Lichen sclerosus (LS) is a chronic dermatosis characterized by atrophic white papules or plaques, most commonly occurring on the anogenital skin. Blisters have been rarely described developing in the background of extragenital LS. A 74-year-old woman with a 4-year history of sclerotic patches on the trunk showed a flaccid bulla on the lower back for 3 months. The histopathological finding of the skin biopsy was consistent with the diagnosis of bullous LS. In this paper, we present this uncommon case, review the literature on extragenital bullous LS, discuss the pathogenesis, and provide some treatment options for the case. 相似文献
BACKGROUND: Lichen sclerosus (LS) is a chronic inflammatory skin disease of unknown aetiology which can be associated with secondary malignancies. Recent evidence supports an autoimmune basis for this disorder, as demonstrated by autoantibodies to extracellular matrix protein 1 (ECM-1). The pathophysiological mechanisms leading to autoimmunity and carcinogenesis are poorly understood. OBJECTIVES: We hypothesized that oxidative stress, which has been demonstrated to be involved in the pathogenesis of several autoimmune and malignant disorders, contributes to these processes in LS. METHODS: Skin biopsies from 16 patients with untreated, histologically confirmed vulval LS were examined immunohistochemically using antibodies against the lipid peroxidation products malondialdehyde and 4-hydroxynonenale and against the oxidative DNA damage marker 8-hydroxy-2'-deoxyguanosine. Protein carbonyls as markers of protein oxidation were visualized using the dinitrophenylhydrazone method. Expression of antioxidant enzymes was investigated. Normal vulval tissue from 16 subjects served as control. RESULTS: In vulval LS tissue a significant increase of lipid peroxidation products was found particularly within the basal cell layers of the epidermis, thus colocalizing with ECM-1. Oxidative DNA damage was detected throughout LS biopsies. Intriguingly, protein oxidation was significantly increased within the dermis of LS lesions, indicating oxidative protein damage in the areas of sclerosis and inflammation. The enzymatic antioxidant defence in LS was found to be significantly disturbed. CONCLUSIONS: This is the first study to demonstrate oxidative damage to lipids, DNA and proteins in LS, revealing a novel pathophysiological mechanism which may contribute to sclerosis, autoimmunity and carcinogenesis. Therapeutic strategies using antioxidants might be a useful new approach in the treatment of LS and could also help to prevent secondary malignancies. 相似文献
A 14‐year‐old boy had segmental vitiligo (L3?4) on the right thigh and leg for 4 years, and was advised to apply topical clobetasol propionate, 0.05%, in the night, with daily sun exposure for 10 min, as he refused to comply with topical psoralens. As there was no response to therapy even after 3 months, the patient stopped the steroid cream but continued with the sun exposure. Subsequently, the patient noticed gradual‐onset, itchy, violaceous, pigmented, raised lesions superimposed on the vitiligo macules. Cutaneous examination revealed violaceous, polygonal papules, 0.5 × 0.5 cm in size, some of which coalesced to form discrete violaceous plaques, confined to areas of vitiligo, with a clear‐cut demarcation from normal skin ( Fig. 1 ). The scalp, palms, soles, nails, and mucosa were normal. Histopathology of the polygonal papules revealed hyperkeratosis, wedge‐shaped hypergranulosis, irregular acanthosis with saw toothing of the rete ridges, basal cell liquefaction, and a band‐like lymphocytic infiltrate ( Fig. 2 ), consistent with lichen planus. The patient was subsequently prescribed fluticasone propionate (0.05%) ointment once daily for the lesions of lichen planus. There was a marked improvement in the lesions of lichen planus after 1 month. Figure 1 Open in figure viewer PowerPoint Violaceous papules of lichen planus colocalized on vitiligo macules with associated leukotrichia seen on the right leg 相似文献
Extragenital lichen sclerosus (LS) is usually asymptomatic and appears as ivory- or porcelain-white macules, papules and plaques. It is most common on the neck, shoulders and upper portion of the trunk. To date, the linear form of extragenital LS corresponding to the lines of Blaschko on the trunk or limbs has been rarely reported. We describe a 25-year-old Korean woman with sequentially occurring lesions of extragenital LS confined to the face following the lines of Blaschko. 相似文献