Histopathological and clinical traps in lichen sclerosus: a case report

Abstract

Lichen sclerosus et atrophicus and limited systemic scleroderma (acrosclerosis) are inflammatory skin diseases that ultimately evolve into two distinct modes of atrophic scar formation, but which can easily be confused clinically. They are very rarely associated. The literature has reported cases in which lichen sclerosus was associated with various forms of scleroderma, but often with localized morphea. The characteristic histopathological picture of lichen sclerosus includes a thin epidermis, with orthohyperkeratosis and vascular degeneration in the basal layer, loss of elastic fibers, and band-like inflammatory infiltrate in the papillary dermis, while systemic sclerosis is characterized by excessive deposition of collagen in the dermis, accompanied by reduction in adnexal structures and their entrapment in collagen, and the presence of perivascular lymphocytic inflammatory infiltrate. We present the case of a 40-year-old female patient clinically diagnosed with systemic scleroderma and lichen sclerosus involving the genital mucosa. Physical examination in conjunction with laboratory findings (elevated antinuclear, anti-Scl-70, anti-SSA antibodies and immunogram) induced the supposition of the coexistence of lichen sclerosus and systemic scleroderma, fact confirmed by pathological examination. Systemic therapy with corticosteroids, immunosuppressive and phlebotropic drugs, peripheral vasodilators and other tropic adjuvants and topically potent topical corticosteroids was initiated. The course was favorable under therapy, the hardened skin slightly regaining elasticity, relief of itching and disappearance of lichen sclerosus lesions. Our case reaffirms the uncommon association of these two disorders. The importance of history, physical and laboratory examinations in making a diagnosis of certainty in emphasized.