Panniculitis in juvenile dermatomyositis: Report of a case and review of the published work

We report a 15‐year‐old girl who presented with indurated, subcutaneous nodules in addition to classical findings of juvenile dermatomyositis. Histopathological examination confirmed the diagnosis of panniculitis associated with juvenile dermatomyositis. Considering that panniculitis is a rare cutaneous manifestation of juvenile dermatomyositis, we present a patient with a brief review of the published work to highlight the importance of keeping juvenile dermatomyositis in mind among the etiologies of pediatric panniculitis.     

Panniculitis in Juvenile Dermatomyositis

Panniculitis is a rarely reported clinical finding in dermatomyositis. We present a 14-year-old African American boy with a 4-year history of dermatomyositis referred for evaluation of tender, indurated plaques and nodules on the trunk and proximal extremities. A biopsy specimen revealed epidermal and dermal changes consistent with dermatomyositis. Although calcification was absent, a striking lobular panniculitis was observed. A total of seven cases of clinical panniculitis in association with dermatomyositis have been published. As in our patient, it typically presents as indurated, tender plaques and nodules on the arms, thighs, and buttocks. Although in the vast majority of cases panniculitis found in association with dermatomyositis is subclinical and represents an incidental histopathologic finding, the case we present further emphasizes the need to recognize panniculitis as a rare, but clinically relevant pathologic feature seen in certain lesions of juvenile dermatomyositis.     

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。     

以脂膜炎为皮肤表现的皮肌炎九例研究

目的 探讨以脂膜炎为皮肤表现的皮肌炎的临床、病理特点.方法 收集2012年10月至2016年7月复旦大学附属中山医院皮肤科诊治的9例以脂膜炎为皮肤表现的皮肌炎患者的资料,分析其临床与病理特征.结果 9例中女6例,男3例,年龄28～73岁.9例患者脂膜炎皮损均表现为触痛性斑块或结节,分别发生在臀、股、腰、背、腹、上肢和腮部,先于、晚于或与皮肌炎特征性皮损和肌肉损害同时发生,其中1例脂膜炎先于皮肌炎特征性皮损30年发生.皮损组织病理示基底细胞液化变性,真皮血管周围及脂肪小叶和间隔内以淋巴细胞、浆细胞为主的炎性浸润,可有脂肪细胞坏死、钙化和膜囊性改变,血管壁纤维素样坏死和管腔闭塞.脂膜炎先于皮肌炎特征性皮损发生的2例患者曾被数次误诊为狼疮性脂膜炎和深部硬斑病.多数患者对联用糖皮质激素和免疫抑制剂的治疗反应良好,但伴脂肪膜性坏死者,对治疗反应差.结论 皮肌炎的脂膜炎表现具有一定组织学特征,与皮肌炎典型皮损的发生可不同步,在皮肌炎典型损害前出现易被误诊,持续跟踪随访对确诊具有重要意义.     

Dermatomyositis panniculitis: A case report

Dermatomyositis‐related panniculitis is a rare cutaneous manifestation of dermatomyositis. There are few reported cases in the medical literature. We present the case of a 60‐year‐old woman with a 2‐year history of dermatomyositis and recent biopsy‐confirmed panniculitis treated with prednisone, cyclophosphamide and i.v. immunoglobulin.     

Panniculitis in a patient with dermatomyositis

Panniculitis is a rarely reported clinical finding in dermatomyositis. It may precede the other manifestations associated with dermatomyositis by as much as 14 months. In all cases, myositis and panniculitis improve simultaneously during treatment. The present report describes the case of a 30-year-old female patient with clinical and histopathological findings consistent with panniculitis two months after the onset of the muscle and cutaneous symptoms that permitted diagnosis of dermatomyositis. The skin lesions regressed following steroid treatment.     

Connective tissue panniculitis: lupus panniculitis,dermatomyositis, morphea/scleroderma

Panniculitis is an uncommon cutaneous manifestation of connective tissue diseases. Our discussion will include panniculitis occurring in the setting of lupus erythematosus, dermatomyositis, and scleroderma/morphea. These subtypes of panniculitis are unified by an active inflammatory stage of the disease that can progress to develop scarring, atrophy, and calcifications. Treatment is most effective if initiated during the active phase of the disease and often requires systemic therapy because of the location of the inflammation. Antimalarials are the initial treatment of choice for most cases of lupus erythematosus panniculitis, whereas corticosteroids in combination with other steroid‐sparing immunosuppressive agents are the first‐line treatment for panniculitis in patients with dermatomyositis. The appropriate treatment for panniculitis in the setting of morphea/scleroderma varies based on clinical severity.     

Subcutaneous changes in dermatomyositis

We report the case of a 42-year-old woman with concomitant panniculitis and dermatomyositis. Painful, indurated lesions on the buttocks, thighs, arms, abdomen and breasts were associated with proximal muscle weakness. Skin biopsy revealed lobular panniculitis, and vacuolar degeneration of epidermal basal cells. Direct immunofluorescence was negative. Serum muscle enzyme (creatine-phosphokinase) levels were elevated, and electromyography demonstrated a myositic process. Muscle biopsy showed an inflammatory myositis. These results were consistent with dermatomyositis associated with panniculitis. Only tive cases of this association have been reported previously. The relationship between these two conditions is discussed.     

Vesiculobullous dermatomyositis with panniculitis without muscle disease

Vesicles and bullae formation is rare in dermatomyositis. We describe a 60-year-old woman who presented with vesiculobullous dermatomyositis with panniculitis and no muscle disease.     

Dermatomyositis treated with high-dose intravenous immunoglobulins and associated with panniculitis

Polymyositis and dermatomyositis are idiopathic inflammatory myopathies characterized by subacute symmetrical weakness of proximal limb and trunk muscles. Dermatomyositis is distinguished from polymyositis by the presence of rash.^1,2 We describe an adult patient with treatment-resistant childhood-type dermatomyositis who made a good response to high dose intravenous immunoglobulins. Additionally, there was evidence of panniculitis which is an unusual histopathological finding in dermatomyositis.     

Lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy

An 11-year-old girl presented with a one-year history of multiple, hard, slightly painful subcutaneous nodules on her right cheek, upper arms, and buttock. Histology of a skin biopsy specimen showed a lobular panniculitis. Laboratory studies revealed positive ANA, anti-double strand DNA, and elevated muscle enzymes. She was diagnosed as having lupus panniculitis. During hydroxychloroquine treatment, erythema over knuckle joints developed. These unusual clinical and laboratory findings of panniculitis associated connective tissue diseases made it difficult to make a precise diagnosis. We report this unusual case of lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy.     

Update on management of connective tissue panniculitides

In connective tissue diseases, panniculitis can be the sole manifestation or can occur along with the underlying disease process. The best described forms of connective tissue panniculitis are lupus erythematosus panniculitis and lupus profundus, panniculitis associated with dermatomyositis, and morphea- and scleroderma-associated panniculitis. These processes cause significant morbidity, such as deep atrophic scars, cosmetic disfigurement, and psychiatric sequelae. Because the inflammation is located in the subcutaneous adipose layer, topical therapies may not penetrate enough to be effective, and systemic agents are required. Despite the large number of reported cases and therapies, recommendations for treatment are based largely on case series and expert opinion due to a lack of controlled therapeutic trials. All treatments are off-label in the United States. The lack of validated clinical outcome measures makes systematic and controlled studies difficult. Nonetheless, further investigation into the most effective therapies for these conditions is needed.     

Panniculitis associated with amyopathic dermatomyositis

Panniculitis is a rare clinical finding in dermatomyositis, with less than 30 cases reported and there is only one case associated with the amyopathic subtype described in the literature. The present report describes a 49-year-old female patient that one year after being diagnosed with amyopathic dermatomyositis, presented indurated, painful, erythematous to violaceous nodules located on the upper limbs, thighs and gluteal region. Skin biopsy revealed lobular panniculitis with a lymphocytic infiltrate. The patient was treated with prednisone and methotrexate, but remained unresponsive to treatment.     

Panniculitis. Part II. Mostly lobular panniculitis

The second part of our review of panniculitis summarizes the clinicopathologic features of the mostly lobular panniculitides. Erythema induratum of Bazin (nodular vasculitis) represents the most common variant of lobular panniculitis with vasculitis, although controversy persists about the nature of the involved vessels. Mostly lobular panniculitides without vasculitis comprise a series of disparate disorders. These include sclerosing panniculitis that results from chronic venous insufficiency of the lower extremities; panniculitis with calcification of the vessel walls such as calciphylaxis and oxalosis; and inflammatory diseases with crystals within the adipocytes such as sclerema neonatorum, subcutaneous fat necrosis of the newborn, and poststeroid panniculitis. Connective tissue diseases, such as systemic lupus erythematosus and dermatomyositis, pancreatic diseases, and alpha(1)-antitrypsin deficiency may also show a mostly lobular panniculitis with characteristic histopathologic features. Lobular panniculitis may also be an expression of infections, trauma, or factitial causes involving the subcutaneous fat. Lipoatrophy refers to a loss of subcutaneous fat due to a previous inflammatory process involving the subcutis, and it may be the late-stage lesion of several types of panniculitis. In contrast, lipodystrophy means an absence of subcutaneous fat with no evidence of inflammation and often the process is associated with endocrinologic, metabolic, or autoimmune diseases. Finally, cytophagic histiocytic panniculitis is the term that has been used to describe two different processes: one is inflammatory, a lobular panniculitis, and the other one is neoplastic, a subcutaneous T-cell lymphoma. The only common feature of these two different processes is the presence of cytophagocytosis in the lesions. (J Am Acad Dermatol 2001;45:325-61.) Learning objective: At the completion of this learning activity, participants should be familiar with the pathogenesis, clinical manifestations, histopathologic findings, and treatment options for the most frequent variants of the lobular panniculitides.     

Panniculitis–an unusual cutaneous manifestation of systemic sclerosis

Systemic sclerosis (SSc) is an autoimmune systemic disease characterized by small vessel involvement that leads to tissue ischemia and fibroblast stimulation resulting in accumulation of collagen (fibrosis) in the skin and internal organs. Lipomembranous panniculitis is a peculiar type of fat necrosis and has been reported with clinical conditions, commonly with peripheral vascular diseases. We describe a case of a 43‐year‐old woman with SSc manifestations, who presented with black scaly skin plaques, associated with thickening of the subcutaneous fat tissue, on the lateral surface of her thighs, her calves, gluteal area and lower abdomen. Biopsy revealed lipomembranous panniculitis. Lipomembranous changes have been seen in connective tissue disorders such as lupus profundus, morphea, systemic sclerosis and panniculitis associated with dermatomyositis, but rarely in thighs, calves, gluteal area and lower abdomen. Almeida MSTM, Lima SCB, Carvalho LL, Almeida JVM, Santos LG, Rolim JRA, Rocha TE. Panniculitis–An unusual cutaneous manifestation of systemic sclerosis.     

系统性红斑狼疮 皮肌炎和系统性硬皮病伴发脂膜炎26例临床分析

目的　探讨系统性红斑狼疮 (SLE)、皮肌炎 (DM)和系统性硬皮病 (PSS)伴发脂膜炎的临床、免疫、病理学特征、发病机理及治疗。方法　对 19例SLE、6例DM和 1例PSS伴发脂膜炎者的有关资料进行分析。结果　脂膜炎发生率为 2 .7% ,其中SLE2 .6% ,DM 5.3 % ,PSS1.1%。表现为单发或多发痛性皮下结节、斑块和溃疡 ,多见于四肢(57.7% )。组织病理学改变为脂肪小叶坏死、间质血管炎、栓塞形成及炎性细胞浸润。经皮质类固醇 +免疫抑制剂等治疗 ,可有明显改善。 3例分别死于败血症和成人呼吸窘迫综合征。结论　脂膜炎为SLE、DM和PSS少见的并发症 ;以发热、痛性皮下结节和溃疡为其特征 ;病理以脂肪小叶坏死为主 ,血管炎及栓塞可能是其发病的重要因素 ;皮质类固醇联合免疫抑制剂治疗有效     

Combination of massive mucinosis, dermatomyositis, pyoderma gangrenosum-like ulcer, bullae and fatal intestinal vasculopathy in a young female

Cutaneous mucinosis secondary to autoimmune collagen vascular disease is well recognized, but manifestation as cellulitis-like massive cutaneous mucinosis preceding dermatomyositis is unusual. Here we report a 21-year-old Taiwanese woman with a large, rapid onset, painful erythematous, edematous plaque, which histopathologically revealed septal panniculitis with fat necrosis and massive mucin deposition. Incapacitated muscle weakness of proximal extremities, generalized edema, heliotrope erythema, and Gottron's papules developed in a short period of time with high titers of serum muscle enzyme. Serological titers of ANA, anti-dsDNA, anti-ENA panels, and erythrocyte sedimentation rate, however, all showed unremarkable results. Diagnosis of dermatomyositis was confirmed by electromyographic findings of myopathy. As the disease progressed, large, deep cutaneous ulceration and vesiculobullous lesions also developed. In spite of aggressive treatment, the patient died 9 months after the disease onset, probably due to the complication of gastrointestinal ischemia and perforation.     

Cutaneous manifestations of internal malignancies in a tertiary health care hospital of a developing country

In a public hospital in Lima, Peru, 24 patients with 16 types of paraneoplastic dermatoses were identified by data collection. The most frequent dermatosis was dermatomyositis (four patients). The other dermatoses were malignant acanthosis nigricans, palmoplantar keratoderma, bullous dermatoses, lymphomatoid papulosis, edematous scarring vasculitic panniculitis, Norwegian scabies, primary systemic amyloidosis, necrolytic migratory erythema, infective dermatitis, pancreatic panniculitis, generalized pruritus, Lesser-Trelat syndrome, and acquired ichthyosis. Most of these paraneoplastic dermatoses were diagnosed before (45.8%) or at the time of (38.5%) the diagnosis of the underlying malignancy. The most frequent underlying malignancies were lymphoma, adenocarcinomas of the upper digestive tract, and malignant neoplasms of the pancreas. The average age of the patients was 47.0 ± 16.9 years and the length of the disease since diagnosis was 13.7 months. The mortality rate was 75%. Paraneoplastic dermatoses are rare dermatologic entities that are difficult to diagnose. Surveillance is also hampered when patients do not have easy access to health care centers due to financial and geographical issues. However, when identified, they might facilitate the early diagnosis of an associated tumor and contribute to increase the surveillance of patients.     

Subcutaneous sweet syndrome

Neutrophilic panniculitis encompasses a heterogeneous group of diseases histopathologically characterized by an inflammatory infiltrate in the subcutaneous fat mainly composed of mature neutrophils. This group of panniculitides includes alpha(1)-antitrypsin deficiency, infectious panniculitis, factitious panniculitis, subcutaneous Sweet syndrome, neutrophilic/pustular panniculitis associated with rheumatoid arthritis, erythema nodosum-like lesions of Beh?et disease, bowel bypass panniculitis, and iatrogenic panniculitis. This article reviews subcutaneous Sweet syndrome, which is a rare idiopathic panniculitis characterized by a dense neutrophilic infiltrate in the subcutis and is often related to hematologic malignancies. The relationship of subcutaneous Sweet syndrome and erythema nodosum is discussed as well as the differential diagnosis with other neutrophilic panniculitis.     

Lipomembranous changes and calcification associated with systemic lupus erythematosus

Lipomembranous changes are histological findings without specific clinical correlations, and are thought to be related to vascular impairment in fatty tissue. We describe a case of systemic lupus erythematosus (SLE) in which subcutaneous induration of the lower leg showed lipomembranous changes and calcification. This patient showed thrombophilia with thrombosis of the femoral and iliac veins, and cerebellar and myocardial infarction. A biopsy specimen from the subcutaneous tissue surrounding the multiple leg ulcers revealed degeneration of fat cells, lipomembranous changes in the fatty tissues, and calcification. Another biopsy specimen from the outer edges of the ulcers showed microthrombi and fibrinoid changes in the vessel walls, and mononuclear cell infiltrates in the dermis. Lipomembranous changes have been seen in connective tissue disorders such as lupus profundus, morphoea, systemic sclerosis, and panniculitis associated with dermatomyositis, but rarely associated with SLE. We speculate that ischaemic changes due to the vascular disturbance of the lower legs may have induced lipomembranous changes and calcification in the subcutaneous tissues in the present case.