Atypical clinical presentation of lichen planus bullous in a systemic sclerosis patient

We report the case of a 54-year-old woman with progressive systemic sclerosis who presented erythematous papules diagnosed in biopsy as lichen planus bullous (LPB). Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by specific antibodies, vascular abnormalities with progressive damage of blood vessels and diffuse fibrosis leading to their failure. Sys-temic sclerosis coexisting with systemic lupus erythema-tosus, rheumatoid arthritis, dermatomyositis, acquired vitiligo, Sjögren syndrome or chronic hepatitis are well documented [1]. Lichen planus (LP) is a common chronic autoimmune disease associated with immunological dysfunction. Both antigen-specific and non-specific mechanisms may be involved in the pathogenesis [2]. Several factors including stress, genetics, systemic diseases, hepatitis viruses and drugs were implicated as causative agents [3]. Clinical presentation of LP varies resulting in 20 subtypes within the disease. The bullous group was divided into LPB and LP pemphigoides (LPP), which are distinguished by clinical, histological and immunological characteristic features. Cases of co-existing autoimmune skin disorders were described many times suggesting that one autoimmune disease may induce another. A female patient aged 54 years suffering from SSc since 2005 was admitted to the Department of Dermatology , Medical University of Lodz. The patient complained of hand and finger joint pain, diarrhea, and dysphagia. Before developing SSc symptoms the patient was diagnosed with gastroesophageal reflux and Barrett's esophagus. The patient has been under dermatological control since 2005 due to SSc and received vasodilators and vitamin E. In December 2011, erythematous papules accompanied by itching appeared on the forearms (Figure 1). Der-matological examination revealed flat-topped elevated papules, which were reddish-purple and Wickham's striae on lesion surfaces. Blisters were not found. In the mucous membrane of the oral cavity, lacy streaks along buccal occlusion line were observed. Laboratory results (blood count, liver and kidney function , erythrocyte sedimentation rate, C-reactive protein, protein electrophoresis, urine test) were within normal range. The echocardiographic research revealed a slight disorder in mobility of the left ventricle walls, slight mi-tral and tricuspid incompetence and trace of pericar-dial effusion. Scintigraphic study of esophageal motility showed slow transit in the lower part. Chest X-ray and spirometry showed no pulmonary change. Results of serum sample testing for HCV antibodies and antigen HBs were negative. No casual or other provocative factors for LP were detected. The examination of the skin biopsy revealed subcor-neal bulla typical of LPB, which in clinical presentation was not observed either within papules or on the unin-volved skin. On histopathological …