嗜酸性脂膜炎国内首次报告

嗜酸性脂膜炎是一种较难确诊的具有多种临床表现的疾病。笔者报告 3例临床表现各异的男性患者 ,组织病理检查均表现为皮下组织有大量的嗜酸性粒细胞浸润。通过查阅文献 ,这是国内首次对嗜酸性脂膜炎的病例报告     

嗜酸细胞性脂膜炎1例

报告1例嗜酸细胞性脂膜炎,患者男,33岁,因双下肢皮损伴肿胀2个月,症状加重伴发热10余天就诊,皮损组织病理检查示皮下脂肪层有弥漫性嗜酸粒细胞为主的浸润,根据临床表现及组织病理所见,诊断为嗜酸细胞性脂膜炎,经抗生素和糖皮质激素治疗后皮损痊愈。     

Eosinophilic Panniculitis

Abstract: Eosinophilic pannicuiitis is a pooriy defined entity with variabie ciinicai features. We report a case of rapidiy eniarging, asymptomatic subcutaneous scalp nodules in a 6-year-old biack boy with atopic dermatitis. The noduies resolved spontaneously over two to three days. Biopsy specimens were remarkable for eosinophilic pannicuiitis without evidence of epidermal change or vasculltis. We beiieve that this is the youngest reported patient with this disorder.     

Panniculitis in childhood

Panniculitis refers to disorders with inflammation of the subcutaneous fat. Such inflammation can be primary or can be a reaction pattern induced by a systemic process. Some types of panniculitis are seen more commonly or exclusively in children. These include erythema nodosum, subcutaneous fat necrosis of the newborn, sclerema neonatorum, poststeroid panniculitis, and cold panniculitis. The most typical clinical finding is tender, erythematous subcutaneous nodules. Clinical clues can aid in the diagnosis of the panniculitides, but pathology is often necessary to confirm the diagnosis. In general, the pediatric panniculitides are treated with supportive care and management of any underlying disorders, but certain types such as infectious panniculitis and malignancy‐related panniculitis require more specific therapies.     

Panniculitis with Eosinophilic Infiltration Due to Gabexate Mesilate (FOY®): Possibility of Allergic Reaction

A 59-year-old Japanese man developed septal panniculitis with eosinophilic infiltration in both forearms and the dorsum of the left hand after a gabexate mesilate intraveous drip infusion for acute pancreatitis through catheters implanted in these sites. Gabexate mesilate at a dose of 1000 mg per day had been given continuously for 8 days, and antibiotics were added by the same infusion route twice a day. All the infusion routes, however, became occluded one after the other. Reddish swelling first occurred at the left wrist 6 hours after occlusion of the infusion route, and, on both forearms, reddish swelling occurred about one week after the occlusion of each route. Patch testing revealed a ++ reaction to gabexate mesilate (10% pet) at days 3 and 7, and skin testing revealed indurated erythema to gabexate mesilate (0.1% aq) at days 2 and 3. The specimens biopsied from the positive skin testing reaction sites showed perivascular infiltrate and slight septal panniculitis. The inflammatory infiltrate consisted predominantly of lymphocytes with small numbers of eosinophils. Staining of the specimen biopsied from the right forearm lesion with anti-eosinophil cationic protein (ECP) antibodies (EG1 and EG2) showed deposition of eosinophil-derived granule proteins at the damaged septal connective tissues of the panniculitis. The panniculitis improved with topical steroid treatment. This case suggested that the concentration of infused gabexate mesilate may have been high enough to damage blood vessels and that gabexate mesilate may have leaked into the surrounding connective tissues, inducing allergic reactions and resulting in leions.     

Panniculitis showing membranocystic changes in the dermatomyositis.

Clinically obvious panniculitis in association with dermatomyositis is rare. We reported a patient with panniculitis showing membranocystic changes in the dermatomyositis.     

Panniculitis with crystals induced by etanercept subcutaneous injection

Panniculitis with lipid crystallization within adipocytes may be seen in several disorders, including crystal‐storing histiocytosis, gouty panniculitis, subcutaneous fat necrosis of the newborn, post‐steroid panniculitis, sclerema neonatorum, oxalosis and subcutaneous fungal infections by mucormycosis, zygomycosis or aspergillosis. Panniculitis at the sites of subcutaneous injection of drugs are frequent, but to our knowledge no crystals have been described in the drug‐induced panniculitis at the sites of subcutaneous injections. We report on a patient who developed a panniculitis with lipid crystallization at the site of etanercept injection.     

嗜酸细胞性脂膜炎1例

报告1例嗜酸细胞性脂膜炎。患者女,40岁。因骶尾部溃疡6个月,周围皮肤变硬4个月就诊。溃疡部位组织病理学检查示真皮层脂肪小叶间密集的嗜酸性粒细胞和淋巴细胞浸润。诊断为嗜酸细胞性脂膜炎。     

以脂膜炎为皮肤表现的皮肌炎1例

报告1例以脂膜炎为皮肤表现的皮肌炎。患者男,20岁。14岁起四肢近端肌力下降,伴面部红斑。1年后大腿出现疼痛性结节,伴吞吐咽困难。组织病理学检查示小叶性脂膜炎伴淋巴细胞,浆细胞浸润。经参剂量糖皮质激素及环磷酰胺、静脉滴性丙种球蛋白治疗后肌炎及脂膜炎病情均缓解,提示脂腊炎可能是炎症性肌病的一个皮肤特征。     

An Unusual Panniculitis Appearing in the Winter with Good Response to Tetracycline

Cold panniculitis is a form of physical panniculitis due to exposure of skin to severe cold. It usually appears on the cheeks of infants and children. It has also been reported on the thighs and buttocks of young females. Its clinical manifestations include red, cold, indurated plaques or nodules which appear one to three days after exposure to low temperatures and resolve spontaneously within several weeks without scarring. The histopathological picture shows a perivascular infiltrate of lymphoid and histiocytic cells at the dermal-subcutaneous junction in the early phase of the reaction (1). After 48 to 72 hours, a well developed panniculitis appears. We report an unusual case of an adult female patient with recurrent panniculitis on her legs appearing in the winter but without any preceding repeated or prolonged exposure to cold. She responded dramatically to oral tetracycline. This drug was successful as a prophylactic agent as well.     

嗜酸性脓疱性毛囊炎

嗜酸性脓疱性毛囊炎主要可分为3种类型，即经典型、免疫抑制相关型和婴儿型，许多患者的发病与艾滋病有关。该文就嗜酸性脓疱性毛囊炎的分类、流行病学、发病机制、临床表现、实验室检查、鉴别诊断和治疗等各方面作一较全面的介绍。     

硬化性脂膜炎5例临床分析

目的了解硬化性脂膜炎的临床特征,提高对本病的认识和诊断。方法对5例确诊的硬化性脂膜炎患者的临床资料进行相关分析。结果硬化性脂膜炎为好发于女性的慢性脂膜炎,表现为下肢皮肤结节或斑块,晚期出现硬化、萎缩、凹陷或明显色素沉着。病理特征为皮下脂肪层的脂肪小叶内见组织细胞和泡沫样细胞,微囊肿形成,脂肪间隔纤维组织增生,胶原增粗硬化。结论硬化性脂膜炎临床较少见,容易误诊,加强对硬化性脂膜炎的认识,有助于减少误诊,明确诊断。     

Lupus Panniculitis Treated by a Combination Therapy of Hydroxychloroquine and Quinacrine

Lupus erythematosus panniculitis (LEP) is an unusual clinical variant of lupus erythematosus (LE) in which the cutaneous inflammatory reaction occurs primarily in the deeper corium. The common clinical features of LEP includes asymptomatic, firm, sharply defined nodules. The histologic findings are characterized by nonspecific panniculitis composed of lymphoid cells, plasma cells, and histiocytes with varying degrees of necrobiotic changes with fibrinoid deposits. In our case, a 24-year-old male patient visited our clinic with non-tender, hard, plaquelike lesions and overlying erythema on the left zygomatic, nasal, and submandibular area. Histopathologic and direct immunofluorescent findings of the lesion were compatible with LEP. His skin lesions waxed and waned with systemic steroid or hydroxychloroquine therapy. He has responded well to a combination therapy of hydroxychloroquine and quinacrine.     

Neutrophilic Panniculitis in a child with MYSM1 deficiency

Neutrophilic panniculitis (NP) with myelodysplasia has been described in adults but not in children. We report a case of NP associated with myelodysplasia in a child with MYSM1 deficiency, a newly described syndrome with primary immunodeficiency (PI), bone marrow failure, and developmental aberrations.     

狼疮性脂膜炎8例临床分析

目的:了解狼疮性脂膜炎的临床特征,提高对本病的认识.方法:回顾性分析在我中心皮肤科门诊收治的8例确诊为狼疮性脂膜炎的患者的临床、组织病理特点及治疗预后情况.结果:皮损好发于头面部、上肢及臀部,临床表现为皮肤萎缩、红斑、皮下结节、浸润性红斑及皮肤破溃.病理的典型改变为皮下脂肪层出现淋巴细胞浸润为主的小叶性脂膜炎.治疗以硫酸羟氯喹单用或合用激素为主,大多数患者对治疗反应较好.结论:狼疮性脂膜炎较少见,其典型的临床和病理特征是诊断的要点,硫酸羟氯喹及中小剂量糖皮质激素治疗有效.     

Eosinophilic dermatosis of hematologic malignancy

Cutaneous involvement by an eosinophil-rich process (eosinophilic dermatosis) may be encountered in the setting of various hematologic malignancies, including mantle cell lymphoma, acute monocytic leukemia, acute lymphoblastic leukemia, large cell lymphoma, myelofibrosis and chronic lymphocytic leukemia (CLL). Of the various hematologic malignancies, eosinophilic dermatosis has been most frequently described in association with CLL. Published previously as insect bite-like reaction and eosinophilic dermatosis of myeloproliferative disease, this rare dermatitis presents as a pruritic, papular and occasionally vesicular eruption associated with an eosinophil-rich infiltrate histopathologically. Although clinical and histopathologic features are similar to insect bites, affected patients frequently deny a history of insect bites. We report a case of eosinophilic dermatosis of hematologic malignancy in a patient with known history of CLL.     

Eosinophilic pustular folliculitis: A review of the Japanese published works

Eosinophilic pustular folliculitis (EPF), also known as Ofuji's disease, is an inflammatory dermatosis that was first described in Japan in 1970. More than 300 cases have been reported so far, and 113 Japanese cases have been reported in Japan since 1980. To comprehend the characteristics of Japanese EPF cases, we classified these cases into three types: classic, immunosuppression‐associated (IS‐EPF), and infancy‐associated (I‐EPF). Trends in age of onset and in distribution and characterization of eruptions differed between the types. We found 91 cases of classic EPF (mean age, 39.7 years), consisting of 66 males (73%) and 25 females (27%), in most of which eruptions primarily affected the face; 18 cases of IS‐EPF (44.2 years), consisting of 15 males (83%) and three females (17%), in which eruptions affected the face less predominantly; and four cases of I‐EPF (7.0 years), consisting of two males (50%) and two females (50%), primarily affecting the scalp. The number of IS‐EPF cases has increased since the late 1990s, reflecting the increasing number of HIV‐positive patients in Japan. Systemic non‐steroidal anti‐inflammatory drugs were effective in more than 70% of cases. Dimethyl diphenyl sulfone, antibiotics including minocycline, psoralen plus ultraviolet A therapy and ultraviolet B treatments worked in some cases. Topical steroids and tacrolimus were also effective in some cases of EPF, while topical indomethacin was less effective.     

伴嗜酸粒细胞浸润的胰腺性脂膜炎一例

患者,女,64岁。双下肢暗红色结节、斑块伴疼痛20余天。组织病理示：脂肪层见鬼影细胞,脂肪细胞间较多嗜酸粒细胞、少许中性粒细胞、淋巴细胞浸润。PET-CT示：1.胰尾部占位,考虑胰腺(原发病灶)恶性肿瘤;2.肝内多发转移瘤;3.腹股沟淋巴结转移可能。肝穿刺组织病理提示来自消化系统的转移性腺癌。诊断：胰腺性脂膜炎(胰腺癌伴肝转移)。目前化疗治疗中。对于临床上出现下肢疼痛性结节、斑块的患者,应详细询问病史,完善相关影像学检查及皮肤组织病理检查,避免胰腺性脂膜炎的漏诊、误诊。     

Panniculitis caused by progesterone injection can be treated by physical therapy

A method for the treatment of panniculitis caused by progesterone injection is introduced. Sixteen patients achieved good results. This is a 9‐year single center retrospective study. Of all the 5633 patients who received progesterone injection, 16 developed panniculitis at the injection site. Pathological examination confirmed the occurrence of panniculitis. The patient received physical therapy. These treatments are determined by the course of the patient. Compared with patients without panniculitis, patients with panniculitis received more than one injection of progesterone. In 16 patients, symptoms and local signs disappeared completely in 15 patients. One patient did not take physical therapy according to the doctor's advice after the treatment improved. However, 1 month later, the patient went to see the doctor again and received the relevant physical therapy, and still achieved good results. Progesterone injection may lead to panniculitis, which is rare but may cause serious consequences. Physical therapy can be effective.