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obsolete epidermal nevus

ICD-10 Codes

Related ICD-10:

Description

Obsolete Epidermal Nevus Description

Obsolete epidermal nevus, also known as keratinocytic nevus, is a rare skin condition characterized by the presence of thick, hystrix-like white or brownish hyperkeratosis [6]. This condition is often associated with multiple vascular anomalies and can be part of a larger syndrome.

Key Features:

  • Thick, hystrix-like white or brownish hyperkeratosis
  • Association with multiple vascular anomalies
  • Can be part of a larger syndrome

Synonyms: Obsolete epidermal nevus is also known as NEVUS, KERATINOCYTIC, NONEPIDERMOLYTIC [3].

Note: The term "obsolete" in this context refers to the fact that this condition may not be commonly recognized or classified under modern dermatological classifications.

Additional Characteristics

  • Obsolete epidermal nevus
  • keratinocytic nevus
  • NEVUS, KERATINOCYTIC, NONEPIDERMOLYTIC

Signs and Symptoms

Based on the provided context, it appears that Epidermal Nevus Syndrome (ENS) encompasses a wide variety of disorders with varying symptoms.

Rare Associations

Epidermal nevus syndromes have been associated with rare conditions such as strabismus, ptosis, microphthalmia, nystagmus, astigmatism, cataract, and bilateral tear duct obstruction [1]. These associations are relatively uncommon and may not be present in all cases of ENS.

Vascular Anomalies

Vascular anomalies, including aneurysm, arteriovenous malformation, vascular coarctation, patent ductus arteriosus, and other cardiovascular abnormalities, have been reported in association with epidermal nevus syndrome [2].

Cutaneous Features

The cutaneous features of ENS can vary greatly depending on the specific type of syndrome. Some common signs include:

  • Blaschko-linear hypopigmentations
  • Hairlessness
  • Mosaic arrangement of digital malformations

Additionally, dermatological examination may reveal hyperpigmented verrucous plaques arranged along lines of Blaschko [3].

Other Symptoms

Other symptoms associated with ENS include:

  • Dry eyes
  • Difficulty sweating due to thick layers of skin clogging sweat glands
  • Fragile skin that cracks easily and frequently

It's essential to note that the specific symptoms present, severity, and prognosis can vary greatly depending on the type of ENS and the presence and extent of associated extra-cutaneous symptoms [4].

References

Additional Symptoms

  • strabismus
  • microphthalmia
  • astigmatism
  • cataract
  • bilateral tear duct obstruction
  • aneurysm
  • arteriovenous malformation
  • vascular coarctation
  • patent ductus arteriosus
  • Blaschko-linear hypopigmentations
  • hairlessness
  • mosaic arrangement of digital malformations
  • hyperpigmented verrucous plaques arranged along lines of Blaschko
  • dry eyes
  • difficulty sweating due to thick layers of skin clogging sweat glands
  • fragile skin that cracks easily and frequently
  • nystagmus
  • ptosis

Diagnostic Tests

Based on the provided context, it appears that there are several diagnostic tests associated with epidermal nevi. Here are some of the relevant tests mentioned:

  • Dermatoscopy: This is a non-invasive imaging technology that can be used to examine the skin and diagnose epidermal nevi (Search Result 8).
  • Total body photography: This involves taking photographs of the entire body to identify any lesions or abnormalities, including epidermal nevi (Search Result 8).
  • Biopsy: A biopsy may be performed to confirm the diagnosis of an epidermal nevus, particularly if there are concerns about malignancy (Search Results 1 and 4).
  • Non-invasive imaging technologies: These include tests such as MRI scans that can be used to evaluate intracranial involvement in certain cases of epidermal nevi (Search Result 9).

It's worth noting that the specific diagnostic tests used may vary depending on the individual case

Additional Diagnostic Tests

  • Biopsy
  • Dermatoscopy
  • Total body photography
  • Non-invasive imaging technologies

Treatment

Treatment Options for Epidermal Nevus

Epidermal nevus, also known as epidermal nevus syndrome (ENS), is a rare genetic disorder characterized by the growth of benign tumors on the skin. While there are various treatment options available, drug treatment plays a crucial role in managing this condition.

  • Keratolytics: These medications increase the rate of skin shedding and can help reduce the size of the growths [4].
  • Topical steroids: Useful for reducing inflammation and itching associated with epidermal nevus [4].
  • Vitamin D analogues: Calcipotriol, a vitamin D analogue, may be tried in each form of this disease, particularly inflammatory linear verrucous epidermal nevus [5].

Other Treatment Options

In addition to drug treatment, other options for managing epidermal nevus include:

  • Surgical excision: In some cases, surgical removal of the affected skin area may be necessary.
  • Laser therapy: Erbium-YAG laser or other types of laser therapy may be used to treat inflammatory linear verrucous epidermal nevus [9].
  • Cryotherapy: This treatment involves freezing the affected skin area to remove the growths.

References

[1] TI Kim, et al. (2015). Management of epidermal nevus with trichloroethane, 5-fluorouracil, bleomycin, and cryotherapy. [2] Vitamin D analogues may work by inhibiting epidermal proliferation, promoting keratinocyte differentiation, and/or exerting immunosuppressive effects on ... [3] AG Zhou (2022). Topical sirolimus therapy for nevus sebaceus and epidermal nevus: A case series. J Am Acad Dermatol. 2022 Aug;87(2):407-409. doi: 10.1016/j.jaad.2021.08.029. [4] Prescription Medications: · Keratolytics: Keratolytics increase the rate of skin shedding and can help reduce the size of the growths. · Topical steroids: Useful ... [5] Apr 5, 2023 — The vitamin D analogue calcipotriol may be tried in each form of this disease, particularly inflammatory linear verrucous epidermal nevus. [6] Oct 3, 2017 — We present here a case of successful treatment of pruritus associated with an epidermal nevus with the use of topical Ketamine 10% – Amitriptyline 5% – ... [7] Learn about Epidermal Nevus Syndromes, including symptoms, causes, and treatments. If you or a loved one is affected by this condition, visit NORD to find. [8] These lesions tend to be very itchy. Treatment of the itching consists of steroid creams or antihistamines (like Benadryl®), but often these only help a little. [9] The inflammatory linear verrucous epidermal nevus (ILVEN) sometimes responds to Erbium-YAG laser or vitamin D analogues.

Recommended Medications

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Differential Diagnosis

Differential Diagnosis of Obsolete Epidermal Nevus

Obsolete epidermal nevus, also known as inflammatory linear epidermal naevus (ILEN), is a rare skin condition characterized by an inflammatory and scarring process affecting the epidermis. When considering the differential diagnosis for obsolete epidermal nevus, several conditions should be taken into account.

  • Linear Lichen Planus: This condition can be distinguished from ILEN by clinical and histopathological criteria. Linear lichen planus is characterized by a linear or band-like distribution of lesions, whereas ILEN typically presents with an inflammatory and scarring process affecting the epidermis.
    • [4] [7]

Additional Differential Diagnoses

  • Linear Lichen Planus

Additional Information

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