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Concept: Lichen sclerosus

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Background  Scleroderma is a connective tissue disease that includes localized and systemic forms. Our recent encounter with a morphea case exhibiting prominent perineural inflammation microscopically prompted us to assess the features of all patients diagnosed with morphea/scleroderma at our institution. Objective/methods  To describe the clinicopathological features of all patients diagnosed with morphea/scleroderma at American University of Beirut Medical Center (AUB-MC) between 1999 and 2010, and compare our findings with those published in the literature. Results  A total of 81 cases (63 women and 18 men) were identified, of which 73 were localized (morphea) and eight were systemic scleroderma. Clinically, plaque type morphea was the most common variant both in adults and children, and seven (9%) cases of morphea were associated with lichen sclerosis et atrophicus (LSA). Histopathologically, perineural inflammation was observed in 49% of cases, and may serve, in addition to other features including lichen sclerosis-like changes (observed in exclusively nine cases of morphea), more diffuse dermal and less subcutaneous sclerosis, and intense inflammation, as clues favouring diagnosis of morphea over systemic sclerosis. Conclusion  The features of morphea/scleroderma patients in this study are generally comparable to those published in the literature, with few differences. Clinically, plaque type morphea was the most common variant both in adults and children and LSA was a frequent association. Histopathologically, perineural inflammation was commonly observed and may serve in addition to lichen sclerosis-like changes and intense inflammation as clues favouring diagnosis of morphea over systemic sclerosis.

Concepts: Lichen sclerosus, Systemic scleroderma, Rheumatology, Scleroderma

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We established diagnostic criteria and severity classification of lichen sclerosus et atrophicus, because there is no established diagnostic criteria or widely accepted severity classification of the disease. Also, there is no clinical guideline for lichen sclerosus et atrophicus in Japan, so we proposed its clinical guideline. The clinical guidelines were formulated by clinical questions and recommendations on the basis of evidence-based medicine according to the New Minds Clinical Practice Guideline Creation Manual (version 1.0). We aimed to make the guidelines easy to use and reliable including the newest evidence, and to present guidance for various clinical problems in treatment of lichen sclerosus et atrophicus.

Concepts: Avicenna, The Canon of Medicine, Medical terms, Lichen sclerosus, Medical literature

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Vulvar lichen sclerosus et atrophicus (VLSA) is a chronic inflammatory skin disease of unknown etiology that mainly affects postmenopausal and perimenopausal women. The primary clinical symptoms of VLSA are itching, burning pain, and dyspareunia that can results in decreased quality of life. Existing therapies including topical corticosteroid ointment, topical calcineurin inhibitors, estrogens, are not very effective for treatment of VLSA.

Concepts: Estrogen, Osteoporosis, Medicine, Menopause, Lichen sclerosus, Vulva

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Lichen planus pemphigoides (LPP) is a rare blistering disease with features of both lichen planus and bullous pemphigoid. LPP typically appears on the extremities and occasionally involves the oral mucosa. Herein, we describe a case of LPP of the vulva of an 80-year-old woman, an uncommon location for this disease process. This clinical scenario can be confused with a number of similarly appearing entities such as erosive vulvar lichen planus, mucous membrane pemphigoid, and erosive lichen sclerosus et atrophicus. In fact, our patient carried a diagnosis of lichen sclerosus by an outside physician for 2 years prior to being properly diagnosed and treated. A detailed discussion of the epidemiology, clinical, and pathogenesis as well as the histologic and immunofluorescence characteristics of this uncommon diagnosis is presented. Our case emphasizes the necessity of microscopic analysis to differentiate lookalike disease states when making a diagnosis and choosing the correct therapeutics.

Concepts: Bullous pemphigoid, Lichen planus, Lichenoid eruptions, Vulva, Medicine, Mucous membrane, Lichen sclerosus, Oral mucosa

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To generate a list of items through international expert consensus consisting of both symptoms and clinical signs for inclusion in an adult vulvar lichen sclerosus severity scale.

Concepts: Medical sign, Symptom, Lichen sclerosus, Vulva

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A variety of disorders may be confused with vulvar or genital lichen sclerosus, as they can share several common features. This article discusses diagnosis, workup, and management of vulvar lichen sclerosus with specific attention on distinguishing it from other dermatologic mimics.

Concepts: Lichen sclerosus, Vulva

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Purpose To assess the effectiveness, tolerability and convenience of the cream formulation of mometasone furoate 0.1% (MMF) in the treatment of active vulvar lichen sclerosus (VLS) and to compare the cream with the ointment formulation. Methods The following efficacy parameters were assessed in 27 VLS patients treated with MMF cream for 12 weeks (group A): i) response rate, ii) percentage of patients achieving an improvement from baseline of ≥75% in subjective and objective scores, iii) mean reduction in subjective and objective scores. These efficacy assessments, as well as those regarding safety and adherence, were compared with the assessments recorded among 37 VLS patients treated with MMF ointment (group B). Results 59.3% (group A) and 78.4% (group B) of patients were considered responders; 44.4% and 40.7% of patients in group A and 54.1% and 45.9% in group B achieved an improvement of at least 75% in subjective and objective scores, respectively. MMF ointment obtained a significantly higher improvement in symptom scores in comparison with the cream formulation.

Concepts: Lichen sclerosus, Following, Comparison, Vulva, Assessment

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Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis, characterized by shiny, atrophic, hypochromic papules with a predilection for the genital and perineal skin. Extragenital involvement may occur, but is rare in the isolated form. LSA more commonly affects prepubertal and postmenopausal women. We describe an unusual case of isolated extragenital LSA, restricted to the wrists and mimicking lichen planus.

Concepts: Lichen planus, Cutaneous conditions, Lichenoid eruptions, Vulva, Lichen sclerosus

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Vulvar lichen sclerosus is a chronic and incurable disease that causes various unpleasant symptoms and serious consequences.

Concepts: Lichen sclerosus, Cure, Vulva, Disease, Medicine

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The use of special silk textiles (Dermasilk) has shown positive effects on chronic inflammatory diseases like lichen sclerosus et atrophicus, atopic dermatitis, diabetic ulcerations, and vulvovaginal candidiasis.

Concepts: Carpet, Vulva, Asthma, Textile, Silk, Acne vulgaris, Lichen sclerosus, Inflammation