12例累及面部的结节病临床病理分析

回顾性分析我院皮肤科2013年至2019年诊断的12例（女8例,男 4例）累及面部的结节病临床资料,患者平均发病年龄53.1岁。12例患者中,4例累及面部以外的皮肤;1例累及多系统（肺、心、肾、肝、脾）,6例累及肺部,4例累及肺门淋巴结。12例患者组织病理均见真皮内非干酪性上皮样细胞肉芽肿,抗酸染色均阴性。     

Cerebrospinal fluid tau concentrations in HIV infected patients with suspected neurological disease

OBJECTIVES: To measure cerebrospinal fluid (CSF) tau in HIV infected patients with acute neurological episodes and to correlate the findings with the type and severity of neurological disease. METHODS: CSF tau was prospectively measured in 76 consecutive HIV infected patients admitted to a specialist unit at UCL Hospitals, London, for investigation of acute neurological episodes: the results were compared with the clinical diagnoses. RESULTS: 24 patients had HIV associated dementia complex (HADC), 10 had lymphoma (including four with primary CNS lymphoma), 20 had cerebral infections (including five with CMV encephalitis, five with VZV infection, seven with cryptococcal meningitis, two with toxoplasmosis, and one with progressive multifocal leucoencephalopathy); 22 patients had miscellaneous conditions, including nine with self limiting headache/fever. 62 patients (82%) had normal CSF tau concentration and 14 patients (18%) had elevated tau. In those with HADC, there was no correlation between the degree of dementia or atrophy on magnetic resonance imaging and CSF tau. Elevated CSF tau was associated with poor outcome as six of eight patients who died within 4 weeks of lumbar puncture had elevated tau (p = 0.0024, two tailed Fisher's exact test). CONCLUSIONS: CSF tau levels are not elevated in the majority of HIV infected patients presenting with acute neurological episodes. CSF tau levels show no correlation with severity of dementia/atrophy on magnetic resonance imaging. Although elevated CSF tau was observed in some patients with conditions causing cerebral necrosis, the finding did not delineate underlying pathology but was associated with poor outcome.     

陕西省麻风新发病例基本情况分析

通过全国麻风防治管理信息系统（LEPMIS）收集陕西省2004-2020年麻风病例资料,对新发麻风患者的基本情况进行回顾性分析。研究纳入了305个麻风新发病例,其中男212例,女93例,MB型麻风272例(89.2%),PB型麻风33例(10.82%)。60岁以上患者49例,20～59岁247例,20以下患者9例。100例（32.8%）有家庭内接触史。42例（13.8%）出现麻风反应,247例（81%）出现神经损害,男性神经损伤率高于女性（86.3% vs 68.8%）。218例（71.5%）出现畸残,其中II级畸残122例,I级畸残96例。     

5例SAPHO综合征的临床特点

目的:了解SAPHO综合征的临床特点.方法:回顾分析2014年9月至2020年1月香港大学深圳医院皮肤科确诊的5例SAPHO综合征患者的临床资料,包括患者的临床表现、实验室检查、影像学检查、治疗及转归等信息.结果:5例患者均出现掌跖脓疱,其中1例伴急性泛发性发疹性脓疱病、1例伴痤疮、1例受累骨关节局部皮肤红肿.关节表现...     

PYODERMA GANGRENOSUM: ASSOCIATIONS REVISITED

Fourteen cases of pyoderma gangrenosum were seen over a period of 24 years at the Hull Royal Infirmary Dermatology Department. Several associated conditions were found. Seven cases were associated with rheumatoid arthritis of which five were sero-positive, including one with Felty's syndrome. One case was associated with both ulcerative colitis and psoriasis; one with polycythemia rubra vera; two patients had diverticular disease including one who also had rheumatoid arthritis; one had positive syphilis serology. In three cases there was no significant associated disease identified. Ten out of the fourteen cases were women, indicating a female preponderance by a ratio of about 2F:1M; a figure similar to that stated by Seitzinger. The age of presentation ranged from 30 to 80 years.     

Case of anaphylaxis caused by ingestion of royal jelly

Anaphylaxis is a severe form of allergic disease. Royal jelly is widely consumed in Japan, but a few cases of anaphylaxis caused by royal jelly have been reported. We encountered a 26-year-old Japanese woman who developed anaphylaxis after drinking a beverage of crude royal jelly including honey. She had a history of bronchial asthma, allergic rhinitis, allergic conjunctivitis, atopic dermatitis and food allergy (abalone). Prick tests were performed with the beverage of crude royal jelly including honey and with two other beverages including honey. Only the crude royal jelly beverage showed a positive reaction. An oral challenge test with the crude royal jelly beverage was not performed, but a similar test with a beverage including honey caused no symptoms. A positive response to the beverage of crude royal jelly was not observed in normal volunteers. A positive diagnosis of anaphylaxis due to royal jelly was made based on the positive prick test, systemic clinical symptoms and the negative prick tests in healthy volunteers. Moreover, the patient had no symptoms when taking lemon and orange, which were present as essences in the crude royal jelly beverage, and also had no response to honey after anaphylaxis. Increased consumption of royal jelly in health food supplements may increase the incidence of royal jelly-related allergic reactions. Therefore, royal jelly should be considered as a causative allergen in food-induced anaphylaxis.     

The occurrence of various collagen diseases in one family: a sister with ISSc, PBC, APS, and SS and a brother with systemic lupus erythematosus.

We encountered siblings who had collagen diseases and related symptoms. Case 1 was a 53‐year‐old woman who had limited cutaneous systemic sclerosis (ISSc) associated with primary biliary cirrhosis (PBC), antiphospholipid antibody syndrome (APS), and subclinical Sjögren's syndrome (SS). Case 2 was a 48‐year‐old man, her younger brother, with systemic lupus erythematosus (SLE) that developed at 32 years of age. Investigation of their family revealed that their mother had Raynaud's phenomenon, arthritis, and subclinical Sjögren's syndrome, and that another younger brother of Cases 1 and 2 had Raynaud's phenomenon and general fatigue. HLA analysis revealed that the sister and brother had some identical HLA antigens in common, including A2, A33 (19), B67, B44 (12), Cw7, DR2, DR6, DR52, and DQ1. The sister, brother and their mother had common HLA antigens including A2, B67, Cw7, DR2, and DQ1. Although Cases 1 and 2 shared the same HLA system, they presented different phenotypes of collagen disease.     

Blue Scalp Discoloration in Infants Caused by Exogenous Source

A blue patch on the vertex scalp of an infant has a broad differential diagnosis, including vascular malformations, melanocytic lesions, trauma, and exogenous or endogenous pigmentation. We present two cases of infants who developed blue patches on the scalp. Extensive examination including MRI and biopsy was recommended for one of the patients to further characterize the lesion and its possible medical implications. In both, the blue discoloration had been due to repetitive contact with the sticker on a tub of baby wipes that had rubbed off onto the scalp. We present these infants to highlight this exogenous and benign cause of blue pigmentation and help practitioners avoid unnecessary examination.     

Pregnancy and porphyria cutanea tarda

A 31-year-old woman developed typical clinical and laboratory signs of PCT at the end of her second pregnancy coincident with the summer season. She had elevated liver function values without history of alcoholism, hepatitis or chemical liver damage. She had taken oral contraceptive only before her first pregnancy which was normal. Her hormone analytic values including estrogens corresponded to normal values in pregnancy. Venesections had a beneficial affect on her condition.     

Medium-dose UV-A1 phototherapy. Successful treatment of cutaneous sarcoidosis

A 82-year-old female had a 2-year history of cutaneous sarcoidosis without systemic involvement. Various treatments including local glucocorticosteroids and tacrolimus ointment had failed. Therefore, we treated our patient with medium-dose UVA1 phototherapy. After 50 sessions with a total dose of 2.640 J/cm(2) all lesions had disappeared. Clinical follow up showed no recurrence of skin lesions after 5 months.     

Wegener's Granulomatosis Successfully Treated with Prednisolone and Potassium Iodide

Wegener's granulomatosis has traditionally been treated with steroids and cyclophosphamide. We used potassium iodide in conjunction with prednisolone to treat a patient with a limited form of this disease who had gangrenous rhinitis and skin involvement including necrotic papules and ulcerations. After three months of this therapy, the mucosal and cutaneous lesions had almost completely disappeared.     

Lipoid proteinosis: report of four siblings and brief review of the literature

Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomal recessive disorder associated with deposition of periodic acid-Schiff (PAS)-positive hyaline material in various tissues including skin, mucous membranes, and internal organs. A family is reported in which four siblings (two boys and two girls) born to nonconsanguineous parents had lipoid proteinosis. All had the characteristic hoarseness of voice and three had skin lesions. The diagnosis was confirmed by the presence of typical features on light and electron microscopy.     

The spectrum of cutaneous lymphomas in Japan: a study of 62 cases based on the World Health Organization Classification

BACKGROUND: The relative incidence of malignant lymphoma subtypes differs according to geographic location. This study investigated the epidemiology of cutaneous lymphoma subtypes in Japan and compared it with other countries. METHODS: Sixty-two patients with cutaneous lymphoma attending the Department of Dermatology, National Hospital Organization Hokkaido Cancer Center were reviewed. The World Health Organization classification of hematopoietic and lymphoid malignancies was adopted. RESULTS: Of the 62 patients, 31 had primary cutaneous lymphoma (PCL) and 31 had secondary cutaneous lymphoma (SCL). T- and natural killer (NK)-cell lymphoma accounted for 80% of PCL, of which, mycosis fungoides accounted for almost 35%. Of the 31 patients with secondary cutaneous lymphoma, 17 patients (54%) had T- and NK-cell lymphoma, including nine adult T-cell leukemia/lymphoma patients, and 14 patients (46%) had B-cell lymphoma, including 11 diffuse large B-cell lymphoma patients. The majority of patients with SCL and NK-cell lymphoma with primary or secondary skin lesions had a poor outcome. CONCLUSIONS: PCL in this study showed a similar incidence to that of other institutions in Japan, while also demonstrating different frequencies from that of other countries, suggesting that the relative frequency of different PCL subtypes differ according to geographical location, similar to previous reports of systemic malignant lymphoma.     

5q14.3 Microdeletions: A Contiguous Gene Syndrome with Capillary Malformation–Arteriovenous Malformation Syndrome and Neurologic Findings

Deletions within chromosome region 5q14.3q15 have been associated with a spectrum of disorders including developmental delay, hypotonia, absent speech, mild facial dysmorphism, seizures, and brain anomalies. Some cases of concomitant neurologic abnormalities and cutaneous vascular malformation associated with 5q14.3 deletion have been reported. Previously reported cases had similar features, including multiple capillary malformations, and neurologic abnormalities, including epilepsy, hypotonia, and developmental delay. We report a case of 5q14.3 neurocutaneous syndrome presenting with multiple capillary malformations, neurologic abnormalities, and microdeletion in chromosome 5q14.3.     

The ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC): report of five cases

We report five patients from two different pedigrees with the ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC). All had features of ectodermal dysplasia, but only three had limb ectrodactyly and orofacial clefting. The present patients illustrate the great phenotypic variability in the EEC syndrome. As no single feature, including any of the three cardinal signs, is mandatory for syndrome diagnosis, a meticulous examination of all family members is needed.     

先天性甲肥厚Ⅰ型一家系调查

报告先天性甲肥厚1个家系,2代共有3人患病,3例患者均有甲增厚,掌跖角化,掌跖多汗,局部摩擦可发生水疱和毛发稀疏等典型表现.先证者手、足背弥漫性角化过度,18只甲营养不良。     

Sunbed-induced pseudoporphyria

The clinical details of four fair-skinned women with recurrent blistering and skin fragility are presented. All had considerable sun-exposure and had used sunbeds for years. Specific causes of bullous dermatoses including drug ingestion, porphyria, cutaneous amyloidosis and epidermolysis bullosa acquisita were excluded. Long-term recurrent exposure to high doses of sunbed radiation in chronically sun-damaged skin appears to be a further cause of pseudoporphyria.     

Nickel dermatitis and diet: clinical improvement and a reduction in blood and urine nickel levels with a low-nickel diet

A 27-year-old nickel-sensitive female who had had continuous spontaneous flare-ups of eczema, including at sites of previous metal contact, experienced a clearing of her eruption after commencing a low-nickel diet. When on the diet, whole-blood and urinary nickel levels fell to half or less of pre-diet values and this coincided with the clinical improvement. Low-nickel diets should be considered for patients who are highly nickel sensitive.     

Necrobiotic xanthogranuloma: a report of four cases

Four patients are described who demonstrate the range of clinical and pathological features seen in necrobiotic xanthogranuloma with paraproteinaemia (NXG). Each patient had the typical periorbital yellow plaques with numerous well demarcated yellow indurated plaques on the trunk. All four patients had evidence of paraproteinaemia. The histopathology showed diagnostic features of intense necrobiosis with xanthomatization, including touton giant cells and the bizarre angulated giant cells of NXG. Previously, the patients had been diagnosed as atypical necrobiosis lipoidica, and the distinction between NXG and other necrobiotic conditions is discussed.