A Case of Linear Type of Lichen Sclerosus et Atrophicus?

We report a case of the linear type of lichen sclerosus et atrophicus (LSA). The patient had linear atrophic lesions from the left upper back to the left hand. Histological findings obtained from the upper back were those of typical LSA. Histology of the forearm showed mild changes suggestive of LSA. There were no histological features suggesting any association with localized scleroderma in either specimen. The relation between LSA and localized scleroderma is unclear at present. This case suggests that there is a linear form in LSA as already recognized in localized scleroderma.     

Localized lichen sclerosus with nail loss

A 52-year-old woman who developed a pale sclerotic second left toe with loss of the nail plate is described. Biopsy showed changes of lichen sclerosus. There were no other skin or genital lesions present.     

High-frequency ultrasound as a useful device in the preliminary differentiation of lichen sclerosus et atrophicus from morphea

High-frequency ultrasound diagnostic equipment (HFUS) using probes with frequencies above 15-MHz has been utilized conventionally in preoperative assessments and postoperative follow-up of skin tumors. The advent of probes with even higher frequencies (up to 30-MHz) has widened the clinical applications of HFUS to inflammatory dermatoses which are histologically confined to the epidermis and upper dermis, such as psoriasis and atopic dermatitis. Ultrasound imaging has the advantage of being a noninvasive and relatively inexpensive technology that is quick and easy to perform. In addition, information obtained from HFUS is unique and useful for clinical diagnosis and evaluation for various skin disorders. We believe that HFUS has the potential to become a powerful tool for either clinical or investigative dermatology. Herein, we present a case of lichen sclerosus et atrophicus (LSA) to which we applied HFUS before skin biopsy and obtained substantial information for the differential diagnosis from morphea, and we reemphasize the usefulness of HFUS in studying the inflammatory skin disorders.     

Topical tacrolimus ointment for the treatment of lichen sclerosus, comparing genital and extragenital involvement

Lichen sclerosus is a chronic inflammatory dermatosis presenting with significant sclerosis, atrophy and pruritus. The treatment for this condition remains unsatisfactory, with potent corticosteroids being the most effective therapy. In this study, we investigated the efficacy and safety of tacrolimus ointment in patients with genital and extragenital lichen sclerosus. Sixteen patients with active lichen sclerosus (10 with anogenital and six with extragenital localization) were treated with topical tacrolimus ointment twice daily. The therapeutic effects were evaluated according to 3 grades: complete response (>75% improvement), partial response (25-75% improvement), or no response (<25% improvement). Applications were continued until complete disappearance or stabilization of the cutaneous lesions. In addition, we conducted telephone surveys to determine the long-term treatment outcome and relapse rate. Objective response to therapy occurred in nine of 10 patients (90%) with anogenital and one of six patients (16.7%) with extragenital lesions. Out of 10 patients with anogenital lichen sclerosus, five showed more than 75% improvement. Complete, partial and no response were achieved in five (50%), four (40%) and one (10%) patient, respectively. During the follow-up period of a mean of 29.3 months, six of nine patients had a relapse of symptoms. However, most patients with extragenital involvement did not respond to tacrolimus, except one patient showing partial response. No significant adverse effects were observed. Topical tacrolimus ointment was a safe and effective treatment for genital lichen sclerosus and should be used for long-term duration to prevent relapse. However, it was not useful for patients with extragenital lichen sclerosus.     

Concomitance of lichen sclerosus et atrophicus and morphea in the same lesions

A 65-year-old woman presented atrophic, slightly indurated plaques on the trunk and a sclerotic, hemorragic plaque on the right forearm. In three biopsies, diagnostic features of both lichen selerosus et atrophicus (LSA) and morphea coexisted. Elastic fibers were absent in the papillary dermis and present in the sclerotic reticular dermis. Although many patients with lesions of both morphea and LSA have been reported, the coexistence of the two histological features in the same lesion is less frequent.     

Ultraviolet A1 phototherapy for the treatment of localized scleroderma

Ultraviolet (UV)A1 phototherapy is effective for T-cell-mediated skin diseases such as atopic dermatitis and mast cell-mediated skin diseases such as mastocytoma. UVA1 phototherapy is also effective against the sclerotic lesions of systemic sclerosis and morphea. Currently, in Japan, access to UVA1 phototherapy is limited because the UVA1 phototherapy device has not yet been approved. On the basis of our experience, we report three patients with localized scleroderma who responded successfully to UVA1 phototherapy. Efficacy was assessed by histological analysis and elastography. UVA1 successfully ameliorated sclerotic lesions, including morphea, linear scleroderma and morphea lesions in a patient with limited cutaneous systemic sclerosis. No side-effects were observed during UVA1 phototherapy.     

A case of lichen sclerosus et atrophicus of the vulva effectively treated with oral etretinate

A 51-year-old female had been suffering from itching of the external genitalia and soreness on urination for one year. Swelling, leukokeratosis and erosion on the external genitalia had been seen for six months. Her clinical findings and symptoms improved markedly after eight months administration of oral etretinate (50–10 mg/day, total dose 6,080 mg). Histologically, reappearance of the rete ridges, rearrangement of squamous cells, disappearance of hydropic degeneration of basal cells, dilated lymph capillaries and dense lymphoid cell infiltration in the middermis were prominent findings after treatment.     

Acral bullous lichen sclerosus intolerant to UVA‐1 successfully treated with narrowband ultraviolet B phototherapy

Lichen sclerosus (LS) is an uncommon, chronic, lymphocyte‐mediated, inflammatory dermatosis characterized by ivory‐white patches with scar‐like atrophy. Extragenital bullous lichen sclerosus may rarely affect palms and soles, causing severe pain and substantially impairing quality of life. We present the first case of acral bullous lichen sclerosus intolerant to UVA‐1 phototherapy successfully treated with low doses of narrowband ultraviolet B phototherapy.     

Tumor necrosis factor-α inhibitor-induced morphea and psoriasiform dermatitis in a pediatric patient with Crohn's disease

Tumor necrosis factor-alpha inhibitor therapy for inflammatory bowel disease may be associated with paradoxical cutaneous adverse events, most commonly psoriasiform eruptions. We present the case of a pediatric female patient with Crohn's disease who developed multiple concurrent cutaneous eruptions while on infliximab treatment, including morphea, psoriasiform dermatitis, and genital lichen sclerosus. Although refractory to skin-directed treatments, all three conditions resolved upon discontinuation of infliximab, supporting their development as a paradoxical reaction to infliximab therapy.     

非生殖器部位特殊形态硬化性萎缩性苔藓1例

报道1例非生殖器部位特殊形态硬化性萎缩性苔藓，并经病理检查确诊。患者男，36岁。躯干、四肢群集分布白色皮疹1年余。皮疹瓷白色，扁平圆形或蝶形，可见小的粉刺样毛囊性角质栓，四周绕以红晕。予0．05％丙酸氯倍他索软膏和0．1％维胺酯霜外用，初显疗效。     

Coexistence of Generalized Morphea with Histological Changes in Lichen Sclerosus et Atrophicus and Lichen Planus

We reported a 44-year-old Japanese woman with generalized multiple sclerotic plaques, which showed hisological findings of morphea. This patient also had an erosive lesion on her mouth; its histological findings were consistent with lichen planus. A sclerotic lesion on her thigh showed the histological findings of lichen sclerosus et atrophicus (LSA). These data suggest that similar etiologic events or closely related pathologic processes are involved in morphea, lichen planus, and LSA.     

硬化萎缩性苔藓合并局限性硬皮病一例

患者男,44岁.躯干、四肢皮肤泛发硬化萎缩斑片进行性加重8年,自觉瘙痒.检查见皮损有色素沉着和色素减退,触之有皮革样硬度.口腔黏膜、肛门、外生殖器未见皮损,不伴系统损害,常规实验室检查无异常发现.组织病理显示,表皮角化过度,萎缩变薄,基底细胞液化变性,真皮乳头层见纯一化变性带.真皮中下层胶原纤维增生、致密、红染,轻度玻璃样变.诊断为硬化萎缩性苔藓合并局限性硬皮病.     

Acrosyringeal variant of extragenital lichen sclerosus et atrophicus

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis of the anogenital area, and approximately 15% to 20% of patients with LSA have extragenital lesions. Here, we report the case of an 18-year-old Korean man presenting with multiple asymptomatic punctated hypopigmented atrophic macules on the dorsa of both feet. Dermoscopic examination revealed hypopigmented atrophic macules with several central keratotic plugs. The histopathologic findings indicated LSA but were confined to the acrosyringium. Based on the clinical and histopathological findings, the patient was diagnosed with an acrosyringeal variant of extragenital LSA. The patient in this case showed a unique histopathological finding in which the typical features of LSA were confined to the acrosyringium, as well as an unusual clinical presentation of non-coalescing atrophic punctate macules on the dorsum of the feet.     

Chronic White Plaque of the Vulva in Postmenopausal Women

We report 13 cases of white lesions of the vulva in postmenopausal women. Nine cases had clinical and histological features typical of lichen sclerosus et atrophicus (LSA). Two cases exhibited mild histological changes suggestive of LSA. Two cases had normal-appearing histology without significant histological changes. Considering the relatively short duration of the disease and relatively small lesions in the last two cases, they may be considered to represent the initial stage of LSA.     

Diagnosis and treatment of oral lichen sclerosus in a pediatric patient

Lichen sclerosus is an uncommon chronic inflammatory disease, which rarely affects the oral mucosa. Here, we describe the occurrence of oral lichen sclerosus with gingival destruction in a 12‐year‐old female patient. After diagnostic confirmation by histopathology, intralesional injection of corticosteroid was performed, producing satisfactory remission of the lesion.     

Ultraviolet A1 phototherapy

Long-wavelength ultraviolet A (340-400 nm; UVA1) therapy is currently available in only a few dermatology departments. Equipment capable of delivering this waveband has been available since 1981, but it is only over the past decade that increasing numbers of studies assessing the potential of this as a therapy have been published. High-dose UVA1, which requires expensive and space-occupying apparatus, is effective as a monotherapy for acute flares of atopic dermatitis, but it has not yet been formally assessed as an adjunct, rather than as an alternative to conventional therapies including potent and very potent topical corticosteroids. Low-dose (which can be administered using a standard phototherapy cubicle fitted with appropriate lamps) and medium-dose UVA1 may be less effective for this indication. Another condition for which UVA1 is effective, and is particularly promising because we have no reliably effective treatment already, is localized scleroderma. It also appears to be effective in systemic lupus erythematosus (although it is not yet clear when it is indicated, and its safety needs to be assessed in more patients) and in polymorphic light eruption (although there have been no studies suggesting that UVA1 will have any advantages over standard prophylactic phototherapies). Open studies and case series suggest that UVA1 may prove beneficial for various other diseases, including cutaneous T-cell lymphoma, lichen sclerosus, keloids, systemic sclerosis and hand dermatitis. In the centres where it is available, UVA1 has already proved a useful addition to the range of phototherapies previously available. However, much more research is needed to confirm its efficacy for many of its potential indications, and to determine when and how it should be used.     

伴发血疱的泛发性硬化性萎缩性苔藓1例

患者女，64岁，四肢，躯干泛发性羊皮纸样萎缩性斑片5年，双乳内下方和剑突处起血疱1个月，组织病理检查：非血疱处皮损符合典型的硬化性萎缩性苔藓的病理改变，血疱处皮损示表皮下大疱形成，疱液中有大量的红细胞。     

Extracellular matrix protein 1 inhibits the activity of matrix metalloproteinase 9 through high-affinity protein/protein interactions

Extracellular matrix protein 1 (ECM1), an approximately 85-kDa glycoprotein with broad tissue distribution, harbors mutations in lipoid proteinosis (LP), a heritable disease characterized by reduplication of basement membranes and hyalinization of dermis, associated with neurologic disorders. The mechanisms leading from ECM1 mutations to LP phenotype are unknown. In this study, we explored ECM1 protein-protein interactions utilizing yeast two-hybrid genetic screen of human placental library, which identified nine interacting proteins, including matrix metalloproteinase 9 (MMP9). The interactions were confirmed by beta-galactosidase assay with isolated clones and by co-immunoprecipitation which narrowed the interacting segment in ECM1 to the C-terminal tandem repeat 2 (amino acids 236-361). This peptide segment also inhibited MMP9 activity in a gelatin-based ELISA assay. We propose that ECM1-mediated reduction in MMP9 proteolytic activity may have relevance to pathogenesis of LP.