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BACKGROUND: Nevus comedonicus (NC) is a rare cutaneous disorder thought to be caused by hamartomatous pilosebaceous tissue proliferation that was first described in 1895. Clinically NC appears as a group of elevated follicular openings often linearly arrayed, giving the appearance of comedones. NC usually manifests at birth but can also present later during adolescence and rarely in adulthood.
METHODS: A 21-year-old medically healthy single male presented with right-sided chest black papules in comedo distribution with areas of superficial ulcerations and suppurations in periareolar distribution since the age of 16. Histopathological examination showed dilated follicular ostia filled with keratin plug, devoid of the hair shaft, and lined by stratified squamous epithelium with compact hyperkeratosis, focal parakeratosis, and patchy atrophy and acanthosis.
UNASSIGNED: Nevus comedonicus often responds effectively to conservative treatment, however some cases need surgery intervention. The patient failed conservative medical and topical treatment, and he was treated by surgical-wide local excision and primary closure of the affected skin with free nipple grafting.
CONCLUSIONS: Nevus comedonicus (NC) is a rare cutaneous pathology secondary to pilosebaceous apparatus developmental defect that usually manifests at birth and can affect any area of skin; they typically manifest as black papules in comedo distribution. They can present as an isolated cutaneous pathology or as a component of nevus comedonicus syndrome. Different therapeutic approaches were described, including topical retinoids, keratolytic agents, oral retinoids, antibiotic therapy, manual extraction of comedos, dermabrasion, and surgical resection of the lesion.

Epidermal nevus syndrome (ENS) comprises a heterogeneous group of neurocutaneous syndromes associated with the presence of epidermal nevi and variable extracutaneous manifestations. Postzygotic activating HRAS pathogenic variants were previously identified in nevus sebaceous (NS), keratinocytic epidermal nevus (KEN), and different ENS, including Schimmelpenning-Feuerstein-Mims and cutaneous-skeletal-hypophosphatasia syndrome (CSHS). Skeletal involvement in HRAS-related ENS ranges from localized bone dysplasia in association with KEN to fractures and limb deformities in CSHS. We describe the first association of HRAS-related ENS and auricular atresia, thereby expanding the disease spectrum with first branchial arch defects if affected by the mosaic variant. In addition, this report illustrates the first concurrent presence of verrucous EN, NS, and nevus comedonicus (NC), indicating the possibility of mosaic HRAS variation as an underlying cause of NC. Overall, this report extends the pleiotropy of conditions associated with mosaic pathogenic variants in HRAS affecting ectodermal and mesodermal progenitor cells.

Nevus comedonicus (NC) is a rare developmental anomaly of the folliculosebaceous apparatus, which appears as numerous dilated papules containing firm, darkly pigmented, horny plugs. It appears shortly after birth and mostly before the age of 10; however, late-onset cases have been reported. There is no gender or racial predilection. Moreover, NC can be a component of nevus comedonicus syndrome, a neurocutaneous disorder with skeletal, ocular, and central nervous system abnormalities. EHK properties in NC are not a common finding and are rarely seen in association with each other. This paper reports a healthy, 27-year-old young woman who has been developing numbers of asymptomatic unilateral linear skin lesions on her chest, waist, right thigh, and popliteal fossa in a unilateral linear pattern over ten years. Skin biopsy revealed dilated follicular ostia with orthokeratotic hyperkeratosis, columns of parakeratosis, cornoid flagellation, epidermolytic hyperkeratosis, and mild acanthosis on its wall.

Nevus comedonicus (NC) is a rare hamartoma of the pilosebaceous unit origin. The association with extracutaneous abnormalities defines NC syndrome (NCS). Fewer than 50 cases of NCS have been reported in the English literature. A 31-year-old woman presented with grouped and linear comedonal papules present from birth and located on the left buttock along Blaschko\'s lines. She had a history of pediatric mood disorder combined with attention-deficit hyperactivity disorder (ADHD) from 5 years of age and was recently diagnosed with sinus bradycardia. Her skin lesion was surgically removed and microscopic findings revealed the aggregation of dilated follicular infundibula filled with prominent laminated keratin plugs, a characteristic finding of NC. This is the first report presenting NCS associated with mood disorder and ADHD. Psychiatric symptoms may represent systemic manifestation of NCS.

We present the case of a 92-year-old woman, with bullous pemphigoid (BP) and a concomitant nevus comedonicus (NC) presenting as an asymptomatic, linear lesion of the entire lower left limb, formed by multiple comedones. Dermoscopy of the NC and histopathology confirmed the clinical and dermoscopic suspect of giant congenital nevus comedonicus. The two entities presented no overlap. In this article, we speculate that a mutation of the NEK9 gene, associated with NC, probably altering the normal follicular differentiation in NC lesions, may hypothetically also influence the expression of BPAG2 in NC. This might possibly influence a protective role of NC lesions towards BP. Undoubtedly, genetic studies would be needed to confirm or reject the proposed hypothesis.

Nevus comedonicus (NC) syndrome is a condition first identified in 1978. The cause of NC syndrome has been recently proven to be a gain-of-function, mosaic postzygotic mutation of the NEK9 gene. A systematic review of the literature retrieved 43 well-established cases of NC syndrome reported so far. Three morphological variants of NC in NC syndrome emerged: (a) the more common, predominantly comedonal type; (b) \"Selhorst type\"; and (c) \"atrophoderma vermiculatum\" type. NC syndrome is mainly associated with ocular, skeletal, and neural abnormalities, most typically ipsilateral congenital cataract and malformations of fingers and toes.

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Nevus comedonicus and hidradenitis suppurativa (HS) are disorders of the pilosebaceous unit sharing a similar pathogenesis of follicular occlusion. To our knowledge, less than 10 cases of HS-like lesions complicating nevus comedonicus have been reported. We describe a six-year-old female child with congenital linear nevus comedonicus in the left axilla and groin, complicated by recurrent HS-like lesions in the two years prior to presenting to our clinic. After a meticulous review of the literature, we propose a novel term, nevus comedonicus suppurativa, for this clinical entity.

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