47例Paget病临床与组织病理分析

本文回顾性分析了47例Paget病的临床及病理表现,其中乳房Paget病17例,均为女性,平均发病年龄54.2岁,平均病程19.6个月;乳房外Paget病30例(男28例,女2例),平均发病年龄68.2岁;平均病程61.96个月。皮损均表现为界清的红斑,表面有渗出、糜烂和结痂。皮损组织病理可见典型的散在或成巢分布的Paget细胞,免疫组化肿瘤细胞表达CK7、CEA、CKpan。45例患者早期误诊为"湿疹"。     

Comedonal, cornifying and hypertrophic Darier's disease in the same patient: a Darier combination

Our 42-year-old patient had comedonal Darier's disease (DD) on the face, comedonal cornifying DD on the upper back, and hypertrophic DD on both legs. Biopsies taken from face, upper back and medial sides of the legs were found to be compatible with these clinical subtypes. The comedonal type was in the classical place, but the hypertrophic and cornifying types were not in the usual sites. In addition to the classic histopathology of DD, we noted multiple, warty dyskeratoma-like structures in the comedonal type, marked compact hyperkeratosis in the cornifying type, and marked papillomatosis in the hypertrophic type.     

Use of infliximab in pyoderma gangrenosum

A 57-year-old man, with a 2-year history of Crohn's disease, presented with a rapidly progressive abdominal ulcer. It was clinically and histologically consistent with pyoderma gangrenosum but it did not respond to either high-dose oral prednisolone or intravenous hydrocortisone. Infliximab resulted in an early, dramatic and sustained improvement. His bowel symptoms, which flared a few weeks prior to the onset of pyoderma gangrenosum, only partially improved. Despite six infliximab infusions, the inflammatory bowel disease is poorly controlled, whereas the pyoderma gangrenosum remains healed.     

Vulval Crohn disease: A case series of 26 patients

Vulval Crohn disease is rare and likely under-reported, leading to difficulty in diagnosis and delay in treatment. In this case series, we report the clinical features and discuss therapy in 26 consecutive patients with vulval Crohn disease, with or without documented gastrointestinal disease, presenting between January 2016 and July 2018. We highlight the need for treatment that is in some cases more aggressive than the requirement to manage the patient's gastrointestinal involvement.     

A case of linear IgA disease occurring in a patient with colonic Crohn's disease

Linear IgA disease has been reported in association with inflammatory bowel disease, in particular ulcerative colitis. We experienced a case of linear IgA disease that occurred simultaneously with colonic Crohn's disease in a 55-year-old woman and rather unusually both skin and bowel disease improved in tandem. We report the presentation, investigations and subsequent improvement of our patient and speculate on possible causes.     

Crohn''s disease presenting as a breast abscess: A case report

A case of cutaneous Crohn's disease (CD) involving the areola-nipple region of both breasts in a 45-year-old woman is reported. The lesion had initially been diagnosed and treated as a simple abscess. Histopathological examination, however, showed granulomatous inflammation with eosinophils suggesting extraintestinal CD. The patient also had a minor area of cutaneous CD localized at the umbilicus and severe anogenital lesions. Twenty-six years previously the woman had undergone a course of sulphasalazine treatment together with steroid enema for chronic inflammatory colitis, but the colitis had been inactive for 25 years. Treatment with sulphasalazine, metronidazole, prednisolone and azothioprine had only minor effect on the skin lesions. CD is a systemic disorder and the most prominent manifestations may be extraintestinal.     

Melanocyte and gonad activity as potential severity modifying factors in C3H/HeJ mouse alopecia areata

Circumstantial evidence has previously suggested gonad derived steroid hormones and melanogenesis related antigens may modify human alopecia areata (AA). AA-like hair loss can be induced in C3H/HeJ mice after skin allografts from spontaneous AA-affected mice. This inducible model was used to evaluate hormones and hair follicle melanocyte presence as disease-severity modifiers. Ten females and 9 males were gonadectomized and received AA-affected allografts. All gonadectomized mice had 2-4 weeks delay in AA onset relative to non-gonadectomized controls. Two females and 4 males failed to develop any AA by 25 weeks after grafting. The experiment was repeated with gonadectomized female and male mice plus non-gonadectomized mice subcutaneously implanted with silastic capsules containing 80 microg 17beta estradiol or 10 mg 5alpha dihydrotestosterone, respectively. Five of 11 ovariectomized and 9 of 11 non-ovariectomized, estradiol supplemented females developed AA with extremely rapid progression. Three of 8 castrated, but none of 11 non-castrated, dihydrotestosterone-supplemented males expressed AA. In a separate study, 14 mice were freeze-branded, producing white hair on the dorsal lumbar region, and later received full-thickness allografts. Thirteen mice developed patchy pigmented and non-pigmented hair loss. One mouse developed diffuse, pigmented hair loss, but with white hair survival persisting 25 weeks after grafting. The results suggest that gonadal steroid hormones can modulate C3H/HeJ mouse AA where estradiol promoted rapid progression of AA while dihydrotestosterone increased resistance to AA onset. In general, both pigmented and non-pigmented C3H/HeJ mouse hair is susceptible to AA. Murine AA susceptibility and severity clearly involves an interplay between genetic and epigenetic factors.     

Etiopathogenesis of alopecia areata: Why do our patients get it?

Alopecia areata (AA) is a nonscarring, inflammatory skin disease that results in patchy hair loss. AA is unpredictable in its onset, severity, and duration making it potentially very stressful for affected individuals. Currently, the treatment options for AA are limited and the efficacy of these treatments varies from patient to patient. The exact etiology of AA is unknown. This article provides some insights into the etiopathogenesis of AA and why some people develop it. The current knowledge on the pathogenesis of AA is summarized and some of the recent hypotheses and studies on AA are presented to allow for a fuller understanding of the possible biological mechanisms of AA.     

Epidermolysis bullosa acquisita in childhood

This case report of an 11-year-old girl describes a juvenile form of epidermolysis bullosa acquisita, an autoimmune disease of IgG antibodies to basement membrane type 7 collagen. Our case illustrates an unusually severe, acute inflammatory presentation of this condition with prominent mucosal and constitutional features requiring admission to a paediatric burns unit. The treatment consisted of supportive topical and systemic agents, prednisolone and dapsone. She responded to dapsone alone and the course of the illness was uneventful.     

197例川崎病临床分析

目的：探讨川崎病临床特点及诊断方法。方法：对197例川崎病患儿的临床资料进行回顾性分析。结果：川崎病好发于5岁以内，男孩多发，以典型病例多见，但仍有28．9％患儿为非典型病例。发热是最常见的临床表现，无死亡病例。有35．0％的误诊率，误诊的病种包括上呼吸道感染、药物性皮炎、淋巴结炎等儿科常见病。结论：川崎病早期容易误诊，对不明原因发热5天以上，并伴有川崎病主要临床表现中的任何一项的儿童，应警惕川崎病的可能。     

Cutaneous manifestations of metastatic Crohn's disease

Metastatic Crohn's disease is a rare cutaneous complication of primary Crohn's disease. It is a granulomatous inflammatory process, similar to the pathogenic mechanism of Crohn's disease, that occurs in sites discontiguous from the gastrointestinal tract. Metastatic Crohn's disease can precede the development of Crohn's disease by months to years, and children are more likely to present with metastatic Crohn's disease in the absence of gastrointestinal symptoms. Given that approximately 30% of individuals with Crohn's disease present in childhood, early recognition of extraintestinal manifestations of Crohn's disease such as metastatic Crohn's disease can aid in timely diagnosis and management of bowel disease. We present data from two pediatric cases of metastatic Crohn's disease recently seen at our institution in addition to the 61 reported cases of pediatric metastatic Crohn's disease in the literature. This review article will focus on the epidemiology, pathogenesis, clinical features, and histology of and treatment options for pediatric metastatic Crohn's disease.     

Langerhans Cell Histiocytosis: A Case Report and Glimpses into Its Nomenclature

Class I Langerhans cell histiocytosis (LCH) is described in a two- and a half-year-old male. The initial expression of the disease was conspicuous by the presence of extensive pustular crusted rash and, later in its course, by purpura, diabetes insipidus, otitis media, enlargement of the liver and spleen, and infiltration into the lungs. The patient expired due to extensive hemorrhage over 24 hours.     

A retrospective Aliskiren and Losartan study in non-diabetic chronic kidney disease

AIM: To assess the efficacy of combined Aliskiren and Losartan vs high dose Losartan and Aliskiren alone in chronic kidney disease (CKD).METHODS: This is a retrospective study of 143 patients with non-diabetic CKD comparing combined Aliskiren (150 mg/d) with Losartan (100 mg/d) therapy vs High dose Angiotensin receptor blockers (ARB) (Losartan 200 mg/d) and the third group Aliskiren (150 mg/d) alone. This study involved only patient medical records. Entry criteria included those patients who had been treated with the above drugs for at least 36 mo within the 5 years period; other criteria included proteinuria of 1 g or more and or CKD Stage 3 at the start of the 36 mo period. The study utilised primary renal end points of estimated Glomerular Filtration Rate (eGFR) < 15 mL/min or end stage renal failure.RESULTS: Patients treated with high dose ARB compared to the other two treatment groups had significantly less proteinuria at the end of 36 mo (P < 0.007). All 3 groups had significant reduction of proteinuria (P < 0.043, P < 0.001). Total urinary protein was significantly different between the 3 groups over the 3-year study period (P = 0.008), but not eGFR. The changes in eGFR from baseline to each year were not significantly different between the 3 therapeutic groups (P < 0.119). There were no significant differences in the systolic and diastolic blood pressure between the 3 drug groups throughout the 3 years. The incidence of hyperkalemia (> 5.5 mmol/L) was 14.2% (7/49) in the Combined Aliskiren and ARB group, 8.7% (4/46) in the Aliskiren alone group and 6.3% (3/48) in the High dose ARB group (P < 0.001).CONCLUSION: This study in non-diabetic CKD patients showed that Combination therapy with Aliskiren and ARB was effective but was not safe as it was associated with a high prevalence of hyperkalaemia.     

Evaluating the severity of dermatologic disorders

Assessment of a patient's disease severity is an essential component of formulating therapeutic strategies. However, disorders of the skin are often not amenable to strict classification criteria, and the dermatologist relies upon personal thresholds of severity when assessing the patient's overall condition. A number of grading systems have arisen, primarily from the need for standardized end points in clinical trials; in some circumstances, these severity assessments may assist the clinician in the evaluation and treatment of dermatologic disease. In this review, we will summarize the results of available severity scores of frequently encountered dermatologic disorders and discuss their utility in the management of disease in a clinician's office.     

IgG4相关的硬化性疾病

IgG4相关硬化性疾病是近年被认识的一种疾病,被认为与自身细胞、体液免疫反应异常有关。患者血清IgG4水平升高,病变器官纤维化,并有显著IgG4阳性浆细胞浸润。病变可以累及许多器官,部分病例伴有皮肤的病变。皮肤病变以头颈部多见,可为红斑、斑片或皮下结节,有痒感。IgG4相关皮肤疾病糖皮质激素治疗有效。     

Allopurinol induced generalized eosinophilic pustular folliculitis

A 71-year-old Cambodian man who was commenced on allopurinol for the treatment of gout developed a generalized papulopustular follicular eruption 8 weeks following introduction of the drug. The skin biopsy findings were consistent with that of eosinophilic pustular folliculitis. Resolution of the rash took place during the 8 weeks following cessation of allopurinol and treatment with oral and topical corticosteroids.     

莱特勒-西韦病

报告1例莱特勒-西韦病（非类脂组织细胞增多症）。患儿男,2个月。出生时颈部及下肢即有棕黄色丘疹、斑丘疹、鳞屑及痂皮,逐渐累及头面部、躯干部及四肢,皮损反复发作。皮损印片可见大量泡沫样组织细胞。     

Macro‐micro dermatology: Erythematous papules and lentigo‐like macules – a new entity?

Galli‐Galli disease is usually characterized by reticulate hyperpigmentation of the flexures. Rare patients may present without this finding, instead having only localized erythematous papules and brown, lentigo‐like macules on the trunk and extremities. Histological changes consist of elongated and in part hyperpigmented rete ridges and areas of acantholysis.