Inflammatory linear verrucous epidermal nevus: Difference between revisions

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Inflammatory linear verrucous epidermal nevus
Inflammatory linear verrucous epidermal nevus
Synonyms	ILVEN
Pronounce	N/A
Specialty	Dermatology
Symptoms	Pruritus, verrucous papules
Complications	N/A
Onset	Childhood
Duration	Chronic
Types	N/A
Causes	Genetic mutation
Risks	Family history
Diagnosis	Clinical diagnosis, skin biopsy
Differential diagnosis	Psoriasis, linear epidermal nevus
Prevention	N/A
Treatment	Topical corticosteroids, retinoids, laser therapy
Medication	N/A
Prognosis	N/A
Frequency	Rare
Deaths	N/A

Alternate names[edit]

ILVEN; Linear verrucose epidermal nevus; Verrucous epidermal nevus; Verrucous nevus; Inflammatory linear verrucous epidermal naevus; Inflammatory linear verrucous epidermal nevus

Definition[edit]

Inflammatory linear verrucous epidermal nevus (ILVEN) is a type of skin overgrowth, called epidermal nevus.

Genetics[edit]

ILVEN is caused by a genetic change that occurs after conception (somatic mutation).

Signs and symptoms[edit]

It is characterized by skin colored, brown, or reddish, wart-like papules (nevi).
The nevi join to form patches or plaques that often follow a pattern on the skin known as the "lines of Blaschko".
The affected areas of the skin may be red, itchy, and inflamed.
ILVEN typically presents from birth to early childhood and often is limited to one side of the lower half of the body.
It affects females more often than males.
It usually occurs alone; however, rarely ILVEN can be associated with other symptoms as part of an epidermal nevus syndrome.
Rarely, ILVEN can become cancerous (for example, basal cell carcinoma or squamous cell carcinoma).

Diagnosis[edit]

A diagnosis of ILVEN syndrome should be considered in patients with extensive verrucous, pruritic epidermal nevi, and/or systemic abnormalities.
A thorough mucocutaneous, neurologic, ophthalmic, and orthopedic examination is necessary with specific investigations depending on the involved system.
A regular follow-up is necessary due to the risk of malignant transformation of the nevi and development of systemic manifestations.<ref>Kumar, C. A., Yeluri, G., & Raghav, N. (2012). Inflammatory linear verrucous epidermal nevus syndrome with its polymorphic presentation - A rare case report. Contemporary clinical dentistry, 3(1), 119–122. https://doi.org/10.4103/0976-237X.94562</ref>.

Treatment[edit]

There is no one treatment or cure for ILVEN. Products used to soften the skin (emollients), moisturizers, and topical steroids may be utilized to relieve symptoms of dryness and itching.
Recently, several patients have been treated with laser therapy with good results.

Diseases of the skin and appendages by morphology

Growths

Epidermal	* Wart Callus Seborrheic keratosis Acrochordon Molluscum contagiosum Actinic keratosis Squamous-cell carcinoma Basal-cell carcinoma Merkel-cell carcinoma Nevus sebaceous Trichoepithelioma
Pigmented	* Freckles Lentigo Melasma Nevus Melanoma Postinflammatory hyperpigmentation
Dermal and subcutaneous	* Epidermal inclusion cyst Hemangioma Dermatofibroma (benign fibrous histiocytoma) Keloid Lipoma Neurofibroma Xanthoma Kaposi's sarcoma Infantile digital fibromatosis Granular cell tumor Leiomyoma Lymphangioma circumscriptum Myxoid cyst

Rashes

With
epidermal
involvement

Eczematous	* Contact dermatitis Atopic dermatitis Seborrheic dermatitis Stasis dermatitis Lichen simplex chronicus Darier's disease Glucagonoma syndrome Langerhans cell histiocytosis Lichen sclerosus Pemphigus foliaceus Wiskott–Aldrich syndrome Zinc deficiency
Scaling	* Psoriasis Tinea (Corporis Cruris Pedis Manuum Faciei) Pityriasis rosea Secondary syphilis Mycosis fungoides Systemic lupus erythematosus Pityriasis rubra pilaris Parapsoriasis Ichthyosis
Blistering	* Herpes simplex Herpes zoster Varicella Bullous impetigo Acute contact dermatitis Pemphigus vulgaris Bullous pemphigoid Dermatitis herpetiformis Porphyria cutanea tarda Epidermolysis bullosa simplex
Papular	* Scabies Insect bite reactions Lichen planus Miliaria Keratosis pilaris Lichen spinulosus Transient acantholytic dermatosis Lichen nitidus Pityriasis lichenoides et varioliformis acuta
Pustular	* Acne vulgaris Rosacea Folliculitis Impetigo Candidiasis Gonococcemia Dermatophyte Coccidioidomycosis Subcorneal pustular dermatosis

Without
epidermal
involvement

Red

Blanchable
Erythema

Generalized	* Drug eruptions Viral exanthems Toxic erythema Systemic lupus erythematosus
Localized	* Cellulitis Abscess Boil Erythema nodosum Carcinoid syndrome Fixed drug eruption
Specialized	* Urticaria Erythema (Multiforme Migrans Gyratum repens Annulare centrifugum Ab igne)

Nonblanchable
Purpura

Macular	* Thrombocytopenic purpura Actinic/solar purpura
Papular	* Disseminated intravascular coagulation Vasculitis

Indurated

* Scleroderma/morphea

Miscellaneous
disorders

Ulcers	*
Hair	* Telogen effluvium Androgenic alopecia Alopecia areata Systemic lupus erythematosus Tinea capitis Loose anagen syndrome Lichen planopilaris Folliculitis decalvans Acne keloidalis nuchae
Nail	* Onychomycosis Psoriasis Paronychia Ingrown nail
Mucous membrane	* Aphthous stomatitis Oral candidiasis Lichen planus Leukoplakia Pemphigus vulgaris Mucous membrane pemphigoid Cicatricial pemphigoid Herpesvirus Coxsackievirus Syphilis Systemic histoplasmosis Squamous-cell carcinoma

Skin cancer of the epidermis

Tumor

Carcinoma

BCC	* Forms Aberrant Cicatricial Cystic Fibroepithelioma of Pinkus Infiltrative Micronodular Nodular Pigmented Polypoid Pore-like Rodent ulcer Superficial Nevoid basal-cell carcinoma syndrome
SCC	* Forms Adenoid Basaloid Clear-cell Signet-ring-cell Spindle-cell Marjolin's ulcer Bowen's disease Bowenoid papulosis Erythroplasia of Queyrat Actinic keratosis
Adenocarcinoma	* Aggressive digital papillary adenocarcinoma Extramammary Paget's disease
Ungrouped	* Merkel-cell carcinoma Microcystic adnexal carcinoma Mucinous carcinoma Primary cutaneous adenoid cystic carcinoma Verrucous carcinoma Malignant mixed tumor

Benign
tumors

Acanthoma	* Forms Large-cell Fissuring Clear-cell Epidermolytic Melanoacanthoma Pilar sheath acanthoma Seboacanthoma Seborrheic keratosis Warty dyskeratoma
Keratoacanthoma	* Generalized eruptive Keratoacanthoma centrifugum marginatum Multiple Solitary
Wart	* Verruca vulgaris Verruca plana Plantar wart Periungual wart

Other

Epidermal nevus	* Syndromes Epidermal nevus syndrome Schimmelpenning syndrome Nevus comedonicus syndrome Nevus comedonicus Inflammatory linear verrucous epidermal nevus Linear verrucous epidermal nevus Pigmented hairy epidermal nevus syndrome Systematized epidermal nevus Phakomatosis pigmentokeratotica
Other nevus	* Nevus unius lateris Patch blue nevus Unilateral palmoplantar verrucous nevus Zosteriform speckled lentiginous nevus
Ungrouped	* Cutaneous horn

NIH genetic and rare disease info[edit]

NIH info on Inflammatory linear verrucous epidermal nevus

Inflammatory linear verrucous epidermal nevus is a rare disease.

Rare and genetic diseases

Rare diseases - Inflammatory linear verrucous epidermal nevus

A-Z list of rare diseases	* 0-9 \| A - B - C - D - E - F - G - H - I - J - K - L - M - N - O - P - Q - R - S - T - U - V - W - X - Y - Z
Also see	* Comprehensive list of genetic and rare diseases
Resources	* NIH GARD, US Genetic and Rare Diseases Info Rare Disease Day Undiagnosed Diseases Network Database of rare diseases at Orphanet Rare Disease UK Rare diseases search engine

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Categories:

@@ Line 1: / Line 1: @@
+{{SI}}
+{{Infobox medical condition
+| name           = Inflammatory linear verrucous epidermal nevus
+| image          = [[File:Inflammatory_verrucous_linear_epidermal_nevus.jpg|left|thumb|Inflammatory linear verrucous epidermal nevus]]
+| caption        = Inflammatory linear verrucous epidermal nevus on the skin
+| synonyms       = ILVEN
+| specialty      = [[Dermatology]]
+| symptoms       = [[Pruritus]], [[verrucous]] [[papules]]
+| onset          = [[Childhood]]
+| duration       = [[Chronic]]
+| causes         = [[Genetic mutation]]
+| risks          = [[Family history]]
+| diagnosis      = [[Clinical diagnosis]], [[skin biopsy]]
+| differential   = [[Psoriasis]], [[linear epidermal nevus]]
+| treatment      = [[Topical corticosteroids]], [[retinoids]], [[laser therapy]]
+| frequency      = Rare
+}}
 == '''Alternate names''' ==
 ILVEN; Linear verrucose epidermal nevus; Verrucous epidermal nevus; Verrucous nevus; Inflammatory linear verrucous epidermal naevus; Inflammatory linear verrucous epidermal nevus
 == '''Definition''' ==
 Inflammatory linear verrucous epidermal nevus (ILVEN) is a type of skin overgrowth, called [[epidermal nevus]].
-[[File:Inflammatory verrucous linear epidermal nevus.jpg|thumb]]
+[[File:Inflammatory verrucous linear epidermal nevus.jpg|left|thumb]]
 == '''Genetics''' ==
 ILVEN is caused by a genetic change that occurs after conception ([[somatic]] mutation).
 == '''Signs and symptoms''' ==
 * It is characterized by skin colored, brown, or reddish, wart-like [[Papule|papules]] (nevi).
 * The nevi join to form patches or plaques that often follow a pattern on the skin known as the "lines of Blaschko".
 * The affected areas of the skin may be red, itchy, and inflamed.
 * ILVEN typically presents from birth to early childhood and often is limited to one side of the lower half of the body.
 * It affects females more often than males.
 * It usually occurs alone; however, rarely ILVEN can be associated with other symptoms as part of an epidermal nevus syndrome.
 * Rarely, ILVEN can become cancerous (for example, [[basal cell carcinoma]] or [[squamous cell carcinoma]]).
 == '''Diagnosis''' ==
 * A diagnosis of ILVEN syndrome should be considered in patients with extensive verrucous, pruritic epidermal nevi, and/or systemic abnormalities.
 * A thorough [[mucocutaneous]], [[neurologic]], [[ophthalmic]], and orthopedic examination is necessary with specific investigations depending on the involved system.
 * A regular follow-up is necessary due to the risk of malignant transformation of the nevi and development of systemic manifestations.<ref>Kumar, C. A., Yeluri, G., & Raghav, N. (2012). Inflammatory linear verrucous epidermal nevus syndrome with its polymorphic presentation - A rare case report. Contemporary clinical dentistry, 3(1), 119–122. https://doi.org/10.4103/0976-237X.94562</ref>.
 == '''Treatment''' ==
 * There is no one treatment or cure for ILVEN. Products used to soften the skin ([[Emollient|emollients]]), moisturizers, and [[Topical steroid therapy|topical steroids]] may be utilized to relieve symptoms of dryness and itching.
 * Recently, several patients have been treated with [[laser therapy]] with good results.
 <references />
 {{Diseases of the skin and appendages by morphology}}
 {{Skin tumors, epidermis}}
 [[Category:Epidermal nevi, neoplasms, and cysts]]
 {{rarediseases}}
 {{stub}}