A Rare Lymphoma in a Patient with Amyopathic Dermatomyositis

A 37-year-old Caucasian woman was evaluated for a photosensitive dermatitis. A positive anti-nuclear antibody with a titer of 1 : 1280 and a speckled pattern was noted and she was diagnosed with subacute cutaneous lupus erythematosus (SCLE). Although the initial dermatologic diagnosis was SCLE, a skin biopsy suggested the additional possibility of dermatomyositis because of increased dermal mucin. We began following her at the request of the dermatology department, and a diagnosis of amyopathic dermatomyositis was made based on the lack of objective muscle weakness, normal muscle enzymes, negative double-stranded DNA, SSA/SSB, and RNP/Smith antibody panel, and especially on the cutaneous examination findings. A malignancy evaluation included a normal CT scan of her chest, abdomen, and pelvis, esophagogastroduodenoscopy, colonoscopy, mammography, pelvic ultrasound, Papanicolaou smear, and endometrial biopsy. She developed vaginal bleeding 1 year after the onset of her skin manifestations. Repeat gynecologic evaluation, including cervical biopsy, revealed a large B-cell cervical lymphoma. Amyopathic dermatomyositis and lymphoma of the cervix are both rare conditions. Our case emphasizes the importance of considering underlying malignancy at the time of diagnosis and while the patient is followed clinically. Additionally, the clinician must remain vigilant in evaluating any new clinical changes in follow-up care. To our knowledge, this is the first documented case of lymphoma of the cervix in the setting of amyopathic dermatomyositis.     

Nodular Cystic Fat Necrosis with Calcification in a Patient with Juvenile Dermatomyositis

Nodular cystic fat necrosis is a rare, benign form of encapsulated fat necrosis with distinct histology, characterized by cystic fat necrosis with lipomembranous changes and, in later stages, calcification. We report the case of a 7‐year‐old child with juvenile dermatomyositis who presented with three asymptomatic, firm, mobile nodules on the arms and neck. Histology was consistent with nodular cystic fat necrosis with prominent calcification. This is an unusual presentation of this entity because it has never been previously reported in association with juvenile dermatomyositis.     

Dermatomyositis Associated with Primary Intramuscular B Cell Lymphoma

A rare case of dermatomyositis associated with primary intramuscular malignant lymphoma is described. A 40-year-old Japanese woman noticed swelling of the right thigh during the treatment of dermatomyositis with prednisolone, azathioprine and cyclophosphamide. A biopsy specimen taken from the right quadriceps muscle revealed infiltration of lymphoma cells which were positive for CD20. We reviewed 12 cases of dermatomyositis associated with malignant lymphoma reported in Japanese literature between 1984 and 1996.     

Dermatomyositis with Multiple Skin Ulcers Showing Vasculitis and Membrano-cystic Lesion

A 63-year-old woman with dermatomyositis (DM) developed multiple punched-out ulcers of the skin. She had not only a past history of gastric cancer 10 years prior to the onset of DM, but also a familial history of cancer. A skin biopsy from one of the ulcers revealed both vasculitis and membrano-cystic lesion in the same specimen. She died of interstitial pneumonitis one year after the onset of DM. Vasculitis and membrano-cystic lesion may be an important marker of a poor prognosis in DM.     

Primary Cutaneous B‐Cell Lymphoblastic Lymphoma Arising from a Long‐Standing Lesion in a Child and Review of the Literature

Precursor B‐cell lymphoblastic lymphoma (B‐LBL) is a rare entity and primary cutaneous B‐LBL is an even more uncommon diagnosis that typically affects children. A 4‐year‐old boy presented with a persistent rash on his left cheek for almost 2 years and was found to have primary cutaneous B‐LBL. We report this case to emphasize that B‐LBL should be in the differential diagnosis for an otherwise unimpressive persistent lesion in the head and neck region and review all reported pediatric cases of primary cutaneous B‐LBL without extracutaneous involvement.     

儿童多发性肌炎与皮肌炎17例临床分析

目的探讨儿童皮肌炎/多发性肌炎(JDM/PM)的临床特点、辅助检查与治疗。方法回顾性分析本院1994年1月~2004年12月诊治的17例JDM/JPM。结果主要有肌无力、肌痛或肌压痛、关节疼痛和发热等;存在肺、心脏、胃肠道、肾损害,未见伴恶性肿瘤和钙质沉着者;LDH、HBDH、AST、CPK、α2、γ球蛋白升高,HB降低,ESR增快,RF阳性;EMG示肌源性改变,肌活检异常。结论本病以肌肉症状最多见,肌酶升高显著,以心、肺损害多见,经激素治疗均有所缓解,预后较好。     

Multiple Agminated Juvenile Melanoma Arising on a Hyperpigmented Macule

A case of multiple agminated juvenile melanoma (MAJM) arising on a hyperpigmented macule is reported. The patient, a 1-year-old boy, had 9 small nodules on an irregular, hyperpigmented macule on the right shoulder. Histologically, the nodules contained nests of spindle-shaped cells in their epidermis and at the dermoepidermal junction, and the hyperpigmented macule exhibited increased melanin granules in the basal layer and small nests of epithelioid cells. We concluded that MAJM on a hyperpigmented macule is the subtype of the nevus spilus.     

Partial Lipodystrophy Associated with Juvenile Dermatomyositis: Report of Two Cases

Abstract: A 27-year-old woman and a 13-year-old girl diagnosed with juvenile dermatomyositis in childhood developed clinical findings of partial lipodystrophy 10 years after diagnosis. Exhaustive clinical and laboratory examinations showed an association with other abnormalities: hy-pertrichosis, steatohepatitis, and an abnormal insulin response to the glucose loading test in the first patient. Hypertrichosis, steatohepatitis, insulin-resistant diabetes mellitus, and acanthosis nigricans were observed in the second patient. Renal function was normal in both patients. Although a localized form of lipodystrophy has been reported associated with connective tissue disease (connective tissue lipoatrophy), the partial form has been infrequently described in association with juvenile dermatomyositis.     

An Aggressive Spindle Cell Squamous Carcinoma Arising in a Patient with Long-standing Erythroderma

Spindle cell squamous carcinoma (SCSC) of the left hand arising in a patient with longstanding erythroderma is reported. Histopathologically, spindle shaped atypical cells were observed neighboring the cells of well differentiated squamous cell carcinoma. These two types of tumor cells, spindle cells and well differentiated cells, were present side by side and merged into each other. The erythroderma had been present for over 20 years, and both clinical and histopathological findings suggested cutaneous T cell lymphoma, but were not diagnostic for mycosis fungoides, Sézary syndrome, or adult T cell lymphoma. Flow cytometry of peripheral blood cells showed a low CD4/CD8 ratio which suggested impaired T cell function. Multiple metastases of SCSC occurred in a short period and the patient died ten months after his first visit to us. The aggressive course of this case was unusual, and may be due to immunological abnormalities associated with the long standing erythroderma with impaired T cell function.     

趋化因子SDF-1及其受体CXCR4在皮肌炎皮损中的表达

目的研究趋化因子SDF-1及其受体CXCR4在皮肌炎皮损中的表达及其意义。方法采用免疫组化ABC法检测及图像分析观察22例皮肌炎皮损和15例正常对照皮肤石蜡切片中SDF-1和CXCR4的表达。结果SDF-1和CXCR4在皮肌炎皮损中的表皮基底层、棘层、颗粒层及腺体、血管平滑肌中均有较强表达,而在正常皮肤中表达下降,两者有显著性差异(均P<0.05)。结论SDF-1和CXCR4可能与皮肌炎的发生和发展相关,SDF-1和CXCR4为皮肌炎的免疫治疗提供了新的靶分子。     

Calcinosis Cutis in Juvenile Dermatomyositis Responsive to Aluminum Hydroxide Treatment

Calcinosis cutis is a frequent complication of juvenile dermatomyositis; however, its treatment remains unsatisfactory. We report a case of calcinosis cutis in juvenile dermatomyositis which was successfully treated with oral aluminum hydroxide. Almost complete clearing of calcinosis was observed after 8 months of therapy. There were no adverse effects from therapy. Aluminum hydroxide is worth trying in treating calcinosis cutis.     

Leukemic Phase of Follicular Lymphoma after Treatment with Etanercept in a Patient with Psoriasis

Etanercept, a fully human soluble recombinant p75 tumor necrosis factor (TNF) receptor that blocks the binding of TNF to cell surface receptors, is approved for the treatment of psoriatic arthritis and other rheumatic conditions in the US and Europe. Ever since the introduction of anti-TNF treatment in patients with inflammatory autoimmune diseases, there have been concerns about a possible tumor-promoting effect of such a measure. We report a rare case of leukemic phase of follicular lymphoma in a 62-year-old man with moderate-to-severe plaque psoriasis receiving long-term treatment with etanercept for 3 years. Although the association is intriguing, no causal relationship is suggested.     

Dermatomyositis,Carcinoma of Colon and Meningioma in the Same Patient

Dermatomyositis and carcinoma of colon were diagnosed in a 66-year-old woman. Meticulous physical examination excluded further systemic or cutaneous involvement. The musculocutaneous disorders responded well to daily oral corticosteroid, and the malignant tumor was totally removed surgically. After a seven-year follow-up of actual dermatomyositis controlled by maintenance doses of prednisone ranging from 5 to 15 mg daily, the patient developed a meningioma. Current concepts and data regarding various aspects of the combination between dermatomyositis and tumors are discussed. To our knowledge, this is the first reported case of meningioma associated with dermatomyositis.     

Analysis of Lymphocyte Subpopulations in Peripheral Blood in Adult and Juvenile Cases of Dermatomyositis

Dermatomyositis, recognized as an autoimmune disorder, occurs not only in adults but also in children. In this study, we evaluated lymphocyte subpopulations in the peripheral blood of 24 adult dermatomyositis and 14 juvenile dermatomyositis patients and in 17 healthy adults and 9 healthy children by flow cytometry using monoclonal antibodies. When compared with healthy adults and adult patients with inactive dermatomyositis, the adult patients with active disease had significantly lower percentages of CD3⁺ and CD8⁺ cells and a significantly higher percentage of CD20⁺ cells. In contrast, juvenile dermatomyositis patients had lymphocyte subpopulations not significantly different from those of healthy children; the activity/inactivity of disease did not make any difference. These results support our hypothesis that adult and juvenile dermatomyositis may be diseases of entirely different scope.