ILVEN‐like Persistent Psoriasiform Dermatitis Confined to a Congenital Becker Nevus

Abstract

A 10-year-old boy presented with a 5-year history of an intractably pruritic, recalcitrant psoriasiform plaque in a broad vertical band on the left buttock, with histologic as well as clinical features suggestive of an inflammatory linear verrucous epidermal nevus. This lesion was completely superimposed upon a congenital Becker nevus. We postulate that the restricted distribution and persistence of the psoriasiform plaque reflected an inflammatory response limited to the aberrant clone of cells composing the Becker nevus, a manifestation of cutaneous mosaicism that could be characterized as an "inflammatory Becker nevus."

Widely Spread Unilateral Inflammatory Linear Verrucous Epidermal Nevus (ILVEN)

“…ILVEN is an epidermal nevus that clinically and histologically mimics linear psoriasis [ 3 , 4 , 5 , 6 , 7 ]. In addition, ILVEN occasionally responds to the antipsoriatic drug calcipotriol, and even responds to anti-TNF antibodies such as etanercept [ 3 ].…”

“…These reports strongly suggested the significance of these proinflammatory cytokines in the pathogenesis of psoriasis. Inflammatory linear verrucous epidermal nevus (ILVEN) is an epidermal nevus that clinically and histologically mimics linear psoriasis [ 3 , 4 , 5 , 6 , 7 ]. Since the treatment for ILVEN varies widely from the administration of topical ointment for psoriasis to invasive methods such as carbon dioxide gas laser 8 , the differential diagnosis between ILVEN and psoriasis is necessary.…”

Congenital Linear Becker’s Nevus, with Underlying Breast Hypoplasia that Spontaneously Corrected during Pregnancy: Role of Androgen Receptors

“…Clinically the differential diagnosis included a linear epidermal nevus, which would not be expected to have underlying breast hypoplasia, and a Becker's nevus, that has rarely been reported as congenital and/or linear [3,5]. Histopathologic examination of a biopsy specimen taken from the nevus demonstrated epidermal acanthosis with elongation of the rete ridges, prominent basal hyper pigmentation and hamartomatous enlarged smooth muscle fibers within the dermis, features that would be consistent with a diagnosis of Becker's nevus [3].…”

“…Although the commonest age of presentation is around puberty, congenital and late-onset lesions have been reported; [3,4] linear lesions are also very rare [5].…”

Rethinking the prosaicism of mosaicism; is it in us?

“…Epidermal nevi represent a straw man for rethinking mutation frequency. The underlying mutations exhibit reliable phenotypic penetrance, and functional consequences are well studied (1–3). An epidermal nevus of the epidermolytic type is observed to arise in at least one in 800 000 live births (1,4) via spontaneous mutation of either keratin 1 or 10.…”