCS) 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.
What are the clinical features of comedo naevus?
Lesions often grow more quickly at puberty. They can appear anywhere on the body but are most commonly found on the face, trunk, neck and upper extremities.
What is comedo naevus syndrome?
Comedo naevus syndrome is when comedo naevus is associated with other medical conditions. Like other epidermal naevi, comedo naevus is rarely associated with other abnormalities of the cell of origin, the embryonic ectoderm. These may include:
What causes a comedo naevus?
The exact cause of comedo naevus is unknown, but it is thought to be due to cutaneous mosaicism; that is, a line of cells with a genetic error. If this error occurs early in the development of the embryo, the cells may spread out to cause multiple comedo naevi.
What are the complications of comedo naevus?
Rarely, at puberty or later, a comedo naevus may develop inflammatory acne -like lesions within it. These can lead to cysts, recurrent bacterial infections, abscesses, and scarring. These should be treated with appropriate antibiotics or surgical drainage. Topical and oral treatment for acne may improve the appearance.
What is the best treatment for comedo naevus?
Some improvement of lesions may be seen with the use of topical tretinoin, salicylic acid or ammonium lactate lotion.
Are You Confident of The Diagnosis?
Treatment Options
- MEDICAL Topical 1. Topical retinoid cream (tretinoin 0.1%), 2. Topical retinoid cream (0.1%) combined with topical corticosteroid ointment (mometasone furoate) 3. Topical 12% amonium lactate lotion 4. Topical tacalcitol 5. Topical tazarotene 6. Topical calcipotriene Systemic 1. Systemic antibiotics 2. Intralesional steroids SURGICAL
Optimal Therapeutic Approach For This Disease
- The first step is to be certain of the diagnosis. No particular labarotory test is indicated, unless there is other involvement apparent or suspected based on clinical or other findings. Skin biopsy of the lesion should be done to confirm the diagnosis. Localized lesions may be treated on a cosmetic basis, unless there are complications such as secondary infection. Spontaneous regre…
Patient Management
- Prognosis is excellent barring associated conditions (see unusual scenarios section) and with adequate local management. One case report has been seen in a patient with nevus comedonicus complicated with lichen striatus and linear morphea. A case has been reported in which hidradenitis suppurativa was seen possibly in association with nevus comedonicus.
What Is The Evidence?
- Solomon, LM, Esterly, NB. “Epidermal and other congenital organic nevi”. Curr Probl Pediatr. vol. 6. 1975. pp. 3-56. (This is an extremely comprehensive review of epidermal nevi by one of the giants in the field.) Vidaurri-de La Cruz, H, Tamayo-Sanchez, L, Duran-McKinster, C. “Epidermal nevus syndromes: clinical findings in 35 patients”. Pediatr Dematol. vol. 21. 2004. pp. 432-439. (Of thirt…