Ilven Q82.5

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Blaschko-linear inflammatory dermatosis; dermatitic epidermal nevus; Inflammatory linear verrucous epidermal nevus; Inflammatory Linear verrucous epidermal nevus; Inflammatory nevus linear verrucous epidermal; Linear inflammatory nevus verrucosus

History
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Altman and Mehregan 1971

Definition
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Sharply defined mosaic dermatosis with eczematous, lichenoid or psoriatic papules or plaques with different surface textures, arranged in stripes (in the Blaschko lines)

These formations arranged according to the Blaschko line pattern (not sporotrichoid along the lymphatic channels or segmental) are highlighted by their arrangement, which is unusual for other macro patterns (see below Blaschko linear inflammatory dermatoses).

Occurrence/Epidemiology
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w:m=4:1

Manifestation
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Congenital or occurring in early childhood. In 75% of the patients the onset of the disease is <5 years. Rarely first appearance in adulthood. Very sporadic reports of familial occurrence have been made.

Localization
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Different localisations are possible: mainly trunk and extremities, less frequently face and neck.

Clinical features
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Sharply defined, linear or band-shaped red plaque(s) (or aggregated papules) with different (smoothlichenoid, eczematous or psoriasiform, verrucous) surface characteristics.

These formations impress by their patterns which are unusual for other body patterns and therefore very conspicuous (see below Blaschko lines; see below mosaic, cutaneous).

The lesions can be ichthyosiform, eczematous, psoriasiform, lichen planus-like or M. Darier-like in character.

Itching may be present. Arthritic changes may be associated with a psoriasiform ILVEN.

Histology
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Very different pattern. As the ILVEN does not represent an "independent disease" but only an inflammatory dermatosis in a special (linear) arrangement (e.g. psoriasiform or eczematous ILVEN), the histological result will reflect a clinical correlate (see also Lichen striatus).

Therapy
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  • Purely nurturing or anti-inflammatory therapy with weak or medium-strength glucocorticoids depending on the degree of inflammation, e.g. with 1% hydrocortisone emulsion, 0.1% triamcinolone acetonide cream R259, 0.1% methylprednisolone cream(e.g. Advantan), 0.1% hydrocortisone butyrate cream (e.g. Alfason). Individual successes were described with topical tretinoin, 5-fluorouracil and calcitriol (e.g. Silkis).
  • In small flocks the excision should be discussed. Grinding or laser treatment of the skin is not very useful as recurrences occur almost regularly (explanation: the nevoid transformation does not only affect the epidermis but also the dermal structures).
Overall, the success of the listed treatment modalities is unsatisfactory. A spontaneous regression, which often occurs, must be taken into account! In this respect it is also possible to wait, taking into account the patient's level of suffering.

Progression/forecast
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Sometimes slow magnification. Spontaneous (and surprising) healing is possible until adulthood.

Literature
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  1. Böhm M (2003) Successful treatment of inflammatory linear verrucous epidermal naevus with topical vitamin D3 (calcitriol). Br J Dermatol 148: 824-825
  2. Lee IW et al (1999) Inflammatory linear verrucous epidermal naevus arising on a burn scar. Acta Derm Venereol 79: 164-165
  3. Sidwell RU et al (2001) Pulsed dye laser treatment for inflammatory linear verrucous epidermal naevus. Br J Dermatol 144: 1267-1269
  4. Ulkur E et al (2005) Carbon dioxide laser therapy for an inflammatory linear verrucous epidermal nevus: a case report. Aesthetic Plast Surgery 28: 428-430
  5. Unna PG (1894) The histopathology of skin diseases. A. Hirschwald, Berlin
  6. Vissers WH et al (2004) Immunohistochemical differentiation between inflammatory linear verrucous epidermal nevus (ILVEN) and psoriasis. Eur J Dermatol 14: 216-220

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Last updated on: 29.10.2020