红皮病型结节病一例

患儿女, 8岁,因皮肤红斑、丘疹半年,加重 1个月于 1999年 10月 15日入院。入院前半年患儿无明显诱因头皮出现红色斑丘疹,上覆少许鳞屑,未引起重视 ,后皮疹逐渐向全身发展伴轻微瘙痒,在当地诊断为银屑病,给予药物口服及外用药治疗（具体不详）,病情无好转,皮损逐渐增多,融合成片。 1个月前全身皮肤弥漫潮红,双侧腹股沟及颈项部出现包块,无畏寒、发热。在当地医院住院做骨髓穿刺示“粒细胞与红细胞比例偏低,红细胞系统增生活跃”,腹股沟淋巴结活检示“淋巴结结核”,右前臂皮损活检示“皮下纤维组织增生伴散在淋巴细胞浸润”,…     

红皮病型结节病二例并文献复习

两例患者(男女各1例)表现为红斑、丘疹及脱屑,伴轻微瘙痒,皮肤组织病理特点符合结节病诊断。男性患者胸部CT示双下肺少许间质病变。女性患者胸部CT示两肺散在小结节影,双腋窝淋巴结增大。两例患者糖皮质激素治疗均有效。     

红皮病型结节病一例

临床资料患者,男,16岁。因全身红斑、脱屑6年余、全身浅表淋巴结肿大5年。6年前无明显诱因胸前出现红斑、丘疹,自觉瘙痒。未引起重视,皮疹逐渐向全身发展,在当地诊断为＂银屑病＂,给予药物口服及外用药治疗（具体不详）,效果不佳,皮损渐增多,融合成片,呈弥漫性潮红、脱屑;1年后颈部、双侧耳后、腋窝、腹股沟出现包快,无畏寒、发热。     

隐性遗传先天性鱼鳞病样红皮病1例报告

隐性遗传先天性鱼鳞病样红皮病１例报告宫淑敏史月君朱英华隐性遗传先天性鱼鳞病样红皮病又名板层样鱼鳞病，是一种罕见的角化性遗传性疾病，现报告１例。患者女，２７岁，未婚，全身皮肤干燥、鳞屑２７年于１９９４年３月８日来诊。患者于生后４ｈ皮肤被胶样膜覆盖，１～...     

表现为红皮病的遗传性皮肤病

红皮病病因复杂,既有先天遗传因素,也有后天继发性因素。成人和儿童发病原因各有其特点。在确立红皮病诊断后,找寻原因及给予及时的治疗是必要的,因此,归类及阐述红皮病发病病因具有重要意义。此文将表现为红皮病的遗传性皮肤病做一综述,为临床提供诊断思路。     

获得性鱼鳞病

获得性鱼鳞病是一种非遗传性皮肤病,其特征为成人身体某些部位的皮肤发生干燥、粗糙,伴有显著鳞屑.它可以并发恶性肿瘤、自身免疫和炎症性疾病、代谢和内分泌疾病以及感染性疾病等,也可与服用某些药物有关.该文对获得性鱼鳞病作一较全面的介绍,重点介绍其与系统性疾病的关系.     

获得性鱼鳞病研究进展

获得性鱼鳞病在临床和组织学上类似于常染色体显性遗传性寻常型鱼鳞病,但生物学表现不同.前者主要表现为皮肤干燥、粗糙和显著的鳞屑;组织病理检查显示:表皮角化过度,颗粒层变薄或缺失.获得性鱼鳞病与多种系统性疾病和某些药物有关,治疗上主要是消除致病因素和对症处理.     

大疱性鱼鳞病样红皮病1例

患儿女,8月龄,因全身皮肤反复发生红斑、脱屑8个月就诊。其母诉称患儿出生后次日即出现全身皮肤红斑及脱屑,并有蚕豆大小水疱数个。疱壁薄,轻触碰即破,约1周疱干涸,形成鳞屑,反复发作至今。有时水疱较大如核桃。患儿系34周早产儿。母孕期无上感及特殊用药史。父母非近亲婚配,生育2胎,长子已14岁,健康。患儿为第2胎。家族中无类似疾病及鱼鳞病等皮肤病史。患儿发育差,消瘦,体重5ｋｇ,余未见特殊。皮肤科情况:全身皮肤除颜面外弥漫性潮红、干燥及脱屑,几乎无正常皮肤可见。双小腿伸侧可见大小不等的疣状角化鳞屑性损害。左小腿有蚕豆至5分币大…     

先天性大疱性鱼鳞病样红皮病1例

先天性大疱性鱼鳞病样红皮病（bullous congenital ichthyosiform erythroderma,BCIE）又称表皮松解性角化过度（epidermolytic hyperkeratosis,EHK），是一种罕见的常染色体显性遗传性疾病，现将所见1例报告如下。     

非水疱性先天性鱼鳞病样红皮病1例

1　临床资料　患儿男 ,18天。全身皮肤鱼鳞样脱屑 15天。系二胎二产 ,孕 3 5周早产 ,出生时无窒息史 ,无羊水污染。出生后患儿全身皮肤有鱼鳞样黄色厚痂皮 ,伴脱屑 ,脱屑后皮肤潮红 ,痂皮脱落以摩擦部位多见 ,脱落后少许皮肤又出现一层薄痂皮。曾在外院按“湿疹”治疗 (具体不详 )。母孕期 5 0天时患感冒未服任何药物。父母均体健 ,非近亲结婚。其兄长 ,8岁 ,体健。家族中无遗传病史。体检 :体温正常 ,P 12 0次 /min ,R 45次/min ,体重 2 6kg。营养欠佳 ,早产儿貌 ,反应可 ,哭声响亮。头面四肢躯干皮肤过度角化透亮 ,上覆有大片淡黄色厚痂…     

Erythrodermic psoriasis in a dialyzed patient successfully treated with Secukinumab

Erythrodermic psoriasis is a severe, life‐threatening condition with additional complications, when occurring in hemodialyzed patients, as the majority of treatments are contraindicated. A 44‐years‐old man, of Philippine origins, with a 15‐years‐history of psoriasis treated with cyclosporine developed progressive hypertension and renal insufficiency. Despite drug dismission, renal function worsen to end‐stage, and hemodialysis was necessary three times a week. Phototherapy was not able to control the skin condition, progressing to erythroderma, and after nephrology consultation, the patient consent to the off‐label secukinumab treatment, at the standard regimen (300 mg subcutaneously once weekly at weeks 0‐4 followed by 300 mg every 4 weeks). Seven days after the first injection, a rapid improvement was noted, with the psoriasis area severity index (PASI) score passing from 31.5 to 17.6. At the 52‐week‐follow‐up visit, the patient was completely clarified, without any side effects. The case supports secukinumab effectiveness and safety in difficult patients, including erythrodermic psoriasis with end‐stage renal failure, as drug plasma levels seem not to be affected by hemodialysis. Results are rapidly achieved, and long term maintained, with the additional advantage of a very comfortable monthly administration.     

Cutaneous Mycobacterium avium-intracellulare infection masquerading as sarcoidosis

Mycobacterium avium-intracellulare (MAC) infection may have different skin manifestations, including cutaneous granulomas. Granulomatous skin reactions have distinct morphologic and histopathologic appearances. We present the case of an adolescent male with cutaneous MAC, misdiagnosed as sarcoidosis after initial biopsy results, demonstrated preservation of reticulin fibers and absence of organisms within granulomas. Sarcoidal granulomas often stain positive for reticulin fibers, which could be used to distinguish them from the infectious kind. This case should alert clinicians to the fact that the presence or quantity of intact reticular fibers may not be a reliable tool to differentiate between a sarcoidal and an infectious granuloma. Our case also highlights the diagnostic challenge of cutaneous MAC infection.     

Mycophenolate mofetil may serve as a steroid-sparing agent for sarcoidosis

Sarcoidosis is an inflammatory disease with potentially severe mucocutaneous manifestations. Mycophenolate mofetil (MMF) is an immunosuppressive drug extensively used in organ transplantation. Its use has been rapidly expanded into autoimmune and inflammatory diseases. We report the first successful and safe use of MMF in five patients with sarcoidosis.     

Granulomatous mycosis fungoides presenting as an acquired ichthyosis

We report a case of a 69-year-old gentleman who presented with a 3-month history of unexplained fevers and malaise who developed generalized pruritus, alopecia and an ichthyosiform erythematous eruption on his forearms, legs, chest and back. Skin histology, immunophenotyping and molecular features were consistent with granulomatous mycosis fungoides. He has been successfully treated with twice weekly PUVA photochemotherapy.     

Subcutaneous sarcoidosis

Subcutaneous sarcoidosis has been reported to occur in 1.4% to 6% of patients with systemic sarcoidosis. Most reported cases are in women, most often in their fifth and sixth decades, and appear as multiple, asymptomatic, hardly indurated subcutaneous nodules without changes in the overlying epidermis. The lesions are characteristically located in the upper extremities, mainly in the forearms, and usually are bilateral and asymmetric. In most cases the lesions appear at the beginning of systemic sarcoidosis and are not associated with chronic fibrotic disease. Histopathologically, sarcoidosis is characterized by noncaseating naked granulomas involving fat lobules, with minimal to no septal involvement.     

瘢痕结节病一例

报告1例手术后瘢痕处发生的结节病。患者女,54岁,因颈部手术切口处出现多个结节10月就诊。患者17年前行甲状腺手术。皮损组织病理示真皮全层、皮下组织及肌肉组织内非干酪样肉芽肿形成。     

皮下型结节病一例并文献复习

患者,女,51岁。左膝肿块半年,右膝皮下结节1月余。双膝部皮损组织病理：真皮深部至皮下可见大的由组织细胞、上皮样细胞形成的结节,境界清楚,无干酪样坏死。网状纤维染色：（+）,结节周围有网状纤维增生。诊断：结节病（皮下型）。予羟氯喹联合复方甘草酸苷治疗1个月后,结节明显消退。     

A case of sarcoidosis involving the tongue.

Sarcoidosis is a systemic granulomatous disorder which commonly affects the skin. Involvement of the tongue is rare; a review of the previous literature over the last 30 years revealed only six cases of sarcoidosis affecting the tongue. We studied a case of sarcoidosis involving the tongue in a 32-year-old Japanese woman with characteristic clinical and pathological findings. She visited our department with a complaint of a tongue lesion of which she had been aware for a month. A diagnosis of sarcoidosis was made for the lesion by clinical and pathological examinations. Oral involvement by sarcoidosis is rare, however this disorder should be considered as a possible cause of intraoral granulomatous lesions.     

Histopathological and immunohistochemical assessment of acquired ichthyosis in patients with human T-cell lymphotropic virus type I-associated myelopathy

BACKGROUND: Patients with human T-cell lymphotropic virus type I (HTLV-I)-associated myelopathy frequently display cutaneous alterations such as acquired ichthyosis. OBJECTIVES: Elucidation of the pattern of acquired ichthyosis in HTLV-I-associated myelopathy. METHODS: Skin fragments from 10 patients with HTLV-I-associated myelopathy presenting with acquired ichthyosis were assessed by histopathological and immunohistochemical tests. We used anticytokeratin antibodies related to normal keratinization (K1/K10), and others related to cutaneous conditions such as activation, migration and hyperproliferation of keratinocytes (K6/K16), and involucrin, a precursor protein in the formation of the protein envelope in keratinocytes. For quantification of the proliferating basal and parabasal cells the anti-Ki-67 antibody was employed. RESULTS: On light microscopy, all skin specimens displayed orthokeratotic hyperkeratosis and hypogranulosis. Three of them presented focal parakeratosis. A slight to moderate perivascular infiltrate of mononuclear lymphocytes was observed in seven cases, three of which showed discrete spongiosis with epidermotropism of lymphocytes. All fragments displayed coexpression of K1, K10 and K16 in the suprabasal layers. Expression of involucrin was also observed in all cases, in the upper spinous and granular layers. Focal expression of K6 was observed in three cases, under a parakeratotic area. The mean number of Ki-67+ basal and parabasal cells was 3.5 cells per mm, similar to that in control skin. CONCLUSIONS: In acquired ichthyosis related to HTLV-I-associated myelopathy, histopathology revealed orthokeratotic hyperkeratosis and a perivascular inflammatory infiltrate of mononuclear lymphocytes, with areas of parakeratosis and foci of epidermotropism in rare cases. The expression profiles of K1, K10 and involucrin were similar to those in normal skin. The diffuse coexpression of K16 with K1 and K10 throughout the analysed epidermis, as well as the occurrence of restricted areas of parakeratosis expressing K6, indicate the presence of keratinocyte activation with induction of the alternative keratinization pathway, probably dependent on the cytokines liberated by the mononuclear cells of the dermal inflammatory infiltrate infected with HTLV-I. The absence of acanthosis and of increased cellular kinetics, as shown by the low rate of Ki-67 antigen expression, allow the inference that the pattern of acquired ichthyosis related to HTLV-I-associated myelopathy may be retentional. The observation of foci of parakeratosis expressing K6 in three specimens suggests that, at least in certain areas and in some cases, interference with epidermal differentiation and maturation occurs.