Rom J Morphol Embryol 2016, 57(2 Suppl):817–823 ISSN (print) 1220–0522 ISSN (online) 2066–8279 CASE REPORT Histopathological and clinical traps in lichen sclerosus: a case report DACIANA ELENA BRĂNIŞTEANU 1) , DANIEL CONSTANTIN BRĂNIŞTEANU 2) , GABRIELA STOLERIU 1) , DAN FERARIU 3) , CĂTĂLINA MARIA VOICU 1) , LOREDANA ELENA STOICA 4) , CONSTANTIN CĂRUNTU 5) , DANIEL BODA 6) , FLORINA MIHAELA FILIP-CIUBOTARU 7) , ANDREEA DIMITRIU 1) , CEZAR-DORU RADU 8) 1) Department of Dermatology, “Grigore T. Popa” University of Medicine and Pharmacy, Iassy, Romania 2) Department of Ophthalmology, “Grigore T. Popa” University of Medicine and Pharmacy, Iassy, Romania 3) Department of Pathology, Regional Institute of Oncology, Iassy, Romania 4) Department of Dermatology, University of Medicine and Pharmacy of Craiova, Romania 5) Department of Immunology, “Victor Babeş” National Institute for Research and Development in Pathology and Biomedical Sciences, Bucharest, Romania 6) Dermatology Research Laboratory, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania 7) Department of Adult Family Medicine, “Grigore T. Popa” University of Medicine and Pharmacy, Iassy, Romania 8) “Gheorghe Asachi” Technical University, Iassy, Romania 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. Keywords: lichen sclerosus et atrophicus, systemic scleroderma, acrosclerosis.  Introduction Lichen sclerosus et atrophicus and limited systemic scleroderma (acrosclerosis) are inflammatory skin disorders that ultimately evolve into two distinct types of scar formation. Lichen sclerosus et atrophicus is a disease of unknown etiology, which most commonly involves the genital mucosa, the causative factors of which are genetic pre- disposition and infectious agents (human papilloma virus, spirochetes). This disease is a chronic inflammatory dermatosis that presents with severe itching, epidermal atrophy and dermal sclerosis affecting predominately the anogenital area of post-menopausal females [1]. It can affect both sexes, mainly females in the fifth and sixth decades of life [2]. The literature reports cases in which lichen sclerosus is associated with various forms of scleroderma, but most commonly with localized morphea [3]. There is an association with autoimmune diseases like vitiligo, thyroid disorders, type I diabetes, and alopecia areata [4]. Other pathogenic factors are low levels of androgens, genetic susceptibility, chronic infections, and trauma [4–6]. Characteristic histopathological features of lichen sclerosus involve epidermis and superficial dermis, with marked attenuation of the epidermis with hyper- keratosis, degeneration of the basal layer, follicular plugging, a band of homogenized collagen in the papillary dermis above a lymphocytic infiltrate [7]. Extensive vacuolar degeneration of the basal layer and edema may lead to fragility of the dermal–epidermal junction resulting in bulla formation in the case of a bullous lichen sclerosus [8]. Scleroderma is a connective tissue disease of unknown cause, produced by autoimmune mechanism, and charac- terized by marked fibrosis of the dermis and epidermal atrophy associated with visceral involvements. While lichen sclerosus does not cause significant damage to internal organs, limited systemic scleredema is associated with extracutaneous involvements that occur in the late stages of disease, in order of frequency these being pulmonary, renal and/or gastrointestinal. R J M E Romanian Journal of Morphology & Embryology http://www.rjme.ro/