Ilven Q82.5

Altman and Mehregan 1971

Sharply delineated mosaic dermatosis arranged in stripes (in the Blaschko lines) with eczematous, lichenoid or psoriasiform papules or plaques of varying surface texture.

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

w:m=4:1

Apparently, a mutated cell clone that represents a locus minoris resistentiae has grown during embryogenesis. Due to a realization factor X, an otherwise generalized disease manifests itself, exclusively localized and exclusively in the Blaschko lineages.

Mostly acquired in early childhood. In 75% of sufferers, the onset of the disease is <5 years. First occurrence in adulthood is rare. Very occasionally, familial occurrence has been reported (?).

Sharply demarcated, linear or band-like, red plaque(s) (or aggregated papules) with varying (smooth-lichenoid, eczematous or psoriasiform, verrucous) surface texture. These formations are impressive because of their patterns, which are unusual for other body patterns and therefore very conspicuous (see below Blaschko lines; see below Mosaic, cutaneous).

Lesions may be ichthyosiform, eczematous, psoriasiform, lichen planus-like(lichen striatus), or Darier-like in character. Itching may be present. In psoriasiform ILVEN, arthritic changes may be associated (in rare cases).

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).

• Purely maintenance or antiphlogistic therapy with weak or moderate 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). Isolated success has been described with topical tretinoin, 5-fluorouracil , and calcitriol (e.g., Silkis).
• Excision should be discussed for small foci. Abrasion or laser of the skin is of little use, since recurrences occur almost regularly (explanation: nevoid transformation affects not only the epidermis but also the dermal structures).

Overall, the success of the listed treatment modalities is unsatisfactory. A not rarely occurring spontaneous regression has to be taken into account! In this respect, taking into account the patient's suffering, it is also possible to wait.

Sometimes slow enlargement. Spontaneous (and surprising) regressions are possible at any age (see figure).

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