局限于腹部的丘疹型皮肤淀粉样变性

报告1例局限于腹部的丘疹型皮肤淀粉样变性。患者女,35岁。因下腹部丘疹伴瘙痒1年就诊。皮损组织病理检查示真皮乳头可见团块状均质淡红染物质。红染物质PAS和刚果红染色均阳性。免疫组化示细胞角蛋白34βE12和CK5/6在淀粉蛋白团块中均呈阳性表达,AE1/E3和CK10/13在淀粉蛋白团块中均呈阴性表达。诊断:丘疹型皮肤淀粉样变性,根据免疫组化结果提示淀粉样蛋白主要来源于基底层凋亡的角质细胞。     

苔藓样和斑状皮肤淀粉样变的临床病理和免疫组化研究

Clinicopathologicalandimmunohistochemicalstudyonlichenoidandmacularamyloidosis皮肤淀粉样变是以淀粉样蛋白沉积于皮肤为特征的疾病。结节性皮肤淀粉样变的淀粉样变蛋白是由局部浆细胞产生的’‘，属于免疫球蛋白人轻链物质‘’‘。而苔药样和斑状皮肤淀粉样变的淀粉样蛋白的组织来源仍有争议”。我们用抗角蛋白和抗波形蛋白抗体进行免疫组化染色，对淀粉样蛋白的来源进行了探讨。l病例、材料和方法1．l标本：选择199o－1996年，临床表现典型的原发局限性皮肤淀粉样变病例的石蜡包理组织ZO份。其中男12例，女8例；年龄19－68岁，平均…     

16例皮肤淀粉样变临床病理分析

淀粉样蛋白(amyloid)在皮肤组织内沉积压,称为淀粉样变性。本文报导16例皮肤淀粉样变。其中苔藓样型8例,斑状型7例;结节型1例。因本病常有瘙痒和色素沉着,早期临床上与扁平苔藓,痒疹以及一些色素性皮肤病难以区别。为了提高对本病的认识,现对16例原发性皮肤淀粉样变进行临床病理分析。 材料和方法 1.临床资料:16例皮肤淀粉样变均选自本院门诊患者。其中40—49岁2例;50—59岁8     

内分泌、代射、营养障碍性皮肤病

20 0 3 0 75 4　原发性皮肤淀粉样变 1 0 9例临床和病理分析 /刘小军 (浙江金华铁路医院皮肤科 )…∥皮肤病与性病 . 2 0 0 2 ,2 4 (4) . 1均为男性 ,苔藓样型 5 2例 (47.7% ) ,斑状型2 8例 (2 5 .7% ) ,混合型 2 0例 (1 8.3 % ) ,皮肤异色病样型 4例 (3 .7% )。组织病理共同特征为表皮角化过度 ,棘层肥厚 ,真皮乳头层有淡红色均匀一致的淀粉样物质沉积 ,多位于真皮乳头内 ,淀粉样蛋白正上方基底细胞可液化变性 ,色素失禁。结晶紫染色 ,真皮乳头层均有紫红色的团块物。各型尚有其特征。在讨论中指出本病发病部位不定 ,皮疹类型变化多端 ,注…     

内分泌、代谢、营养障碍性皮肤病

962032 苔藓样型皮肤淀粉样变的免疫组化和超微结构研究/汪晨…//中华皮肤科杂志。-1996,29(1).-55～56 对9例苔藓样型皮肤淀粉样变(PLCA)采用免疫组化和电镜观察的方法对淀粉样蛋白的来源进行了研究。皮肤病理示角朊细胞不同程度的退化变性,细胞样小体形成及真皮层淀粉样蛋白沉积。电镜下见淀粉样蛋白微丝直径约6nm×10nm,认为淀粉样蛋白微丝是     

类似白癜风的淀粉样变

名古屋大学皮肤科在过去5年中,共发现78例皮肤淀粉样蛋白沉着病例,其中9例(11.5%)伴有白斑,8例是原发性皮肤局限性淀粉样变,1例是由天疱疮继发的全身性淀粉样变.在白斑部也有淀粉样蛋白沉着.男4例,女5例.白斑部位为头顶部2例、背部3例,脸、肘、下肢外侧、足背部各1例.所有病例在其他部位均伴有斑状和苔藓样淀粉样变.白斑形状不规则,特别是头顶部2例,为点状融合性白斑,周围有小白点,白斑内有点状及岛状色素沉着,     

在福尔马林固定、石蜡包埋切片中用抗角蛋白抗体对淀粉样蛋白进行免疫组化特性的研究

作者采用福尔马林固定,石蜡包埋切片,用抗角蛋白抗体对原发限局性皮肤淀粉样变(AD)及其他类型淀粉样变中的淀粉样蛋白进行免疫组化研究。选择9例原发限局性皮肤淀粉样变,8例系统性淀粉样变和5名正常人皮肤标本以及3种抗体:单克隆抗角蛋白抗体EAB—903和EAB—904,以及二种单克隆抗角蛋白抗体的混合抗体MAK—6,用ABC方法进行染色。并用连续切片Dylon染色以证实在这些切片上有淀粉样蛋白的存在。     

皮肤淀粉样变中淀粉样蛋白4种染色法的比较

目的对4种特殊染色法显示淀粉样蛋白的优劣进行对比分析,为原发性皮肤淀粉样变探寻更好的组织病理学诊断方法。方法将本院皮肤科近10年确诊为原发性皮肤淀粉样变的33例皮损组织石蜡标本重新切片后分别进行刚果红、甲基紫、结晶紫和过碘酸雪夫氏反应(PAS)4种特殊染色,显微镜下观察组织切片中淀粉样蛋白的染色情况。结果33例标本中的淀粉样蛋白经4种特殊染色后均呈阳性着色;刚果红、甲基紫、结晶紫和PAS分别将淀粉样蛋白染成砖红色、蓝紫色、紫色和紫红色;前3种方法的染色效果不稳定,部分切片中可见淀粉样蛋白与周围组织分辨不清;而在所有PAS染色切片中,淀粉样蛋白与周围组织的颜色对比明显,但其特异性不强。结论在选择确诊原发性皮肤淀粉样变的特染方法时,PAS染色结合其他3种染色中的任何一种能够弥补单一方法的不足。     

带状疱疹样皮肤转移癌1例

患者男,78岁。胸部丘疹、结节伴疼痛1月余。查体见以左胸部为主的散在或簇集的丘疹、结节,似带状分布,后背部未累及。皮损组织病理示:肿瘤组织位于真皮中上层,肿瘤细胞呈立方形,具异型性,可见病理性核分裂,呈灶状分布,有的细胞空泡化,有的肿瘤团块中腺样结构形成。CK7(+),CK20(-),AE1/AE3(+),CK5/6(-)。诊断:皮肤转移性腺样鳞癌。     

内分泌、代谢、营养障碍性皮肤病

960616 皮肤淀粉样变表皮中胶样小体的研究/张美华…//临床皮肤科杂志。-1995,24(5).-296～298 55例诊断为局限性皮肤淀粉样变中50例为苔藓样淀粉样变,5例为斑状淀粉样变,所有病例皮损活检镜下见真皮乳头部均有淡嗜伊红性均匀一致无定形物质,并经结晶紫染色证实为淀粉样物质。结果除4例苔藓样型、1例斑状淀粉样变表皮内胶样小体阴性外,其余50例表皮均可见胶样小体。4例斑状型胶样小体数共14个,平均每个标本3.5个,46例苔藓样型胶样小体总数728个,平均每个标本15.82个。结果给组织病理诊断提供一个线索,即可以在表皮内有胶样小体或较多胶样小体的下方真皮乳头内寻找淀粉样蛋白,表皮内胶样小体也可以作为病理上诊断皮肤淀粉样变一个辅助条件。表1参8     

Cytokeratin expression in lichen amyloidosus and macular amyloidosis

AIM: To understand the role of epidermal cells in the pathogenesis of lichen amyloidosus (LA) and macular amyloidosis (MA). METHODS: We carried out immunohistochemical investigations on cytokeratins (CKs) in amyloid deposits in formalin-fixed and paraffin-embedded tissue specimens from eight persons with LA and 12 with MA. The primary antibodies of CK1-8 (AE3), CK10 (DEK-10), CK14 (LL002), CK17 (E3), CK18 (DC10), CK19 (KS19.1), CK5/6/18 (LP34) and CK8/18 (5D3) were used in the study. RESULTS: In amyloid deposits, immunoreactivity with only two monoclonal antibodies (CK1-8 and CK5/6/18) was observed in 14 cases (eight LA and six MA), confirming the hypothesis that epidermal cells participate in amyloid formation of LA and MA. COMMENTS: All of the CKs detected in amyloid deposits were basic type (type II). It seems plausible either that acidic CKs might be degraded faster than basic types in amyloidogenesis or that paraffin-embedded tissue specimens are less sensitive than frozen tissue sections. The results of our study suggest that when paraffin-embedded specimens are investigated by immunohistochemical methods, CK5 antibody is useful in the diagnosis of LA and MA.     

Two different pathogenetic pathways in lichen amyloidosus and macular amyloidosis

Summary In lichen amyloidosus (LA) and macular amyloidosis (MA), small amyloid deposits occur in the upper papillary dermis. Previous electron-microscopic studies have indicated an epidermal origin of the amyloid, where degenerating keratinocytes drop into the dermis and undergo transformation to amyloid. While this mechanism seems possible at least in MA, we suggest an alternative pathogenetic pathway in LA, in which amyloid fibrils seem to form on the dermal surface of living basal keratinocytes. It is possible that the different morphology of the amyloid in LA and MA is explained by partially different pathogenetic mechanisms although the amyloid in both conditions may be chemically closely related.     

Immunofluorescence and histochemical studies of localized cutaneous amyloidosis

Lichen amyloidosus (LA) and macular amyloidosis (MA) are two forms of localized cutaneous amyloidosis in which the amyloid occurs as larger and smaller deposits respectively in the papillary dermis. The histogenesis of the amyloid of these conditions is unknown. By using an indirect immunofluarescence technique we showed that LA and MA do not react with antibodies against different previously characterized amyloid fibril proteins. These results indicate that the amyloid of LA and MA is different from other known types of amyloid. Protein AP, which was demonstrated in amyloid of MA and LA, is known to be present in all forms of amyloid and is of unknown significance. Antiserum against keratin did not react with the larger homogeneous amyloid bodies, but showed a weak reaction with some small deposits. Histochemical staining failed to show keratin in any of the tissues containing LA or MA.     

Immunohistochemical studies on fibrillin in amyloidosis, lichen ruber planus and porphyria.

The pathogenesis of macular amyloidosis and lichen amyloidosis remains unsolved and the primary amyloid fibril protein(s) has not yet been identified. Ultrastructural association of skin amyloid with elastin associated microfibrils has been noted earlier. The presence of fibrillin in conjunction with such microfibrils was recently demonstrated immunohistochemically. The presence of fibrillin immunoreactivity in the amyloid deposits in skin biopsies from 3 patients with macular amyloidosis and 3 patients with lichen amyloidosis was studied, using monoclonal anti-fibrillin antibodies. For comparison, skin specimens were studied from five patients with lichen ruber planus, four patients with erythropoietic protoporphyria and from a patient with myeloma-associated cutaneous amyloidosis. Renal specimens from two cases of the amyloid A type of renal amyloidosis also were investigated. There was no immunostaining either of the keratin bodies in specimens of lichen ruber planus, the cutaneous PAS-positive vascular deposits in patients with erythropoietic protoporphyria, or the amyloid deposits in specimens of systemic amyloidosis and it was faint or absent in amyloid deposits in the specimens from patients with lichen amyloidosis. In contrast, distinct fibrillin immunoreactivity could be demonstrated in amyloid deposits in specimens from patients with macular amyloidosis. It was sometimes absent in deposits located in the upper part of the papillary dermis, close to the dermal epidermal junction zone, while consistently strong in deposits located lower down in the dermis. The results suggest that fibrillin or part of the fibrillin molecule may be present in some of the amyloid deposits in specimens of macular amyloidosis.     

Primary localized cutaneous amyloidosis: a clinicopathologic study from Saudi Arabia

Background Primary localized cutaneous amyloidosis (PLCA) refers to deposition of amyloid in apparently previously normal skin with no evidence of deposits in internal organs. Certain ethnic groups are particularly predisposed. The objective of this study was to investigate the occurrence of PLCA in a hospital-based set-up and to review the clinicopathologic findings of histologically confirmed cases. Methods Methods included a retrospective evaluation of the records and paraffin sections of skin biopsies of all patients, diagnosed clinically as cutaneous amyloidosis in a tertiary care teaching hospital during the period 1987–95. Results Twenty-one out of 42 suspected cases were histologically confirmed as PLCA; the total number of biopsied patients during the whole period was 920. Eleven were cases of lichen amyloidosis (LA) and 10 were of macular amyloidosis (MA). All were adults, and women dominated in MA. The mean duration of symptoms was shorter for the latter subtype. Histologically epidermal changes were the main differentiating feature between LA and MA. Conclusions PLCA is a rare chronic progressive skin disorder affecting adults, with a prevalence of 0.15 among patients attending the dermatology clinic in a tertiary care teaching hospital in Saudi Arabia. There were few differences in demographic profile or histochemical characteristics between LA and MA. Meticulous histologic examination of sections and even sequential biopsies may be needed to confirm the diagnosis in clinically suspected cases.     

Thermosensitive lichen amyloidosis

BACKGROUND: A 26-year-old male presented with a 3-year history of lichen amyloidosis. On examination, there was a pigmented papular eruption with a ripple pattern affecting the limbs and trunk but sparing the axillae, antecubital and popliteal fossae, central chest, neck and face. There was also prominent sparing of the skin overlying the superficial veins of the limbs. The sparing of the superficial veins of the limbs by lichen amyloidosis raised the possible role of cutaneous temperature in governing the distribution of amyloid deposits in our patient. OBSERVATIONS: Total body infrared thermography demonstrated consistent sparing of the amyloid deposits in areas with higher cutaneous temperatures such as the neck and axillae as well as the course of the superficial veins. The cooler areas such as the extensor surfaces of the arms and legs corresponded to areas of amyloid deposition. Narrow band ultraviolet B (NBUVB) phototherapy over a 5-month period resulted in a marked improvement of pruritus and clearing of the amyloid deposits. CONCLUSIONS: Our patient clearly demonstrated lichen amyloidosis in a thermosensitive distribution. This may be a gross manifestation of previous reports of in vitro thermosensitivity of amyloid fibril formation and may have potential implications in treatment at least in a subset of patients demonstrating this clinical feature.     

Successful treatment of lichen amyloidosis with combined bath PUVA photochemotherapy and oral acitretin

Lichen amyloidosis (LA) is a chronic, pruritic skin disorder characterized by brownish-grey papules on extensor surfaces of legs and rarely on the trunk. Thioflavin T-positive amyloid deposits are found in the papillary dermis of affected skin, which is the only organ involved. A variety of therapeutic regimens for lichen amyloidosis have been described; however, in many cases with only limited effect. We report on two patients with lichen amyloidosis with typical clinical symptoms not responding to local treatment. A combined regimen with bath psoralen ultraviolet A (PUVA) and oral acitretin was initiated, resulting in nearly complete resolution of the papules and impressive relief from the severe pruritus. The beneficial response has persisted for 8 months. The suggested combined therapy with bath PUVA photochemotherapy and oral acitretin represents an efficacious and practical treatment modality for lichen amyloidosis with long-lasting effects.     

Role of colchicine in primary localised cutaneous amyloidosis

Fifteen patients with primary localised cutaneous amyloidosis (PLCA), of which 8 had macular amyloidosis (MA) and 7 lichen amyloidosis (LA), received oral colchicine 1 mg/day in 2 divided doses for a period of 3 months. Pruritus completely disappeared in all MA patients and 30-60% diminution occurred in LA patients within 15 days. Flattening of the papules and diminution in thickness of the skin also started within one month in all LA patients, within 90 days of therapy pigmentation almost disappeared in ail patients of MA and size of papuies decreased by 80-98% in all LA patients. No significant side effect was seen in these patients due to colchicine therapy.     

Cytokeratins in primary cutaneous amyloidosis

The expression of keratins was investigated immunohistochemically on formalin-fixed and snap-frozen primary cutaneous amyloidosis tissue with a panel of monospecific and polyspecific antikeratin antibodies which recognized keratins K1, K5, K6, K7, K8, K10, K14, K16, K17, K18, and K19. Amyloid deposits in frozen section of seven cases of macular amyloidosis and lichen amyloidosus always reacted with antibodies LP34 (labeling K5, K6 and K18) MNF (labeling K5, K6, K8, K10, K17 and K18) and RCK 102 (labeling K5 and K8); frozen section in one case each of the seven cases also reacted with antibodies LL001 (labeling K14), Lp1K (labeling K7 and K17), and LP2K (labeling K19), LP1K (labelling K7 and K17), and LP2K (labelling K19), In formalin fixed section of 13 cases of macular amyloidosis and lichen amyloidosus amyloid deposits were labeled with LP34 in three section LL020 ()labelling keratins K5 and K6) in one section and LP2K in two section. In nodular primary cutaneous amyloidosis amyloid deposits were not labeled with any antikeratin antibodies. These data confirm that amyloid in macular amyloidosis and lichen amyloidosus contains keratin epitopes, and suggests derivation of the fibrillar component from keratin intermediate filaments Several different keratins appear to undergo conversion to amyloid, LP34, MNF 116 and RCK 102 antibodies, which have in common the labelling of keratin K5, may be useful in the diagnosis of macular and popular amyloidosis with frozen tissue section.     

Intermittent use of topical dimethyl sulfoxide in macular and papular amyloidosis

Background Severe and therapy-resistant pruritus is the most prominent feature of macular (MA) and lichen (LA) amyloidosis that leads to further amyloid deposition by recurrent frictional trauma to the epidermis. Of the various therapeutic modalities with variable success, the most encouraging and beneficial effect has been observed with topical dimethyl sulfoxide (DMSO) therapy. In a previous study, we achieved marked clinical improvement in nine of 10 patients in a daily treatment regimen over 6–20 weeks, but relapses occurred in the post-treatment follow-up period. The aims of this study are to investigate whether the patients would benefit from intermittent therapy and to determine the optimal application interval of DMSO to maintain the relief of symptoms. Methods Thirteen patients with histopathologically verified cutaneous amyloidosis (five MA, two LA and six biphasic) were enrolled in the study. They were treated once daily with a 50 or 100% DMSO solution until pruritus disappeared. Then, DMSO was applied at increasing intervals until the widest effective application interval for maintenance of relief was reached. Patients were regularly followed-up by a scoring system for pruritus, papules, and pigmentation, control biopsies, photographs, blood biochemistry, and side-effects. Results The mean time required for the disappearance of pruritus was 4.1 weeks. Remarkable flattening of the papules was achieved after an average therapy period of 9 weeks. After a total therapy period of 6.5 months, a nearly 50% remission in pigmentation and >70% flattening of papules were achieved. The widest effective DMSO application interval was 8.6 days. The side-effects of therapy were contact urticaria, desquamation, burning sensation, and garlic-like breath odor, which were more prominent with the higher concentration of DMSO. In interval therapy, side-effects were tolerated more easily than in daily therapy. No reduction of amyloid deposits was revealed in control biopsies. Conclusions Locally applied DMSO can break the vicious “pruritus–amyloid deposition–pruritus” cycle in patients with MA and LA. In addition to its daily use, interval therapy seems to maintain this effect and enables patients to tolerate side-effects more easily.