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.
[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.
[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.
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).
- The dermis shows scattering of chronic inflammatory infiltrate (Munro's microabscess) sometimes giving a spongiform appearance.
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].
[7] CO2 laser surgery can resurface the skin to give a flat, smoother and more normal appearance, but does not remove the lesion.