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. Most commonly it affects the face and neck area and, by exception, other anatomical regions, including genital area, palms, and soles. 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. Optimal Therapeutic Approach for this Disease, Unusual Clinical Scenarios to Consider in Patient Management. nevus pigmentosus: [ ne´vus ] (pl. If you have problems viewing PDF files, download the latest version of Adobe Reader, For language access assistance, contact the NCATS Public Information Officer, Genetic and Rare Diseases Information Center (GARD) - PO Box 8126, Gaithersburg, MD 20898-8126 - Toll-free: 1-888-205-2311, A rare, syndromic nevus characterized by the association of typically unilateral, closely arranged, linear, slightly elevated, multiple, nevus comedonicus lesions located usually on the face, neck, trunk or limbs (with or without a central, dark, firm, hyperkeratotic plug and secondary acneiform lesions) with extracutaneous ocular, skeletal, and/or, expand submenu for Find Diseases By Category, expand submenu for Patients, Families and Friends, expand submenu for Healthcare Professionals. We hope you’re enjoying the latest clinical news, full-length features, case studies, and more. (This is an extremely comprehensive review of epidermal nevi by one of the giants in the field. This section provides resources to help you learn about medical research and ways to get involved. Curr Probl Pediatr. Some lesions are also associated with deeper anomalies in the same locations in muscle and bone. 2004. pp. Localized lesions may be treated on a cosmetic basis, unless there are complications such as secondary infection. ), Sinha, A, Natarajan, S. “Linear morphea, nevus comedonicus, and lichen striatus in a 5-year-old girl”. vol. Lesions are characteristically arranged in bands or groups, and may be in a linear array along Blaschko’s lines. Nevus comedonicus is a type of hamartoma that arises from a developmental anomaly of the mesodermal part of the pilosebaceous gland. We remove all identifying information when posting a question to protect your privacy. Nevus comedonicus may be localized or have an extensive involvement, the latter showing a unilateral predominance with only a few cases presenting bilaterally. Get the latest research information from NIH: https://www.nih.gov/coronavirus (link is external). Have a question? Key words: microneedling; nevus comedonicus; nevus comedonicus treatment. Patients with any of these associations are sometimes referred to as having the “nevus comedonicus syndrome.” This syndrome should be considered in the context of epidermal nevus syndromes. Dermoscopy is useful in differentiating nevus comedonicus from comedones of acne, other rare epidermal nevi, such as sebaceous nevus, and hair follicle nevus. Nevus comedonicus is characterized clinically by groups of enlarged follicuiar openings with corneal plugs resembling comedones. 28. Lesions usually present at birth, though sporadic cases of development later in life have been reported. When associated lesions are cancer, such as basal cell cancers, close monitoring of the patient is necessary to identify and treat any such malignancies. 21. Prior lesions have included herpes zoster, lichen planus, vaccination, furunculosis, and ph… This agent acts by accelerating exfoliation of epithelium, resulting in expulsion of keratin plugs from comedonal lesions. (HPO) . Registration is free. Malignant degeneration in lesions of nevus comedonicus itself has not been reported. Nevus sebaceous (or nevus sebaceus, also known as an "Organoid nevus" and "Nevus sebaceous of Jadassohn" is a congenital, hairless plaque that typically occurs on the face or scalp. Nevus comedonicus appears to be a subset of epidermal nevus. Unilateral Facial Nevus Comedonicus A 12-year-old girl presented with asymptomatic skin lesions over the left side of the face, which were present since birth. No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC. Nevus comedonicus is rare, accounting for only 1% of epidermal nevi. “Nevus comedonicus. 1968. pp. [ 21] Inflammatory linear verrucous epidermal … 2011 Jan-Feb. pp. Lesions may worsen at puberty or during mensturation. Nevus comedonicus (NC) is an infrequent developmental anomaly manifesting as aggregated open comedones. all the symptoms listed. Who is at Risk for Developing this Disease? The first step is to be certain of the diagnosis. Surgical intervention may be required for larger lesions. Some lesions may appear verrucous, especially over prominences such as elbows or knees. There is no racial predisposition. A very few sporadic cases have been reported in later years, usually following irritation or trauma at the affected site, thus representing Koebner’s isometric response in patients with prior nevus comedonicus elsewhere, or Wolf’s isotopic response in patients without prior history of nevus comedonicus. “Hidradenitis suppurativa complicating naevus comedonicus: the possible influence of mechanical stress on the development of hidradenitis suppurativa”. Solomon, LM, Esterly, NB. A case with palmar involvement involvement and review of literature”. While it can appear anywhere on the head, it most often occurs on the scalp… vol. September 1, 2020, NIH-Supported Research Survey to Examine Impact of COVID-19 on Rare Diseases Community The incidence of EN has been reported as 1 to 3 It’s typically firm, round, smooth, and may have hair coming out of it. This table lists symptoms that people with this disease may have. About half of the cases are apparent at birth, with the remainder arising before puberty, especially in the first ten years of life. The condition develops within the first decade of life in most patients. 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. Skin biopsy of the lesion should be done to confirm the diagnosis. Nevus comedonicus presents as grouped, dilated follicular openings with dark keratin plugs. Nevus comedonicus is typically found on the face, trunk, neck, and upper extremities. A registry supports research by collecting of information about patients that share something in common, such as being diagnosed with Nevus comedonicus syndrome. Nevus comedonicus has been associated with a number of other entities. 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.1,2It can occur congenitally or develop later in life, most commonly diagnosed at the age of approximately 10 years. Questions sent to GARD may be posted here if the information could be helpful to others. The type of data collected can vary from registry to registry and is based on the goals and purpose of that registry. ), (Of thirty-five patients with the epidermal nevus syndrome, three [8%] were found to have a nevus comedonicus. More severe lesions with cystic disease may require systemic antibiotics (tetracycline 500mg orally twice daily or doxycycline 100mg orally twice daily) and intralesional steroids (IL kenalog 5-10mg/cc). 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). Rare developmental abnormality of the skin in which grouped hair follicles filled with horny plugs constitute a prominent feature. vol. May 21, 2020. A nevus sebaceous (NEE vuhs sih BAY shus) is a type of birthmark that usually appears on the scalp. There may be epithelial budding out from the walls as well. Topical tazarotene 0.05% cream has been combined with topical calcipotriene 0.005% cream with success. December 1, 2020, Rare Diseases Are Not Rare - Gallery of Creative Work Raises Awareness of Rare Diseases Disease - Nevus comedonicus ))) Map to ... and abscesses. Secondary inflammation is often present, and may lead to cicatrix formation. This information comes from a database called the Human Phenotype Ontology 432-439. 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