Acral lichen sclerosus et atrophicus

BACKGROUND: Lichen sclerosus et atrophicus rarely affects the feet or hands and in this case, it is generally part of widespread cutaneous involvement. We report a case of lichen sclerosus et atrophicus involving only the extremities and the vulvar and perigenital area. PATIENTS AND METHODS: A 56-year-old woman presented with lesions of the hands and feet, with ivory white papules on the dorsal aspect of the feet and the distal phalanx of the fingers, a few small keratotic papules with central depressions in the hollow of the palms, erythema on soles and thenar and hypothenar eminences. Further examination revealed lichen sclerosus et atrophicus of the vulva and genitocrural skinfolds. Histological study of these various cutaneous lesions yielded similar results and revealed the typical features of lichen sclerosus et atrophicus. DISCUSSION: A few cases of lichen sclerosus et atrophicus confined to the hands and/or feet have been reported, involving the palms and soles or nail folds, but none has so far affected the genitalia. To our knowledge, no cases of lichen sclerosus et atrophicus involving both faces of the hands and feet and the genital region have ever been reported.     

Familial lichen sclerosus et atrophicus in association with CREST syndrome: a case report

Lichen sclerosus et atrophicus is an uncommon disease which appears to be multifaetorial in aetiology. We describe a case of a young woman with CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly and telangiectasial who has a documented family history of two sisters with lichen sclerosus et atrophicus. She presented with vulvar pruritas in association with dyspareunia. and biopsy of atrophic white vulvar lesions was consistent with lichen sclerosus et atrophicus. Lichen sclerosus et atrophicus has been previously noted to occur in association with morphoea and lichen planus, although it has never been reported in conjunction with CREST syndrome.     

Lichen sclerosus et atrophicus, morphea, and coexistence of both diseases. Histological studies using lectins.

Histological studies using three lectins, lens culinaris agglutinin, soybean agglutinin, and Ulex europaeus agglutinin-I, were carried out in a case of coexistent lichen sclerosus et atrophicus and morphea, five cases of morphea, and two cases of lichen sclerosus et atrophicus. The lectin staining patterns of the formaldehyde-fixed epidermis of patients with morphea were not different from those of normal epidermis, but epidermis of patients with lichen sclerosus et atrophicus showed different staining patterns. Lens culinaris agglutinin stained the basal and the spinous layers of the normal epidermis and that of patients with morphea but stained only the basal cells of the epidermis from patients with lichen sclerosus et atrophicus; epidermal Ulex europaeus agglutinin binding was observed only in the cases of lichen sclerosus et atrophicus. Moreover, in the patient with coexistent diseases, the morphea lesion showed the staining profiles of morphea and the lichen sclerosus et atrophicus lesion showed the staining patterns of lichen sclerosus et atrophicus, respectively.     

Milia occurring in lichen sclerosus et atrophicus

Large numbers of milia were seen in areas of skin affected with bullae in a 68-year-old woman who had extensive lichen sclerosus et atrophicus. Although milia are frequently seen in other sub-epidermal bullous disorders, they have not previously been reported in lichen sclerosus et atrophicus.     

24例硬化性萎缩性苔藓临床及病理分析

目的:分析24例硬化性萎缩性苔藓患者的临床、病理表现.方法:对我中心诊治的24例硬化性萎缩性苔藓患者的临床资料进行临床、病理、误诊及治疗情况的回顾性分析.结果:硬化性萎缩性苔藓多见于青年女性,以20～40岁多见,男女比例1:2,皮损好发于外阴;部分区域萎缩和部分区域增生的11例,占45.8%;外用他克莫司疗效好;早期误诊12例(占50%).结论:临床特点及组织病理学检查为诊断本病的依据,医务人员应加强对早期硬化性萎缩性苔藓的认识.     

Spirochetal forms in the dermal lesions of morphea and lichen sclerosus et atrophicus

Morphea and lichen sclerosus et atrophicus are cutaneous diseases that are manifest by an early edematous stage, followed later by sclerosis and atrophy. They share features with acrodermatitis chronica atrophicans and erythema chronicum migrans, diseases that have been linked to infection by the spirochete Borrelia burgdorferi. A modified silver stain was used to identify the presence of spirochetes in skin biopsy specimens of patients with morphea and lichen sclerosus et atrophicus. Spirochetal forms were identified in the lesional skin of 10 of 25 patients with morphea and in 10 of 21 cases of lichen sclerosus et atrophicus. These spiral forms of bacteria had a significant tendency to occur in early and fully developed lesions of morphea and in early lesions of lichen sclerosus et atrophicus, whereas they tended to be absent in lesions demonstrating late pathological changes.     

Lichen sclerosus et atrophicus. A study of 76 cases and their relation to diabetes

A review of 76 patients with lichen sclerosus et atrophicus reveals a number of cases in which this disorder is associated with glucose intolerance or diabetes mellitus. The performance of an oral glucose tolerance test is therefore recommended for all patients with lichen sclerosus et atrophicus.     

The development of lichen sclerosus et atrophicus in monozygotic twin girls

The development of vulval lichen sclerosus et atrophicus in monozygotic twins is described. This is the first report of the occurrence of lichen sclerosus et atrophicus in two genetically identical individuals, and is considered to provide further evidence that inherited factors are of relevance in the aetiology of this disorder.     

Diagnostic criteria,severity classification and guidelines of lichen sclerosus et atrophicus

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.     

A comparison of morphoea and lichen sclerosus et atrophicus in vitro: the effects of para-aminobenzoate on skin fibroblasts.

To study the effects of para-aminobenzoate on the dermis, fibroblast cell lines derived from lesions of lichen sclerosus et atrophicus, from morphoea and from normal skin were incubated with Potaba in vitro. Monolayer cultures containing Potaba showed a dose-dependent inhibition of proliferation beginning at 1,000 micrograms/ml with total inhibition at 10,000 micrograms/ml. Mean ID50 values for the three groups were not significantly different. There was a similar dose dependent inhibition of glycosaminoglycan secretion in all 3 groups, except at 10,000 micrograms/ml where secretion by lichen sclerosus et atrophicus and morphoea fibroblasts was significantly more inhibited than normal lines. Inhibition of the glycosaminoglycan secretion at 10-1,000 micrograms/ml was a direct effect of the drug rather than an indirect effect of changes in cell density, and lichen sclerosus et atrophicus fibroblasts produced about 40% more GAG than the morphoea or normal lines growing at similar densities. Collagen synthesis was increased in both lichen sclerosus et atrophicus and morphoea cell lines, with increased non-collagenous protein in morphoea lines. These results confirm that there are differences between lichen sclerosus et atrophicus and morphoea, and suggest glycosaminoglycan secretion as a possible target for the therapeutic action of Potaba.     

Lichen sclerosus et atrophicus in children

A 7-year-old girl with lichen sclerosus et atrophiais on her back was reported. The lesion showed a complete clinical cure after one year of topical corticosteroid therapy without any significant atrophy. The prognosis of lichen sclerosus et atrophicus in children is better than that in adult cases, so spontaneous resolution could not be ruled out.     

Chronic borreliosis presenting with morphea- and lichen sclerosus et atrophicus-like cutaneous lesions. a case report.

We report on a case of chronic cutaneous borreliosis with manifestations clinically compatible with morphea and lichen sclerosus et atrophicus. The histopathologic features of these lesions were those of acrodermatitis chronica atrophicans. Our case illustrates the concept that clinical aspects of morphea and lichen sclerosus et atrophicus pertain to the spectrum of cutaneous borreliosis.     

Lichen sclerosus et atrophicus and perianal carcinoma: a case report

Lichen sclerosus et atrophicus is known to be associated with squamous cell carcinoma of the vulva. A case report is presented of lichen sclerosus et atrophicus with squamous carcinoma of the perianal region. The patient also had pernicious anaemia and hypothyroidism.     

Lichen sclerosus et atrophicus of the scalp

A patient is described who developed pruritic lesions of the scalp with increasing hair loss and plaque formation over a period of 15 years, in association with patches if white atrophic skin on her trunk and vulva in the last 5 years. Biopsies of the scalp and trunk lesions both showed lichen sclerosus et atrophicus. This would appear to be the first recorded case of lichen sclerosus et atrophicus of the scalp in the English literature.     

Generalized morphoea, lichen sclerosus et atrophicus and primary biliary cirrhosis

A patient with primary biliary cirrhosis, generalized morphoea and lichen sclerosus et atrophicus is reported. This observation re-inforces the hypothesis that these three conditions all have an underlying auto-immune basis.     

Decrease in epidermal CD44 expression as a potential mechanism for abnormal hyaluronate accumulation in superficial dermis in lichen sclerosus et atrophicus

CD44 is a polymorphic integral membrane glycoprotein that serves as the principal cell surface receptor for hyaluronate, the major component of the extracellular matrix. CD44 is abundantly found in the skin and functions as a cell adhesion molecule. In a recent study we have observed a massive dermal accumulation of hyaluronate as a result of the in vivo selective suppression of CD44 in keratinocytes in mice expressing a keratin 5 promoter-driven CD44 anti-sense transgene. As the histologic features of the dorsal skin of these transgenic mice display some similarities to those of the skin lesions of lichen sclerosus et atrophicus, we explored the nature of the material accumulated in the dermis of genital and extragenital lesions of 14 patients with lichen sclerosus et atrophicus by Alcian Blue and human CD44 receptor globulin stainings, as well as the epidermal expression of CD44 protein and mRNA by immunohistochemistry and in situ hybridization. In this study we provide evidence that hyaluronate is accumulated in the superficial dermis of lichen sclerosus et atrophicus lesions, in particular by the use of human CD44 receptor globulin staining, which binds specifically to hyaluronate. In addition we show that the protein and mRNA expression of CD44 in the epidermis of the involved lichen sclerosus et atrophicus skin from genital and extragenital areas is significantly decreased, and in some cases completely lost. In contrast, keratinocyte CD44 expression was un-altered in the skin lesions of lupus erythematosus, scleroderma and reticular erythematous mucinosis, despite the presence of a mucinous material in the dermis. These results suggest that a decrease in CD44 in the keratinocytes may be correlated with an abnormal dermal accumulation of hyaluronate in the lesions of lichen sclerosus et atrophicus, and may play a pathogenetic role in this disease. J Invest Dermatol 115:1054-1058 2000     

Is morphoea caused by Borrelia burgdorferi? A review

The aetiology of morphoea and lichen sclerosus et atrophicus is still unknown. Since the detection of Borrelia burgdorferi (B. burgdorferi) as the causative agent of Lyme disease, there has been debate about a possible association between B. burgdorferi and morphoea. Initial serological and cultural studies showed controversial results. The introduction of polymerase chain reaction (PCR) initially suggested an association between B. burgdorferi and morphoea. We reviewed the literature on B. burgdorferi (specific serology, immunohistology, culture, lymphocyte stimulation and DNA detection by PCR) since 1983, using Medline and Current Contents. Histological and immunohistological detection of B. burgdorferi was reported in 0-40% (20 of 82) of the cases with morphoea and in 46-50% (17 of 36) of the cases with lichen sclerosus et atrophicus. Cultivation of spirochetes from lesional skin succeeded in five patients (five of 68) with morphoea, but failed in patients with lichen sclerosus et atrophicus. In Europe and Asia, serological detection of antibodies against B. burgdorferi was described in 0-60% (138 of 609) of patients with morphoea and in 19% (six of 32) in the U.S.A. For lichen sclerosus et atrophicus 0-25% of the published cases (three of 23) in Europe and Asia were seropositive. DNA from B. burgdorferi was detected by PCR in 0-100% (17 of 82) of the tissues of patients with morphoea in Europe and Asia, but not a single case among 98 patients was reported to be positive from the U. S.A. In Europe and Asia, borrelial DNA was detected in 0-100% (nine of 28) of the cases with lichen sclerosus et atrophicus, whereas in the U.S.A. none of 48 patients was positive. There are two possible explanations for these contradictory findings: the most likely is that B. burgdorferi is not a causative agent for morphoea. Another possible explanation could be that a subset of morphoea is caused by a special subspecies of B. burgdorferi that is present in Europe and Asia but does not occur in the U.S.A.     

Extragenitaler Lichen sclerosus et atrophicus – Behandlung durch gepulsten Farbstofflaser

A 17-year old female patient with extragenital lichen sclerosus et atrophicus was treated with the pulsed dye laser. Local and systemic therapy before treatment showed no effect. The lesions were removed completely with four treatment sessions. As for side effects, no pigment changes and no visible scarring was observed. The patient experienced no recurrence within a follow-up time of 7 months. The mechanism whereby lichen sclerosus et atrophicus is altered by the pulsed dye laser is unknown.     

Morphea Coexisting with Lichen Sclerosus et Atrophicus

A 65-year-old woman presented with four autoimmune diseases. These include morphea, lichen sclerosus et atrophicus, insulin-dependent diabetes mellitus, and autoimmune thyroid disease. The relationship between morphea and lichen sclerosus et A is discussed, as is the link between these conditions and autoimmunity.     

Lichen Sclerosus et Atrophicus An Electron Microscopic Study

The ultrastructural changes in seven cases of lichen sclerosus et atrophicus are described. An interesting epidermal alteration was the presence of collagen fibrils in the intercellular space; furthermore, the basal cells were altered by condensation and homogenization of the tonofibrils. The basement membrane was not uniform and often multilayered. The anchoring fibrils were numerous. In the cutis intertwining strands of fine filaments, bundles of immature collagen and normal collagen could be found. In lichen sclerosus et atrophicus the dermo-epidermal interrelationship is disturbed.