Inflammatory linear verrucous epidermal nevus

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Inflammatory linear verrucous epidermal nevus
Other namesILVEN
Inflammatory verrucous linear epidermal nevus.jpg
Verrucous linear plaque following the lines of Blaschko over the right upper arm of a young girl

Inflammatory Linear Verrucous Epidermal Nevus is a rare disease of the skin that presents as multiple, discrete, red papules that tend to coalesce into linear plaques that follow the Lines of Blaschko. The plaques can be slightly warty (psoriaform) or scaly (eczema-like). ILVEN is caused by somatic mutations that result in genetic mosaicism. [1] [2] [3] There is no cure, but different medical treatments can alleviate the symptoms.

Contents

Classification

ILVEN is a condition that normally only affects one side of the body (unilateral). Usually the left side of patients is affected. The condition is persistent and forms along characteristic lines. It usually appears on an extremity in infancy or childhood. Altman and Mehregan described six characteristic features of ILVEN: (1) early age of onset, (2) predominance in females (4:1 female-male ratio), (3) frequent involvement of the left leg, (4) pruritus, or "itchiness" (5) marked refractoriness to therapy, and (6) a distinctive psoriasiform and inflammatory histologic appearance. [4]

Genetics

Most cases are sporadic, but a familial case, with the condition occurring in a mother and her daughter, has been described.[ citation needed ]

It also has been proposed that activation of an autosomal dominant lethal mutation that survives by mosaicism may be the cause of the lesions. The mutated cells may survive in the due to proximity of normal cells. Another theory is that retrotransposable elements may be the cause of all skin conditions along the Lines of Blashko. Some dogs have a coat variation based upon a similar mechanism. [5]

The classification of the disease has much to do with the appearance and location of the lesions (phenotype characteristics). As this is a rare condition, it is possible that more than one genotype could cause a similar phenotype. Different genes have been implicated, but the number of patients studied in each case is very low.[ citation needed ] There is some evidence that interleukins 1 and 6, tumor necrosis factor α, and intercellular adhesion molecule-1 are upregulated in ILVEN, similar to psoriasis.

Histopathology

-The plaques are characterized histologically by hyperkeratosis which is a thickening of the outer layer of skin. Hyperkeratosis is often associated with an abnormal amount of keratin production. Also characteristic is moderate acanthosis a thickening of the stratum spinosum with elongation of rete ridges.[ citation needed ]

- Characteristic histologic feature is regular alternation of slightly raised parakeratotic areas without a granular layer (hypogranulosis) and slightly depressed orthokeratotic areas with prominent granular layer (hypergranulosis). Orthokeratotic hyperkeratosis is characterised by hyperkeratosis with non-nucleated cells. Parakeratotic hyperkeratosis is characterised by hyperkeratosis with nucleated cells.

- The orthokeratotic area shows a basket-weave-pattern.

- The dermis shows scattering of chronic inflammatory infiltrate (Munro's microabscess) sometimes giving a spongiform appearance.

This is very similar to linear psoriasis, but it has been noted that the diseases are distinct entities by immunohistochemical analyses. [6]

Immunohistochemistry

Patients with ILVEN with and without associated psoriasis, the number of Ki-67 positive nuclei, tended to be lower than is typically found in psoriasis. [6] Additionally, the number of keratin-10 positive cells and HLA-DR expression was higher as compared to psoriasis. In ILVEN without associated psoriasis all T-cell subsets and cells expressing NK receptors were reduced as compared to psoriasis, except for CD45RA+ cells. In particular the density of CD8+, CD45RO+ and CD2+, CD94 and CD161 showed a marked difference between ILVEN without psoriasis and psoriasis itself. T cells relevant in the pathogenesis of psoriasis are markedly reduced in ILVEN without psoriasis as compared to psoriasis.

Treatment

Reported treatments include topical agents, dermabrasion, cryotherapy, laser therapy, and surgical excision. These therapies have a high failure rate because of incomplete relief of symptoms, scarring, or recurrence [ citation needed ].

Though similar in appearance, ILVEN will not respond to therapies known to affect psoriasis. ILVEN can be very difficult to live with but can be treated. The most effective method is full-thickness excision of the lesion. [7] CO2 laser surgery can resurface the skin to give a flat, smoother and more normal appearance, but does not remove the lesion.

History

The condition later known as ILVEN was first described by Paul Gerson Unna in 1896. [8] ILVEN appears very similar to psoriasis. However, it was not until 1971 that the disorder was described and clearly defined as a distinct entity by Altman and Mehregan in a case study of 25 patients. [4] The Dupre and Christol described histopathological criteria in 1977. [9]

Epidemiology

ILVEN usually appears in infancy or early childhood. The condition is very rarely begun in adulthood. ILVEN occurs predominantly in females (female-male ratio, 4:1) with no racial predominance.

See also

Related Research Articles

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Psoriasis is a long-lasting, noncontagious autoimmune disease characterized by patches of abnormal skin. These areas are red, pink, or purple, dry, itchy, and scaly. Psoriasis varies in severity from small localized patches to complete body coverage. Injury to the skin can trigger psoriatic skin changes at that spot, which is known as the Koebner phenomenon.

<span class="mw-page-title-main">Epidermolytic hyperkeratosis</span> Medical condition

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<span class="mw-page-title-main">Skin condition</span> Any medical condition that affects the integumentary system

A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of this system is as a barrier against the external environment.

<span class="mw-page-title-main">Nevus</span> Mole or birthmark; visible, circumscribed, chronic skin lesion

Nevus is a nonspecific medical term for a visible, circumscribed, chronic lesion of the skin or mucosa. The term originates from nævus, which is Latin for "birthmark"; however, a nevus can be either congenital or acquired. Common terms, including mole, birthmark, and beauty mark, are used to describe nevi, but these terms do not distinguish specific types of nevi from one another.

<span class="mw-page-title-main">Dysplastic nevus</span> Medical condition

A dysplastic nevus or atypical mole is a nevus (mole) whose appearance is different from that of common moles. In 1992, the NIH recommended that the term "dysplastic nevus" be avoided in favor of the term "atypical mole". An atypical mole may also be referred to as an atypical melanocytic nevus, atypical nevus, B-K mole, Clark's nevus, dysplastic melanocytic nevus, or nevus with architectural disorder.

<span class="mw-page-title-main">Lentigo maligna</span> Medical condition

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<span class="mw-page-title-main">Blaschko's lines</span> Anatomical structure

Blaschko's lines, also called the lines of Blaschko, are lines of normal cell development in the skin. These lines are only visible in those with a mosaic skin condition or in chimeras where different cell lines contain different genes. These lines may express different amounts of melanin, or become visible due to a differing susceptibility to disease. In such individuals, they can become apparent as whorls, patches, streaks or lines in a linear or segmental distribution over the skin. They follow a V shape over the back, S-shaped whirls over the chest and sides, and wavy shapes on the head. Not all mosaic skin conditions follow Blaschko's lines.

<span class="mw-page-title-main">Angiokeratoma</span> Medical condition

Angiokeratoma is a benign cutaneous lesion of capillaries, resulting in small marks of red to blue color and characterized by hyperkeratosis. Angiokeratoma corporis diffusum refers to Fabry's disease, but this is usually considered a distinct condition.

<span class="mw-page-title-main">Cutis marmorata telangiectatica congenita</span> Medical condition

Cutis marmorata telangiectatica congenita is a rare congenital vascular disorder that usually manifests in affecting the blood vessels of the skin. The condition was first recognised and described in 1922 by Cato van Lohuizen, a Dutch pediatrician whose name was later adopted in the other common name used to describe the condition – Van Lohuizen Syndrome. CMTC is also used synonymously with congenital generalized phlebectasia, nevus vascularis reticularis, congenital phlebectasia, livedo telangiectatica, congenital livedo reticularis and Van Lohuizen syndrome.

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Skin biopsy is a biopsy technique in which a skin lesion is removed to be sent to a pathologist to render a microscopic diagnosis. It is usually done under local anesthetic in a physician's office, and results are often available in 4 to 10 days. It is commonly performed by dermatologists. Skin biopsies are also done by family physicians, internists, surgeons, and other specialties. However, performed incorrectly, and without appropriate clinical information, a pathologist's interpretation of a skin biopsy can be severely limited, and therefore doctors and patients may forgo traditional biopsy techniques and instead choose Mohs surgery.

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<span class="mw-page-title-main">Porokeratosis</span> Medical condition

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<span class="mw-page-title-main">Linear verrucous epidermal nevus</span> Medical condition

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<span class="mw-page-title-main">Supernumerary nipples–uropathies–Becker's nevus syndrome</span> Medical condition

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Stasis papillomatosis is a disease characterized by chronic congestion of the extremities, with blood circulation interrupted in a specific area of the body. A consequence of this congestion and inflammation is long-term lymphatic obstruction. It is also typically characterized by the appearance of numerous papules. Injuries can range from small to large plates composed of brown or pink, smooth or hyperkeratotic papules. The most typical areas where injuries occur are the back of the feet, the toes, the legs, and the area around a venous ulcer formed in the extremities, although the latter is the rarest of all. These injuries include pachydermia, lymphedema, lymphomastic verrucosis and elephantosis verrucosa. The disease can be either localized or generalized; the localized form makes up 78% of cases. Treatment includes surgical and pharmaceutical intervention; indications for partial removal include advanced fibrotic lymphedema and elephantiasis. Despite the existence of these treatments, chronic venous edema, which is a derivation of stasis papillomatosis, is only partially reversible. The skin is also affected and its partial removal may mean that the skin and the subcutaneous tissue are excised. A side effect of the procedure is the destruction of existing cutaneous lymphatic vessels. It also risks papillomatosis, skin necrosis and edema exacerbation.

References

  1. Happle, R. (1995). "What is a nevus? A proposed definition of a common medical term". Dermatology. 191 (1): 1–5. doi:10.1159/000246468. PMID   8589475.
  2. Odom, Richard B.; Davidsohn, Israel; James, William D.; Henry, John Bernard; Berger, Timothy G.; Dirk M. Elston (2006). Andrews' diseases of the skin: clinical dermatology. Saunders Elsevier. ISBN   0-7216-2921-0.
  3. Freedberg; et al. (2003). Fitzpatrick's Dermatology in General Medicine (6th ed.). McGraw-Hill. ISBN   0-07-138076-0.
  4. 1 2 Altman J, Mehregan AH (1971). "Inflammatory linear verrucose epidermal nevus". Arch. Dermatol. 104 (4): 385–389. doi:10.1001/archderm.1971.04000220043008.
  5. Happle, R. (2002). "Transposable elements and the lines of Blaschko: a new perspective". Dermatology. 204: 4–7. doi:10.1159/000051801.
  6. 1 2 Vissers WH, Muys L, Erp PE, de Jong EM, van de Kerkhof PC (2004). "Immunohistochemical differentiation between inflammatory linear verrucous epidermal nevus (ILVEN) and psoriasis". Eur J Dermatol. 14 (4): 216–220.
  7. Binodini Behera; Basanti Devi; Bibhuti B Nayak; Bharti Sahu; Bhabani Singh & Manas R Puhan (2013). "Giant Inflammatory Linear Verrucous Epidermal Nevus: Successfully Treated with Full Thickness Excision and Skin Grafting". Indian J Dermatol. 58 (6): 461–463. doi: 10.4103/0019-5154.119959 . PMC   3827519 . PMID   24249899.
  8. Unna, Paul Gerson (1896). The Histopathology of the Diseases of the Skin. New York, NY: Macmillan.
  9. Dupre A, Christol B (1977). "Bilateral inflammatory linear verrucous epidermal nevus localized on the lip and with minimal histological lesions". Ann. Dermatol. Venereol. 104: 163–4.

Further reading