网状红斑性黏蛋白沉积症一例

报告1例网状红斑性黏蛋白沉积症，患者以胸背部的持久性光加重性网状红斑为主要表现，伴有点片状表面萎缩的白斑和坏死结痂，不伴系统损害，常规实验室检台无异常发现。组织病理显示．真皮有单核细胞浸润和阿辛蓝染色阳性的黏蛋白沉积.根据患者皮损的临床病理特征、组织病理及阿辛蓝染色等．诊断符合网状红斑性黏蛋白沉积症，本病临床少见。     

网状红斑性粘蛋白病综合征

本文作者报道1例网状红斑性粘蛋白病(REM)综合征.患者为29岁高加索妇女,1977年因胸中部和背部红斑性发疹而就诊.患者在日晒后皮疹加重,突起和瘙痒.用倍他米松17戊酸盐油膏治疗无效.体检可见:前胸和上背部有网状斑丘疹性红斑.临床上诊断为REM综合征,病理检查支持这一诊断.口服阿的平100mg,每日2次,临床上明显好转.皮损组织病理学检查显示表皮正常,血管和附件周围淋巴细胞浸润,偶见浆细胞.阿新蓝染色真皮内有阳性染色物呈纤维状沉积.皮损直接免疫荧光检查:基底膜有颗粒状IgM、IgA和C_3沉积.血红蛋白、白细胞、血小板计数和ESR正常.抗核抗     

网状红斑黏蛋白病1例

报告1例网状红斑黏蛋白病。患者男，49岁。头面部、胸背部红斑、丘疹伴瘙痒10年，日晒后皮损无明显改变。组织病理检查示真皮乳头层，网状层上部血管及毛囊周围淋巴细浸润，真皮胶原束之间有阿新蓝染色阳性的黏蛋白沉积。     

网状红斑性黏蛋白沉积症1例并文献复习

报告1例网状红斑性黏蛋白沉积症并复习相关文献。患者女，20岁，以面、颈、胸、背、双上臂持久性光加重性网状红斑为主要临床表现，无系统损害，相关实验室检查无异常。组织病理学示：真皮中上层较大量阿辛蓝染色阳性的黏蛋白沉积，血管周围少量淋巴细胞浸润，直接免疫荧光示基膜区IgM呈线状沉积：根据临床表现、组织病理及阿辛蓝染色等诊断为网状红斑性黏蛋白沉积症。     

网状红斑黏蛋白病并发蕈样肉芽肿1例

报告网状红斑黏蛋白病并发蕈样肉芽肿1例。患者女。42岁。因躯干、上肢红斑14年,皮损增多,加重5年就诊。6年前行皮损组织病理学检查示真皮乳头层及网状层有大量黏蛋白样物质沉积,诊断为网状红斑黏蛋白病,入院皮肤科检查：头部、面部、颈部、躯干、四肢见片状轻度浸润性红斑,部分融合成网状,腹部红斑上可见紫红色浸润性斑块。皮损组织病理检查：真皮肉淋巴细胞苔藓浸润,部分细胞异形,少量浸润细胞移入表皮,形成Pautrier样微脓种,免疫组化染色结果示：LCA（＋＋＋）,CD45RO（＋＋＋）,诊断。蕈样内芽肿。     

毛囊黏蛋白病1例

患者男,82岁,面部、躯干浸润性红斑伴痒1月余来院就诊。组织病理示:真皮毛囊上皮水肿,黏液池形成,毛囊内及周围少量淋巴细胞浸润。阿新蓝染色(+)。免疫组化示:CD3(+)、CD4(+)及UCHL-1(+)。诊断为毛囊粘蛋白病向嗜毛囊性蕈样肉芽肿转化。     

内分泌、代谢、营养障碍性皮肤病

20 0 0 1562　网状红斑粘蛋白综合征一例 /李俊民 (河南开封市二院皮肤科 )…∥中华皮肤科杂志 .- 1999,32( 6) .- 4 2 0女 ,36岁。额、颧、颊、眉间弥漫性红斑 ,无脱屑 ,颈肩部可见融合性网状红斑 ,胸、背、腹、四肢、腹股沟处暗红色斑块 ,并见多片直径 2～ 4 mm大小淡红色毛囊性丘疹 ,圆顶、质韧、孤立、不融合。组织病理学检查 :表皮轻度水肿 ,真皮浅层血管周围少许淋巴细胞浸润 ;阿新蓝染色示真皮乳头层及网状层粘蛋白样物质大量沉积。图 2　 (赵恩兵 )2 0 0 0 1563　苔藓样和斑状皮肤淀粉样变的临床病理和免疫组化研究 /马小玲 (北京医…     

肢端持续性丘疹性粘蛋白病1例

本文报导一例56岁男性,肢端持续性丘疹性粘蛋白病(APPM)患者。近期的报告认为该病是皮肤粘蛋白病的局灶型,皮肤粘蛋白病是一组临床疾病。组织病理显示真皮内有特征性均质化。这种均质化是由于成纤维细胞产生过量的粘多糖沉积在真皮所致。粘多糖由透明质酸以及小量的硫酸软骨素和肝素组成,作者首次在英国杂志上报导一例肢端持续性丘疹性粘蛋白病。     

肿胀性红斑狼疮1例

患者男 ,3 0岁 ,面部、上胸背及双手背水肿性红斑 5 0天。实验室检查免疫学指标均正常 ,皮损组织病理检查示 :表皮未见明显异常 ,真皮血管周围少量慢性炎性细胞浸润 ;阿新蓝染色示真皮胶原纤维间有粘蛋白沉积 ,诊断为肿胀性红斑狼疮。     

毛囊黏蛋白病1例

患者男,82岁,面部、躯干浸润性红斑伴痒1月余来院就诊.组织病理示：真皮毛囊上皮水肿,黏液池形成,毛囊内及周围少量淋巴细胞浸润.阿新蓝染色（＋）.免疫组化示：CD3（＋）、CD4（＋）及UCHL-1（＋）.诊断为毛囊粘蛋白病向嗜毛囊性蕈样肉芽肿转化.     

Reticular erythematous mucinosis and acral papulokeratotic lesions associated with myxoedema due to Hashimoto thyroiditis.

A case is described of a patient with generalized myxoedema due to Hashimoto thyroiditis involving lesions of reticular erythematous mucinosis and acral papulokeratotic lesions with 'church spire' histological pattern. Substitutive treatment with thyroid hormone led to a rapid regression of the cutaneous lesions. The infrequent association of reticular erythematous mucinosis lesions and other forms of cutaneous mucinoses with hypothyroidism is discussed, with special emphasis on the fact that the underlying thyroid disease in all cases was Hashimoto thyroiditis. The association of distal keratotic papules with hypothyroidism has not been previously reported in the literature.     

Prurigo pigmentosa successfully treated with low-dose isotretinoin

BACKGROUND/AIMS: Prurigo pigmentosa (PP) is an uncommon inflammatory disease with pruritic reddish papules, papulovesicules or vesicules that are symmetrically localized on the trunk and nape, accompanied by reticular hyperpigmentation. Currently available therapeutic options seem somewhat unsatisfying. Herein, we report an 18-year-old female with PP successfully treated with low-dose isotretinoin. METHODS: The patient presented with a symmetrical pruritic eruption on the lateral sides of her trunk with erythematous papules on a hyperpigmented background. Based on the typical clinical and associated histological findings, the patient was diagnosed as PP and a treatment with low-dose isotretinoin 0.3 mg/kg/day (20 mg/day) was started. RESULTS: After a total of 3 months of 20 mg/day isotretinoin therapy, all erythematous macules and papules resolved and the residual pigmentation had almost disappeared. CONCLUSION: Low-dose isotretinoin is not only adequate for the improvement of erythematous lesions, it also helps resolve the reticular hyperpigmentation of PP. Further studies are needed to observe the efficacy of isotretinoin in the treatment of PP.     

Congenital reticular ichthyosiform erythroderma--ichthyosis variegata: a case report and review of the literature

We present a 32-year-old white patient with congenital reticular ichthyosiform erythroderma, also known as ichthyosis variegata. The patient had typical clinical features with areas of normal appearing skin surrounded by erythematous hyperkeratotic patches. The smaller "confetti-like" patches were mostly present on the trunk, while the larger areas forming a reticular pattern predominated on the extremities. Ultrastructural findings demonstrated perinuclear vacuolization, binuclear cells and filamentous deposits compatible with congenital reticular ichthyosiform erythroderma. Besides presenting the case, we review the literature on this rare disorder of keratinization.     

Posttransplantation lymphoproliferative disease with features of lymphomatoid granulomatosis in a lung transplant patient

A 58-year-old lung transplant patient developed worsening shortness of breath and indurated erythematous plaques on the lower left leg. A skin biopsy specimen revealed a dense angiocentric and angioinvasive infiltrate in the mid to reticular dermis and panniculus containing large, atypical lymphocytes with convoluted nuclei and prominent nucleoli. Immunohistochemical stains showed that the atypical cells were of B-cell origin, and that Epstein-Barr virus was present. Molecular studies demonstrated B-cell clonality. The patient was successfully treated with rituximab. The clinical and pathologic features in this case represent posttransplantation lymphoproliferative disease with features of lymphomatoid granulomatosis. The case also highlights the importance of clinical and pathologic examination of cutaneous lesions in the evaluation of lymphoproliferative disorders for patients undergoing transplantation.     

REM syndrome with an unusual location

We report on a patient suffering from REM syndrome. The characteristic reticular erythematous eruptions had primarily affected the abdomen.     

Cutaneous mucinoses and HIV infection

In the last few years cutaneous mucinoses have been reported with increased frequency in HIV patients. We report the occurrence of scleredema, reticular erythematous mucinosis and lichen myxoedematosus in three different HIV-infected patients, review the literature and discuss the possible relationship between mucin deposits and HIV infection. This is the first report of scleredema and the second of reticular erythematous mucinosis in an HIV-infected patient. Only the association of HIV infection with lichen myxoedematosus seems to be more than coincidental.     

Self-healing juvenile cutaneous mucinosis

A healthy 14-month-old black girl presented with a 3-week complaint of "knots" on the face and hands. The lesions were acute in onset and asymptomatic. Multiple, firm, nontender, skin-colored to erythematous nodules were noted on the scalp, forehead, axillae, lower legs, abdomen, and hands. A skin biopsy specimen revealed a well-circumscribed accumulation of mucin in the reticular dermis. Colloidal iron stain was positive. Radiographs showed soft tissue prominence only. Serum protein electrophoresis, thyroid function tests, complete blood count, sedimentation rate, and antinuclear antibody were normal, except for lymphocytosis. Findings were consistent with self-healing juvenile cutaneous mucinosis (SHJCM). SHJCM is a condition of unknown etiology characterized by rapid onset of asymptomatic, indurated papules or nodules. Affected children may have arthralgias, but are otherwise well. Spontaneous resolution is the rule. Most skin lesions in our patient had resolved within 6 months of onset. This patient is unique because of the young age of onset.     

Pimecrolimus 1 percent cream and pulsed dye laser in treatment of a patient with reticular erythematous mucinosis syndrome

We report on the efficacy of twice daily application of pimecrolimus 1 percent cream in a 48-year-old woman with reticular erythematous mucinosis (REM) syndrome and compare its results with pulsed dye laser (PDL) on the other side of her chest and back. The patient was previously treated by hydroxychloroquine but only a fair response was observed. After application of 5 months of pimecrolimus, the lesions completely resolved and the result was comparable with the other side of her body treated by pulsed dye laser PDL. Topical pimecrolimus and pulsed dye laser appear to be effective and safe treatments for REM.