Nevus comedonicus presents as grouped, dilated follicular openings with dark keratin plugs. 28. A nevus sebaceous is a rare type of birthmark that can be found on the face, neck, forehead, or scalp. Nevus comedonicus (comedo nevus) is a rare hamartoma of the pilosebaceous unit. Optimal Therapeutic Approach for this Disease, Unusual Clinical Scenarios to Consider in Patient Management. It starts as a flat pink or orange plaque (slightly raised area). Arch Dermatol. Register for free and gain unlimited access to: - Clinical Updates, with personalized daily picks for you At birth, the lesions were limited to the mandibular region only but they increased in number with advancing age to involve the whole left side of the face. Lesions usually present at birth, though sporadic cases of development later in life have been reported. Five of these patients were found to have nevus comedonicus. If you wish to read unlimited content, please log in or register below. Only about 200 cases have been reported to date. This section provides resources to help you learn about medical research and ways to get involved. Copyright © 2017, 2013 Decision Support in Medicine, LLC. Already have an account? Nevus comedonicus is typically found on the face, trunk, neck, and upper extremities. The in-depth resources contain medical and scientific language that may be hard to understand. Nevus comedonicus syndrome has ocular, skeletal, and central nervous system anomalies. About half of the cases are apparent at birth, with the remainder arising before puberty, especially in the first ten years of life. We want to hear from you. (This is a case report of a palmar case, with an excellent review of the literature before 1968. The National Registry for Ichthyosis & Related Skin Disorders, Rare Diseases Are Not Rare - Gallery of Creative Work Raises Awareness of Rare Diseases, NIH-Supported Research Survey to Examine Impact of COVID-19 on Rare Diseases Community, NCATS Translational Approach Addresses COVID-19. 220. (Courtesy of Bryan Anderson, MD). On histology, there is an aggregation of dilated follicular infundibula with orthokeratotic plugging, absent or very rudimentary sebaceous elements, and the lack of normal hair follicles. ), (Of thirty-five patients with the epidermal nevus syndrome, three [8%] were found to have a nevus comedonicus. Dermoscopy is useful in differentiating nevus comedonicus from comedones of acne, other rare epidermal nevi, such as sebaceous nevus, and hair follicle nevus. Curr Probl Pediatr. Topical retinoid cream (tretinoin 0.1%) has been reported to improve lesions. A registry supports research by collecting of information about patients that share something in common, such as being diagnosed with Nevus comedonicus syndrome. Less common sites of involvement include scalp, and male and female genital mucus membranes. We remove all identifying information when posting a question to protect your privacy. Nevus comedonicus is characterized clinically by groups of enlarged follicuiar openings with corneal plugs resembling comedones. 1968. pp. Epidermal nevus, Nevus comedonicus, Nevus sebaceus INTRODUCTION Epidermal nevi (EN) are hamartomatous lesions derived from epidermal components originating from pluripotent cell mutations. (A case report noting overlapping disorders in conjuction with nevus comedonicus), Dufour, DN, Bryld, LE, Jamec, GB. This agent acts by accelerating exfoliation of epithelium, resulting in expulsion of keratin plugs from comedonal lesions. The nevus is cut out, and the adjacent skin stitched together leaving a small scar. Somatic mutation has been proposed as the etiology of both. Nevus comedonicus (NC) is a rare type of epidermal nevus that clinically presents as grouped dilated follicular openings with keratotic plugs, an appearance similar to comedones. The incidence of EN has been reported as 1 to 3 Nevus comedonicus is a rare, benign hamartoma characterized by closely arranged, honeycomb-like dilated follicular openings with black or brown keratinous plugs typically affecting the face, neck, upper arms, chest, or abdomen.1,2 It can occur congenitally or develop later in life, most commonly diagnosed at the age of approximately 10 years. ), Vidaurri-de La Cruz, H, Tamayo-Sanchez, L, Duran-McKinster, C. “Epidermal nevus syndromes: clinical findings in 35 patients”. The walls of the follicles are lined by several layers of keratinocytes, and are atrophic with sparse stratum granulosum. Another view is that nevus comedonicus is an epidermal nevus involving hair follicles or an appendageal nevus of sweat ducts. Nevus comedonicus is occasionally complicated with other conditions including cataracts, skeletal defects, central nervous system abnormalities or other e … Most part of the cases are sporadic and can appear during puberty or be present since birth, but may be also associated with systemic disorders. Dermatology. Contact a GARD Information Specialist. This information comes from a database called the Human Phenotype Ontology All rights reserved. Manual comedone extraction may help speed up the process. A case with palmar involvement involvement and review of literature”. Prior lesions have included herpes zoster, lichen planus, vaccination, furunculosis, and ph… Nevus comedonicus may be localized or have an extensive involvement, the latter showing a unilateral predominance with only a few cases presenting bilaterally. The HPO collects information on symptoms that have been described in medical resources. The first has only comedo-like eruptions. You can help advance 432-439. Nevus comedonicus (NC) is a benign hamartoma characterised by the occurrence of dilated comedo-like openings, with black or brown keratin plugs, typically localised on the face, neck, upper arms, chest or abdomen. - And More, (This is an extremely comprehensive review of epidermal nevi by one of the giants in the field. Please login or register first to view this content. Such nevi are present at birth, affecting males and females of all races equally. Lesions usually appear on the face, neck, upper arms, chest, or abdomen as one or more groups of closely situated markedly dilated follicular ostia, each filled with a dark keratin plug. The condition is named for an overgrowth of sebaceous glands in the area of the nevus. [1] ), (A case report noting overlapping disorders in conjuction with nevus comedonicus), (An infant developed hidradenitis suppurativa in the inguinal region associated with nevus comedonicus). A variable success rate with various antibiotic treatments have been reported. (An infant developed hidradenitis suppurativa in the inguinal region associated with nevus comedonicus). There may be epithelial budding out from the walls as well. Skin near lesions may appear hypopigmented. Nevus comedonicus (NC) is a rare hamartomatous birthmark (prevalence, 1 in 45,000). - Evidence-Based Guidance Differential diagnosis of nevus comedonicus includes other epidermal nevi, including especially nevus sebaceous of Jadassohn (typically located on the scalp, with three phases of evolution dependent on stage of differentiation of sebaceous gland), infantile acne, familial dyskeratotic comedones (widespread symmetrically distributed comedone-like hyperkeratotic papules, generally located on trunk, face, and arms), Favre-Racouchot disease (open and closed comedones located on actinically damaged skin), chloracne (aceniform eruption secondary to chemical exposure), Darier’s disease (skin-colored or yellow-brown papules with a greasy verrucous apperance, located in seborrheic regions and associated with nail and mucus membrane changes), and acne conglobata with extensive comedone formation (severe form of acne often resulting in disfigurement). In the first group, lesions are only of cosmetic concern, whereas in the second, scarring and suppuration are significant problems. (Of thirty-five patients with the epidermal nevus syndrome, three [8%] were found to have a nevus comedonicus. These resources provide more information about this condition or associated symptoms. Skin biopsy of the lesion should be done to confirm the diagnosis. Nevus comedonicus may vary markedly in severity, promoting some authors to divide patients into two groups. Topical tacalcitol has also been reported to be heplful. The rate of growth is static to slow. A rare, syndromic nevus characterized by the association of typically … Do you have more information about symptoms of this disease? For most diseases, symptoms will vary from person to person. An epidermoid cyst (Epidermal Inclusion cyst, Infundibular cyst), is a benign growth commonly found in the skin and typically appears on the face, neck or trunk, but can occur anywhere on the body. Nevus comedonicus is an uncommon skin abnormality characterized by an aggregation of dilated follicular orifices filled with keratinous material. Copyright © 2020 Haymarket Media, Inc. All Rights Reserved (This is an extremely comprehensive review of epidermal nevi by one of the giants in the field. Sebaceous nevi can give rise to sebaceous … We want to hear from you. While it can appear anywhere on the head, it most often occurs on the scalp… Nevus comedonicus (NC) or comedo nevus refers to closely arranged, grouped, often linear, slightly elevated papules that have at their center keratinous plugs. Secondary inflammation is often present, and may lead to cicatrix formation. While it is almost impossible to remove every cell of a large nevus, the goal is to remove as many cells as possible while at the same time preserving function and minimizing scarring. Half of … While NC is considered an epidermal nevus derived from the hair follicle, it may also represent a hamartoma arising from the pilosebaceous unit. It may also appear on the face but this is less common. Nevus comedonicus (NC) is a rare type of epidermal nevus with predilection for the face and neck area. Solomon, LM, Esterly, NB. 111-6. NORD RareLaunch® Workshops Have a question? Registration is free. Surgical intervention may be required for larger lesions. The second has, in addition, large cysts and scarring from recurrent abscesses and fistula formation. Sometimes, multiple linear lesions that terminate at the midlne may be seen. Reported asssociated entities include basal cell carcinomas and basal cell carcinoma syndromes, Sturge-Weber syndrome, vascular nevi, cataracts, interpapillary elastoma, absent finger and toes, pilar cysts, ecrine tumors including hidroadenoma, spiradenoma and syringocystadeoma papillariferum, hidradenoma papillariferum, Alagille’s syndrome, ichthyosis hysrtix, Becker’s nevus, deep pigmented hairs, skeletal defects, scoliosis, neurological defects, and spine and central nervous system anomalies. Another name used is “sebacous cyst” but this is actually an antiquated misnomer, and is not a term used by dermatologists. ), (This is a case report of a palmar case, with an excellent review of the literature before 1968. vol. Males and females appear to be affected equally. Nevus comedonicus has been linked to a somatic mutation in fibroblast growth factor-2 receptor (FGFR-2). 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