A case of generalized morphea with a high titer of anti-Borrelia burgdorferi antibodies

A 69-year-old male had noticed pruritus on the back for the previous 3-4 years and cutaneous sclerosis with swelling of the dorsum of the neck had developed in the last one and a half years. However, he had never complained of Raynaud's phenomenon of the fingers, dry mouth, or dry eyes. At this first visit to our hospital, he complained of erythematous cutaneous sclerosis with swelling of the dorsum of the neck. Histopathological findings biopsied from the neck showed epidermal hyperplasia with elongation of rete ridges and homogeneous and fibromatous changes of the dermis with dense perivascular cell infiltration consisting of mononuclear cells or lymphocytes with several nests of incontinentia pigmenti. However, there were no sclerotic changes in blood vessels in the upper dermis biopsied from the forearm skin, although slightly homogeneous and fibromatous changes of the dermis were seen. In the clinical course, the cutaneous sclerotic change enlarged to extend to the bottom of the cheek, forearm, and lower legs. These clinical features and histopathological findings led to the diagnosis of generalized morphea. Hematologic examination showed positive anti-Borrelia burgdorferi IgM antibodies, although there were no positive anti-Borrelia burgdorferi IgG antibodies. These results revealed that there can be a close association of localized scleroderma with Borrelia burgdorferi and that generalized morphea may also represent a Borrelia infection.     

Unilateral generalized morphea in childhood

We report a 6-year-old boy with unilateral generalized morphea distributing on the right side of his lower leg, trunk, and upper arm. A skin biopsy from the right thigh showed accumulation of thick collagen bundles extending from the middle dermis to the subcutaneous fat tissue. The levels of antinuclear antibodies, rheumatoid factor, and anti single-stranded DNA antibody were elevated. No severe deformity or functional disabilities were noted. With topical corticosteroid therapy, the sclerotic skin became gradually softer, and no progression of sclerosis has been noted for one year.     

Eosinophilic fasciitis associated with generalized morphea and IgA nephropathy

Eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by increased peripheral blood eosinophils and diffuse fasciitis, generalized morphea (GM) is a subtype of localized scleroderma, and IgA nephropathy is a chronic glomerulonephritis caused by abnormal deposition of IgA in the mesangial area of the glomeruli. We describe a 49‐year‐old male patient with hard skin, cutaneous hyperpigmentation, and proteinuria. The patient had suffered from a long disease course of hard skin, while urine protein was newly detected. Finally, the clinical presentation and physical examination, limb MRI, skin biopsy, and renal biopsy confirmed the diagnosis of eosinophilic fasciitis associated with generalized morphea and IgA nephropathy. This case is the first report of EF associated with GM and IgA nephropathy.     

A case of lipodystrophia centrifugalis abdominalis infantilis with morphea.

A 5-year-old female developed three depressed lesions sequentially on her left hip, left upper arm and left Achilles tendon; we diagnosed them as lipodystrophia centrifugalis abdominalis infantilis (LCAI). She also developed two sclerotic lesions, at almost the same time, on her left upper arm and left forearm; we diagnosed them as morphea. Clinical and histopathological examination revealed that the LCAI and morphea seen in our case were completely different. Although two other cases of co-existing LCAI and morphea have already been reported, they were not described in detail and their morphea lesions were adjacent to their LCAI lesions. We herein report a case of LCAI with morphea in which the lesions were not adjacent.     

Palindromic morphea: multiple recurrence of morphea lesions in a case of systemic sclerosis.

We report an unusual scleroderma case. A 45-year-old woman had recurrent morphea lesions over 10 times in 6 years. She had had preceding inactive systemic scleroderma. New morphea lesions developed cyclically on various portions of her body and improved within 2 years. Interestingly, new lesions have developed on once involved skin as well as uninvolved skin. No exacerbation of systemic scleroderma was induced by outbreaks of new morphea lesions. We could not find a similar case in the literature and named it "Palindromic morphea" because of its unique clinical course. D-penicillamine treatment had a limited effect. Minimal oral prednisolone (5 mg/day) completely suppressed the multiple recurrence of the morphea lesions and enhanced improvement of the sclerosis.     

Generalized morphea and primary biliary cirrhosis coexisting in a male patient

We present further evidence that generalized morphea (GM) and primary biliary cirrhosis (PBC) may be associated. As far as we know, only six cases with this association have been previously reported in the published work, all of which were observed in women. We describe the case of a 62-year-old man diagnosed with M2-antibody-positive PBC who developed multiple generalized indurated plaques on the trunk and extremities 3 years later. Clinical history, laboratory data and histopathological examination were consistent with the diagnosis of GM. The coexistence in a male of these two entities that predominantly affect females reinforces the hypothesis that a pathogenic link exists between GM and PBC. Consequently, PBC should be looked for in all patients with GM.     

Ultrasonography as an auxiliary diagnostic tool for morphea profunda: A case report

Morphea profunda is a rare subtype of localized scleroderma and it is difficult to evaluate the conditions of sclerotic changes at an early stage. Studies using ultrasonography to evaluate localized scleroderma are limited and, to date, the characteristic findings of morphea profunda assessed by ultrasonography have never been reported. Here, we present a case of morphea profunda diagnosed with the assistance of ultrasonography. A 69‐year‐old Japanese woman with a past history of morphea en plaque on her lower abdomen presented with skin indurations of her bilateral lower back and thighs. To evaluate the stiffness of the subcutis, fascia and muscle, we utilized ultrasonography and found an unexpected hyperechogenicity not only of the dermis but also in the deeper tissue. The diagnosis was revised to morphea profunda after we performed a deep skin biopsy, including the muscle tissue. From this case, we assert that ultrasonography is a useful alternative tool to assist in the differential diagnosis of morphea profunda.     

泛发性大疱性硬皮病一例

患者男,51岁。因背部、上肢、指端皮肤变硬、结痂、萎缩伴疼痛5年于2015年1月就诊。2009年6月起患者自觉肩背部、上肢出现多发性片状水肿性斑块,皮肤较硬,轻度萎缩,后在上述部位间断出现水疱、大疱,疱壁较厚,周围有轻度红肿,水疱很快破溃干涸,形成较厚的黑色痂皮,部分形成深在性溃疡,自觉疼痛剧烈,就诊于当地多家医院,诊断不详……     

Discordance of pediatric morphea treatment by pediatric dermatologists

Background/Objectives

Studies describing treatment efficacy in pediatric morphea are lacking. Subspecialists have reached no consensus on how to optimally treat pediatric morphea. The objective of the current study was to describe the most common treatment practices of pediatric dermatologists worldwide who care for children with morphea.

Methods

A survey regarding topical treatment practices of pediatric morphea, with representative case‐based scenarios, was distributed to pediatric dermatologists and results were tallied.

Results

The survey response rate was 13.4%, with 110 respondents in the final analysis. The majority of respondents agreed on red violaceous rim (99%), increased local warmth (75%), raised borders (69%), and dermal thickening (64%) as signs of disease activity. Respondents had less agreement on sclerotic lesions (41%), scaling (43%), dyspigmentation (19%), and atrophy (13%) as signs of disease activity. Ninety‐two percent of respondents used primary therapy or monotherapy with topical medications, including 45% in linear morphea of the limbs and 37% in linear morphea of the head or neck. High‐potency topical corticosteroids were most commonly used (80%), although respondents did not agree on specific regimens. Sixteen different treatment regimens were selected as first‐line therapy for one case scenario of active disease.

Conclusion

The survey found large variation in how pediatric dermatologists treat pediatric morphea. Consensus treatment guidelines developed by pediatric dermatologists and pediatric rheumatologists are urgently needed regarding the efficacy of therapies for pediatric morphea. Prospective studies of treatment efficacy in pediatric morphea are urgently needed as well.     

硬化性苔藓合并扁平苔藓、硬斑病一例并文献复习

硬化性苔藓(LS),扁平苔藓(LP)和硬斑病是三种病因不明的皮肤病,已有多篇任意两种疾病合并的报道,但患者同患LS、LP和硬斑病少见,目前未见国内有相关报道。本文报道1例LS合并LP和硬斑病,并对国外已报道的7例病例进行回顾性分析。结果示8例患者中男2例,女6例,平均年龄(60.3±15.1)岁。8例患者的LS与硬斑病同时发生或硬斑病发病早于LS,同一皮损的病理切片同时具有LS与硬斑病的特征,LP发病可早于或晚于LS和硬斑病;8例患者皮损主要表现为生殖器外LS、泛发型硬斑病、经典或糜烂型LP;6例有免疫相关异常指标,4例合并其他自身免疫性疾病。     

A Case of CREST Syndrome Associated with Sick Sinus Syndrome

Cardiac manifestations of the CREST syndrome or limited systemic sclerosis (ISSc) are very rare. We report a case of CREST syndrome associated with sick sinus syndrome. Histopathology of cardiac muscle revealed fibrotic changes, suggesting that such changes may be pathogenetically related to CREST syndrome.     

《最新皮肤科药物手册》邮寄及征文和招生信息

一、邮书:①马振友主编《最新皮肤科药物手册》88元,②《国际皮肤病分类与名称》766元,③《皮肤美容化妆品制剂手册》50元,④《中华皮肤性病学博览》30元,⑤《尖锐湿疣》20元;⑥徐汉卿主编《微量元素与皮肤病》168元;⑦朱文元,倪容之主编《疑难皮肤病彩色图谱》238元;⑧谭升顺主编《皮肤病治疗经验荟萃》78元。     

积雪甙治疗泛发性硬斑病1例报告

报告1例18岁男性泛发性硬斑病，病史4年，表现为两上臂，躯干及右大腿10余处硬性斑片，伴色素改变，曾用复方丹参片，维生素E治疗无效，予以积雪甙片口服及积雪甙乳膏外用，治疗3个月后，临床及组织病理均显著改善，治疗半年后，皮疹基本消退。     

念珠状发伴全身性毛囊性丘疹一例

[摘要]报告伴全身毛囊性丘疹的念珠状发1例。患者女,20岁,脱发、睫毛脱落伴全身丘疹20年。头发稀疏无光泽,散在分布念珠状发;睫毛短,可见间断的折断睫毛。全身皮肤干燥,项背部及四肢伸侧为著全身毛囊性丘疹。头发光镜检查：发干间断性缩窄,宽部呈梭形,梭形结节间隔不规则。头部皮肤病理示：真皮层毛囊体积减小,分布稀疏。临床表现、头发光镜检查及皮肤病理检查符合念珠状发。     

皮肤窦性组织细胞增生症

报告1例皮肤窦性组织细胞增生症（CSH）。患者女,48岁,仅表现为左面颊部有一斑块。皮损的组织病理特征为真皮及皮下有以组织细胞和淋巴细胞为主的浸润,可见吞噬现象。免疫组化染色组织细胞S-100蛋白和CD68阳性,CD1a标记阴性。阿维A试验性治疗有效。     

Presenting characteristics and progression of pediatric-onset morphea: Interim analysis of a prospective registry

Morphea is a rare fibrosing disorder with a highly variable disease course, which can complicate management. Here, we present a prospective cohort study describing the current treatments used in the management of pediatric-onset morphea and assessing responses to systemic and topical therapies. Most patients demonstrated inactive disease by 1 year, regardless of treatment, though recurrences were common in our cohort overall (39%). Our results support the need for continuous monitoring of all children with morphea following the completion of treatment, including topical treatment, due to high rates of disease relapse.     

青少年与成人皮肌炎/多发性肌炎的比较分析

目的：探讨青少年皮肌炎/多发性肌炎（JDM/PM）的临床表现和预后特点。方法：比较分析25例JDM/PM与143例成人皮肌炎/多发性肌炎（DM/PM）的临床表现、辅助检查结果及预后情况。结果：与成人DM/PM相比,JDM/PM男多于女;初发症状中肌痛和体重下降较少见,临床表现中光敏较多见,较少出现肌痛、咽喉部肌群受累、消瘦和肺、胸膜受累;未见并发恶性肿瘤和死亡;血、尿常规,免疫学,血清酶,肌电图及肌活检结果等则与成人无统计学差异。结论：JDM/PM具有一定的临床表现特征,肺、胸膜较少累及,预后较好。     

Linear scleroderma with prominent multiple lymphadenopathy followed by the development of polymyositis: A case report and review of published work

Localized scleroderma is an inflammatory disorder affecting the skin and underlying tissues, a certain subset of which develops other autoimmune diseases on the basis of a prominent autoimmune background. We here report a unique case of linear scleroderma presenting with a sclerotic plaque on the left thigh, multiple lymphadenopathy in bilateral inguinal and para‐aortic lymph nodes, and hepatosplenomegaly, who later developed polymyositis. We describe the detailed disease course of our case and discuss the clinical significance of multiple lymphadenopathy in localized scleroderma based on a review of published work.