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.     

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。     

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.     

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–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.     

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.     

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 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.     

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.     

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.     

Alpha‐1‐antitrypsin deficiency‐related panniculitis: two cases with diverse clinical courses

Alpha‐1‐antitrypsin deficiency (AATD)‐related panniculitis is an extremely rare and underdiagnosed entity, and there is a paucity of data on its treatment. We report two cases of AATD‐related panniculitis. The first was a 24‐year‐old woman with known AATD who presented with painful leg ulcers refractory to treatment with corticosteroids and colchicine. She had a good response to α1‐antitrypsin infusions but required dose adjustment due to flares in disease activity. The second case was a 38‐year‐old woman who presented with painful nodules on the legs refractory to corticosteroid therapy. Laboratory investigations revealed severe AATD. She had an excellent response to colchicine therapy. In both these cases of AATD, panniculitis was the first clinical manifestation of the disease. AATD‐related panniculitis may have none of the typical clinical clues for AATD, such as a family history, cirrhosis or emphysema. Early identification may help prevent these complications from developing.     

Vemurafenib‐Induced Neutrophilic Panniculitis in a Child with a Brainstem Glioma

Neutrophilic panniculitis is a rare adverse effect of therapy with selective BRAF inhibitors. We report a case of neutrophilic panniculitis in a 15‐year‐old girl receiving treatment with vemurafenib for a brainstem glioma. Clinicians should be aware of this rare but important side effect of vemurafenib. This is the first report of neutrophilic panniculitis in a child treated with vemurafenib.     

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.     

Gouty panniculitis in a 68‐year‐old man: case report and review of the literature

Background Gouty panniculitis is a very rare disease. To date, only eight patients have been reported. In each case, time to diagnosis was lengthy. We describe a 68‐year‐old man with a 6‐month history of tender, erythematous plaques and nodules involving the anterior aspects of the legs Methods and results Histologic examination led to the diagnosis of gouty panniculitis. Conclusion Review of the literature and the present case suggests that hyperuricemia is a necessary but probably not sufficient condition for gouty panniculitis. Why gouty panniculitis very seldom arises as a manifestation of gout is puzzling. In almost all reported patients, uricostatic or uricosuric therapy leads to clinical improvement of the disease.     

Group A Streptococcal Panniculitis

Infectious panniculitis caused by group A beta‐hemolytic streptococcus is rare, especially in immunocompetent patients. Its clinical presentation is usually nonspecific, but skin biopsy may provide information on the source. We describe the case of a previously healthy 2‐year‐old girl who presented with fever, tachycardia, and diffuse erythematous skin nodule; biopsy revealed a deep lobular neutrophilic panniculitis with gram‐positive bacteria.     

Annular Lipoatrophy of the Ankles: Case Report and Review of the Literature

Abstract: Annular lipoatrophy of the ankles also termed “annular atrophic connective tissue panniculitis of the ankles,”“annular atrophy of the ankles,” and “atrophic annular panniculitis of the ankles,” is an inflammatory panniculitis that results in lipoatrophy with a predilection for the ankles. The disorder is seen predominantly in female children and demonstrates a mixed lobular panniculitis. It is likely a disorder of autoimmune etiology, supported by the findings of concurrent autoimmune disorders in several patients. We report a case of a 6‐year‐old female with annular lipoatrophy of the ankles associated with Hashimoto thyroiditis and review the epidemiology and proposed pathogenesis of annular lipoatrophy of the ankles.     

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.     

Disseminated ulcerating lupus panniculitis emerging under interferon therapy of hairy cell leukemia: Treatment‐ or disease‐related?

We report a 43‐year‐old woman, who underwent therapy with interferon‐α for hairy cell leukemia. During interferon‐α therapy she developed multiple subcutaneous swellings, accompanied by fever and fatigue. A skin biopsy revealed lobular, T‐cell lymphocytic panniculitis. In conjunction with the clinical and immunological findings, the diagnosis of lupus panniculitis was made and interferon‐α therapy stopped. Initially, she responded well to oral prednisone and hydroxychloroquine, but after several months she became resistant to it. Her condition worsened, she developed skin ulcers in the inflamed regions. Only with the leukemia‐targeted therapy using cladribine and rituximab her skin condition could be controlled, suggesting hairy cell leukemia as an additional trigger of the lupus panniculitis. Our report is the first one to show induction of lupus panniculitis under interferon therapy of hairy cell leukemia and its presumable sustentation by the latter.