Coexistence of morphea and lichen sclerosus et atrophicus: case report

Abstract

Morphea is an autoimmune disease of the connective tissue, characterized by excessive deposition of collagen, which leads to induration of the skin. It is three times more common in females compared to males. Although it is not an inherited disorder, certain HLA subtypes (HLA-DRB1*04:04 and HLA-B*37) are associated with an increased risk of morphea. Up to 40% of patients with severe forms of morphea have a personal or family history of autoimmune or rheumatologic diseases [1]. Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory condition of the skin and mucous membranes, its most frequent presentation being on the genital regions of the body and in only 15% of cases – extragenital. LSA can start at any age, although it is most often diagnosed in women over 50. The oxidative stress is essential in the pathogenesis of both diseases. Although the coexistence of LSA and morphoea are well established in the literature [2], in medical practice this association is rarely encounter Objective of the study To evaluate the clinical, diagnostic and therapeutic characteristics in a case of morphea associated with lichen sclerosus et atrophicus. Case presentation We present a prospective case involving a 74-years-old woman diagnosed with plaque morphea associated with lichen sclerosus et atrophicus. The disease started 7 years ago with multiple itchy lesions, spread on the trunk, upper limbs and genitals. The previously administered treatments were ineffective and the pathological skin process progressed, the itching becoming very intense. Comorbidities - hypertension and hepatitis C. Her family history was unremarkable. Dermatological examination revealed the presence of sclerotic plaques, with a shiny surface, yellowish-waxy in color, with a violet halo, well-demarcated, with an irregular outline, centered by areas of atrophy. It alternated with multiple polygonal, whitish, pearly papules, with a „parchment-like” surface. Also - melanonychia on the fingers and toes. On the genitals - pearly-white, parchment-like plaques, with a well-defined contour, irregular shape, cracks were attested. Paraclinical investigations revealed thrombocytopenia, lymphocytosis, highly elevated liver tests, positive anti-HCV antibody, positive anti-Borrelia IgG antibody. Dermoscopy revealed whitish fibrotic beams, network-like pigment structures, linear branching vessels and the absence of hair follicles – patterns characteristic for morphea, as well as “comedo-like” openings, telangiectasia of different lengths and calibers, arranged on a pale pink, unstructured, homogeneous background – patterns for LSA. Systemic therapy with Methylprednisolone 0,5 mg/kg, vitamin E, pentoxifylline, ascorbic acid, antihistamines, topical dermatocorticosteroids and heparin gel led to the improvement of the pathological skin process with the paleness of the violaceous border of the lesions and the considerable reduction of itching. Conclusions In the presented case, we emphasize the coexistence of morphea and LSA - distinct pathologies, but with a possible etiological connection. The presence of LSA lesions on the genitals and extragenital is also particular. Dermoscopic patterns confirmed the clinical diagnosis avoiding invasive methods. The administered systemic and topical treatment induced the improvement of the skin manifestations of both pathologies with the remission of itching