毛霉病七例临床与病理分析

目的 探讨毛霉病的临床表现、预后和病理表现之间可能存在的关系。方法 回顾性分析1989-2006年我院诊治的7例经真菌培养证实为毛霉病患者的临床资料和病理切片。结果 7例中鼻脑毛霉病1例、皮肤毛霉病6例，其中2例为毛霉性坏死性筋膜炎。鼻脑毛霉病和毛霉性坏死性筋膜炎患者病情进展快，均以死亡告终；其皮损组织病理表现呈混合性炎症细胞浸润，坏死明显，可见大量菌丝和侵血管现象。4例以浸润性红斑为主要表现的皮肤毛霉病患者病情进展慢，有2例已治愈；其皮损组织病理呈肉芽肿性炎症，菌丝较少，未见明显的侵血管现象。7例均未见明确的菌丝侵神经现象。结论 毛霉病皮损组织病理表现呈混合性炎症细胞浸润，菌丝多，出现明显侵血管现象者预后较差。     

原发性皮肤毛霉病

报告1例原发性皮肤毛霉病.患者男,57岁.右前臂埋置静脉套管针17 d后局部出现红肿,迅速坏死并扩大.患者患2型糖尿病及慢性肾功能不全.皮肤科检查示右前臂大片坏死区,上覆黑色焦痂及渗出.取黑痂及渗液行真菌镜检,镜下可见大量粗大、无分隔、垂直分枝的菌丝,培养第2天即有棉花糖样菌落生长,初步诊断为皮肤毛霉病.皮损行组织病理检查,PAS染色示真皮及皮下组织可见较多粗大、无分隔菌丝,确诊为皮肤毛霉病.给予两性霉素B脂质体泵入、坏死组织清创术、术后外敷两性霉素B脂质体溶液及外用人成纤维细胞生长因子.患者伤口愈合良好,随访4个月无复发.     

The first case of cutaneous mucormycosis caused by Rhizopus azygosporus

A rapidly enlarging leg ulcer appeared in a 54-year-old woman with systemic lupus erythematosus receiving aggressive immunosuppressive therapy. Skin biopsy revealed proliferation of hyphae in the midst of a neutrophilic abscess. Culture yielded Rhizopus azygosporus. As no organ involvement was detected by thorough examination, the patient was diagnosed as having primary cutaneous mucormycosis. Although intravenous amphotericin B therapy seemed to be very effective, it had to be discontinued due to nephrotoxicity. She unfortunately died of subsequent disseminated fungal infection and cerebral infarction in which the primary cause could not be determined. Minimum inhibitory concentrations of several antifungal drugs to the isolate were examined and amphotericin B proved to be the only agent that may potentially reach the effective plasma concentration. This is the first case report of cutaneous mucormycosis caused by R. azygosporus.     

Primary cutaneous mucormycosis in a trauma patient

We report a rare case of primary cutaneous mucormycosis caused by Rhizopus oryzae that occurred in an immunocompetent trauma patient. The patient had encrusted erythematous plaques with pustules on the left shin, which had been abraded in a traffic accident. Histologic examination revealed widespread granulomatous inflammation and characteristic broad, non-septate hyphae with right-angle branching in the dermis. The infection was cured with intravenous amphotericin B therapy.     

多变根毛霉致原发性皮肤毛霉病1例及分子生物学鉴定

目的：报告多变根毛霉致原发性皮肤毛霉病1例及其诊断过程。方法：对1例面部进行性溃烂伴疼痛2年,加重3月的37岁男性患者进行了皮损真菌镜检、培养、组织病理检查,并提取真菌DNA,以真菌通用引物ITS4和ITS5进行PCR扩增。结果：直接镜检及组织病理均发现透明宽大无隔菌丝;真菌培养25℃7天于沙氏培养基（SDA）上生长黄色绒毛状菌落,镜下见透明无隔宽大菌丝,呈直角分叉,见孢子囊及假根。PCR扩增真菌DNA,获得614bp片段,经比对与多变根毛霉具有99%同源性。结论：该病例诊断为＂多变根毛霉所致原发性皮肤毛霉病＂。多变根毛霉可致原发性皮肤毛霉病,分子生物学方法在鉴定真菌致病菌种方面具有准确、快速的作用。     

国内首例总状共头霉所致原发性皮肤毛霉病及文献复习

目的:报道1例总状共头霉所致的原发性皮肤毛霉病并进行文献复习。方法:患者,女,67岁。反复右踝结节、渗液伴疼痛3年。取患者皮损进行真菌镜检及培养,对皮损进行组织病理检查,并对分离菌种行r DNA ITS测序鉴定。结果:显微镜下可见宽而无分隔、成直角分支的菌丝。培养见白色菌落生长,生长较快,菌落呈疏松絮状,灰褐色绒毛状,边缘呈羊毛状向外延伸。皮损组织病理示:真皮全层弥漫性炎细胞浸润,散在多核巨细胞,可见大量团块状菌丝。PAS和嗜银染色阳性。分子测序鉴定为总状共头霉。确诊后外科结合伊曲康唑等治疗取得良好效果。结论:该病例致病菌为总状共头霉,外科治疗结合伊曲康唑治疗有效。     

Primary cutaneous mucormycosis caused by Mucor irregularis in an elderly person

Primary cutaneous mucormycosis is a rare but often lethal severe fungal infection, which usually occurs in immunocompromised patients. We report a case of primary cutaneous mucormycosis caused by Mucor irregularis in an elderly patient. Seven months after the surgical dissection of the involved skin, cutaneous mucormycosis recurred at the peripheral edge of the skin graft. Shortly subsequent to the administration of liposomal amphotericin B, the remaining skin lesion was excised again. M. irregularis is rarely but increasingly reported as a cause of mucormycosis in immunocompetent individuals, especially in Asian farmers. M. irregularis may be largely disseminated in the soils of Asia and thus the trivial trauma at the time of farm work may be a trigger for the onset. These cases tend to leave severe cosmetic damage even in healthy individuals, although the vital prognosis is not affected.     

伊曲康唑治疗1例原发性皮肤毛霉病

应用伊曲康唑治疗１例罕见的由冻土毛霉黄色（或土黄）型所致的原发性皮肤毛霉病,并对其治疗前后的临床、真菌学及组织病理改变进行了观察。临床、真菌学痊愈。斯皮仁诺对毛霉目某些真菌是敏感的,对原发性皮肤毛霉病的治疗是安全有效的。     

Lymphoepithelioma-like carcinoma of the skin with spindle cell differentiation

BACKGROUND: Primary cutaneous LELC is a cutaneous neoplasm with histopathologic features identical to those seen in the undifferentiated subtype of nasopharyngeal carcinoma. It is extremely rare, with only approximately 30 cases reported in the literature. METHODS: We report a case of primary cutaneous LELC arising on the forehead of a 72 year-old male in which a proportion of the neoplastic cells demonstrated distinctive spindle cell morphology. RESULTS: Microscopic examination showed a dense lymphoplasmacytic infiltrate admixed with large spindle-shaped cells with vesicular nuclei, prominent nucleoli, and frequent mitotic figures. These cells were negative for an extensive panel of immunohistochemical markers and positive only for broad-spectrum cytokeratins and epithelial membrane antigen. There was no connection between the tumor and the epidermis and no epidermal dysplasia. In situ hybridization for Epstein-Barr virus was negative. CONCLUSIONS: The spindle cell differentiation in this case is unusual and suggests that in some cases the differential diagnosis of cutaneous spindle cell neoplasms might include primary cutaneous LELC.     

Primary Cutaneous Mucormycosis in an Extremely Preterm Infant Successfully Treated with Liposomal Amphotericin B

Cutaneous mucormycosis is a rare but often fatal invasive fungal infection that occurs most commonly in patients with diabetes, malignancy, and other immunocompromising conditions. We report an extremely preterm (<28 weeks) baby boy who developed polymicrobial sepsis and primary cutaneous mucormycosis within his first 10 days of life. He was successfully treated with medical management alone since he was not a candidate for surgery. Successful treatment of cutaneous mucormycosis without surgical debridement has been reported on only two other occasions. This case highlights the importance of rapid and thorough evaluation of skin lesions when evaluating preterm infants and other immunocompromised patients, even when other sources of infection have been identified.     

Successful treatment of a patient with cutaneous co-infection caused by Mucor irregularis and Klebsiella pneumoniae

The authors report a rare case of primary cutaneous mucormycosis caused by Mucor irregularis and cutaneous Klebsiella pneumoniae infections in a 67-year-old Chinese woman. After the administration of liposomal amphotericin B combined with cefoperazone/sulbactam sodium, the patient recovered. Invasive fungal infection combined with cutaneous bacterial infection should receive attention.     

皮肤T细胞淋巴瘤并发原发性皮肤曲霉病1例

报道1例皮肤T细胞淋巴瘤患者并发上曲霉引起的原发性皮肤曲霉病，患者女性，41岁，左胫前2处相连的巨大黑痂，刮取物直接镜检有透明的分枝分隔菌丝，组织病理检查见较多粗细均匀的有隔菌丝，菌丝呈Y型分枝，菌种鉴定为土曲霉。     

Epidermotropically metastatic malignant melanoma. Differentiating malignant melanoma metastatic to the epidermis from malignant melanoma primary in the epidermis

In four instances, metastases to epidermis from primary cutaneous malignant melanomas at different sites showed histological features similar to those of cutaneous malignant melanoma primary in the epidermis. In these metastases, atypical melanocytes were present within the epidermis and in the upper part of the dermis much as in primary cutaneous malignant melanoma. Therefore, the presence of atypical melanocytes within the epidermis is not in itself an absolute criterion of malignant melanoma primary in skin. Nor does that finding absolutely deny malignant melanoma metastastic to the skin. Features that may enable histologic differentiation of epidermotropically metastatic malignant melanoma from primary cutaneous malignant melanoma are emphasized.     

Primary cutaneous mucormycosis: a diagnosis to consider

Primary cutaneous mucormycosis is a deep fungal infection, mainly seen in diabetics and immunocompromised subjects. Rapid diagnosis and therapy are necessary to avoid fatal outcome. We describe the complete histopathological and microbiological studies of primary cutaneous mucormycosis in a 74-year-old man with several risk factors, such as chronic obstructive pulmonary disease, respiratory acidosis, hemolytic anemia, myelodysplastic syndrome and iatrogenic diabetes, due to corticosteroid therapy. He developed two cutaneous necrotic scars on his left leg. Mucormycosis was suspected and specimens from surgical débridement were histopathologically and microbiologically studied confirming the clinical diagnosis. Amphotericin B was given topically and intravenously resulting in complete healing of the ulcer. Risk factors and microbiological studies are compared with those in the current literature. It is necessary in certain cases to suspect mucormycosis infections in diabetics, immunocompromised subjects and even in healthy individuals. Rapid diagnosis and treatment are important, but they should be based on complete histopathological and microbiological studies, to establish the genus of the causal agent.     

Subcutaneous alternariosis

Alternaria species are common plant pathogens, but a rare cause of human infection. Previously reported cases of cutaneous alternariosis (Alternaria alternata) in both healthy and immunosuppressed hosts have been characterized by a chronic localized ulcerative eruption in exposed sites. Dermal granulomatous infiltration and variable epidermal changes, with hyphae in both the dermis and epidermis, are characteristic. We present a case with certain unique features. This is the first reported human infection with Alternaria dianthicola. In addition, our patient showed the unusual presentation of clinically noninflammatory subcutaneous nodules of the chest wall. The subcutaneous location of the granulomatous process may have been attributable to deep traumatic inoculation. The circumscribed nodular quality of the infection allowed a surgical therapeutic approach.     

Pseudoepitheliomatous hyperplasia secondary to cutaneous aspergillus

Cutaneous aspergillosis commonly occurs in immunocompromised hosts and may also complicate burn wounds. Pseudoepitheliomatous hyperplasia (PH) is a histologic reaction secondary to a wide range of stimuli, including fungal infection. We describe a case of an 18-year-old man, status-post burns over 70% of his total body surface area, with cutaneous aspergillosis of the axilla and secondary PH. A single case of PH secondary to primary aspergillosis has been described in the larynx but, to our knowledge, has never been described cutaneously. Histologic examination of the lesion reveals an irregularly acanthotic epidermis with deep invaginations within the dermis. There is an intense inflammatory reaction within the superficial and deep dermis. Numerous fungal forms are identified within the dermis. Special stains demonstrate septate hyphae with dichotomous branching, which is morphologically consistent with Aspergillus. Therefore, we conclude that cutaneous aspergillosis should be included in the differential diagnosis of causes of PH, especially in a patient population at risk for this infection.     

Zigomicosis (mucormicosis) cutánea en paciente con leucemia

—Cutaneous zygomycosis (mucormycosis) is a rare disease caused by opportunist fungi classified as Zygomycetes; it associates in most of the cases to uncontrolled diabetes mellitus, and secondly with hematological diseases specially leukemia.We report the case of a 21 year-old female with type L2 acute lymphoblastic leukemia, who presented on her nose an indurated, black-purple, necrotic lesion, which extended to the hard palate and paranasal sinuses. She was treated with usual doses of amphotericin B, with poor response at all she died.We discussed if this case was a primary cutaneous case or secondary to a rhinocerebral form of presentation.     

The emergence of mucormycosis as an important opportunistic fungal infection: five cases presenting to a tertiary referral center for mycology

BACKGROUND: Mucormycosis, a rare opportunistic fungal infection, is re-emerging in importance with the increase in prevalence of immunosuppressive states, both as a result of therapy and disease. METHODS: We report five cases of mucormycosis diagnosed by the Dermatology Department and managed jointly with the Medical and Surgical Services of "Dr Manuel Gea Gonzalez" General Hospital in Mexico City, a tertiary referral center for mycology. We also review the current literature including recent advances in medical therapy. RESULTS: Four of the five cases were of the rhino-orbital-cerebral variant, commonly associated with significant mortality, and one of these patients died despite early diagnosis and aggressive management. The fifth case was primary cutaneous mucormycosis and this patient survived infection without relapse. Diabetic ketoacidosis predisposed to infection in four cases and the other was associated with advanced human immunodeficiency virus infection. Radiologic imaging was important in cases of facial involvement in order to evaluate the extent of disease and possible intracranial involvement. All cases were managed with systemic antifungals and surgical debridement, together with the treatment of predisposing factors. CONCLUSIONS: These cases illustrate the need for early clinical recognition and prompt therapy, as well as the requirement for tissue biopsy in order to demonstrate the characteristic morphologic features of this fungal agent in the absence of positive mycology culture results. This report also highlights that, although rhino-orbital-cerebral mucormycosis requires effective multidisciplinary management, the disease not uncommonly presents to dermatologists for diagnosis.     

不规则毛霉感染引起的皮肤毛霉病20例回顾性分析

目的通过对不规则毛霉感染病例的回顾性分析,探讨不规则毛霉感染引起皮肤毛霉病的流行病学、易感因素、临床表现、诊断方法及治疗策略,提高临床医生的警惕性及诊治经验。方法对检索到的20例不规则毛霉感染病例资料进行总结、分析。结果 20例不规则毛霉感染患者均表现为皮肤毛霉病,仅1例同时伴有肺部感染。15例来自中国,1例有糖尿病史,1例有白血病史,10例有外伤、手术或叮咬史。皮损几乎均出现在暴露部位,其中鼻面部13例,四肢7例,病程多呈慢性。早期表现为红斑、丘疹、结节,后期可发展成溃疡、坏死、黑痂及骨质破坏。经真菌镜检、培养、组织病理、ITS区测序等检查证实为不规则毛霉。两性霉素B及其脂质体单独或联合伊曲康唑治疗预后好。结论不规则毛霉感染多发生于免疫正常者,外伤、烧伤、手术及叮咬是最主要诱发因素。感染主要累及鼻面部及四肢皮肤暴露部位,以红斑、坏死为主要表现,极少累及内脏。真菌培养及组织病理检查可以诊断该病,ITS区测序可明确鉴定到种。及早诊断并给予清创及两性霉素B是治疗成功的关键。     

Osteomyelitis secondary to cutaneous mucormycosis. Report of a case and a review of the literature

An unusual case of osteomyelitis secondary to cutaneous mucormycosis developed in a diabetic with chronic renal insufficiency. Recovery followed curettage of the osseous lesion and "suboptimal" treatment with amphotericin B. A review of the literature on mucormycosis of bone and skin follows the report of the case.