Marked improvement of calcinosis in adult dermatomyositis with etidronate therapy

We report a 26-year-old woman with severe calcinosis associated with dermatomyositis. Although calcinosis of the skin or muscles is unusual in adults with dermatomyositis, this patient developed subcutaneous calcinosis with tenderness on the arms, axillary areas, shoulder areas, chest, abdomen, pelvis, and limbs. The calcinosis steadily increased and spread until joint motions were severely limited. Radiographic examination showed extensive soft-tissue calcification with a reticular pattern and severe osteoporosis. The patient was treated with oral etidronate (800 mg/day for 3 months every 6 months) to prevent calcification of the lesions. Three months later, the patient showed a dramatic improvement in symptoms with softening of the calcinosis, reduced pain, and marked increase in joint mobility. Radiographic examination showed marked decreases in the size of the calcified lesions compared to pre-treatment findings, and this effect persisted with a constant progressive efficacy for 3 years. The 3-year course of etidronate therapy also resulted in marked improvement of the severe osteoporosis and the patient was able to return to work and enjoy a normal life. We propose etidronate as a beneficial and effective therapy for calcinosis with osteoporosis.     

Successful cure of dermatomyositis after treatment of nonseminomatous testicular cancer

Although the association between dermatomyositis and cancer is well recognized, there have been a limited number of reports of dermatomyositis associated with testicular cancer. We report the case of a 31-year-old man who was diagnosed with dermatomyositis. Because the patient's serum levels of alpha-fetoprotein and human chorionic gonadotropin beta subunit were elevated, the patient was referred to our department. Physical examination revealed an induration (2 cm x 2 cm) in the left testis and the patient was diagnosed with stage IIIB left testicular cancer. A left high orchidectomy was then performed, and pathological examination revealed embryonal carcinoma. The patient was started on a systemic chemotherapeutic (etoposide, ifosfamide and cisplatin; VIP) regimen. After the third chemotherapy session, metastases had completely disappeared, the serum levels of alpha-fetoprotein and human chorionic gonadotropin beta subunit had normalized and the dermatomyositis symptoms had improved. Cancer is known to be a trigger of dermatomyositis. This case illustrates the importance of palpation of the testes in young patients with dermatomyositis.     

Successful resection of dermatomyositis associated with thymic carcinoma: Report of a case

We report a case of thymic carcinoma associated with dermatomyositis (DM) in a 53-year-old man. The patient presented with the characteristic features of a skin rash with Gottron’s papules, proximal muscle weakness, and increased serum levels of the muscle-associated enzymes. Comprehensive clinical examinations revealed an anterior mediastinal tumor. We resected the tumor and histological examination confirmed squamous cell carcinoma of the thymus. Thereafter, his clinical symptoms improved dramatically and his serum levels of muscleassociated enzymes dropped, indicating that the DM was a paraneoplastic phenomenon. Our search of the literature found only one other case report of DM accompanied by thymic carcinoma, and to our knowledge, this is the fi rst documented case of dramatic improvement of DM after resection of thymic carcinoma. We propose that thymic carcinoma should be added to the list of malignancies that can complicate DM as a paraneoplastic disease. The first two authors contributed equally to this study.     

Flare-up of dermatomyositis along with recurrence of breast cancer

Dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous manifestations. The association between dermatomyositis and malignancy is becoming more clearly delineated. A sort of dermatomyositis is thought to be paraneoplastic syndrome and dermatomyositis may follow clinical course of malignancy. We report a 68-year-old woman with dermatomyositis, whose clinical onset of dermatomyositis was apparently concomitant with breast cancer. Dermatomyositis had settled down and oral steroid could be tapered after the resection of breast cancer. Creatine kinase value was elevated before the detection of the first and second recurrence and in the terminal state. At the second recurrence, skin lesions and creatine kinase value had flared up and immediate metastatic check-up revealed the recurrence. Our case shows dermatomyositis, which was thought to be paraneoplastic syndrome, that followed clinical course of malignancy and suggests immediate check-up is needed for early detection of recurrence when dermatomyositis flares up after the resection of the malignancy.     

Renal angiomyolipoma associated with tuberous sclerosis. Review of the literature

A case of bilateral renal angiomyolipoma associated with tuberous sclerosis is presented. Although in our patient right renal biopsy specimen demonstrated angiomyoma, we believe that the lipomatous element could be found in a larger specimen. Roentgenographic and therapeutic considerations are presented and the different lesions of tuberous sclerosis reviewed. Unlike most patients exhibiting stigmata of tuberous sclerosis, our patient had normal mentality and seizures preceded skin lesions. Fundoscopic examination revealed retinal arteriolitis with an occlusive phenomenon which, to our knowledge, has not been reported previously.     

Synchronous Rectal and Esophageal Cancer Associated with Prurigo Chronica Multiformis: Report of a Case

The skin lesions that appear in association with internal malignancies are called dermadromes or paraneoplastic cutaneous disorders. These skin changes characteristically develop with progression of the internal malignancy. A 75-year-old Japanese man who had been diagnosed as having prurigo chronica multiformis, a form of dermadrome, 5 years previously was referred to our hospital for further investigation. On admission, numerous itchy red papules were present on the right side of the abdomen and the inner aspect of both thighs. Intensive screening for internal malignancies revealed advanced rectal cancer and early esophageal cancer. After surgical resection, the skin lesions improved without any treatment. To the best of our knowledge, this is the first case of synchronous double cancers associated with prurigo chronica multiformis. Received: October 31, 2000 / Accepted: July 17, 2001     

Dermatomyositis sine myositis and antisynthetase syndrome

We describe a 66-year-old woman with cutaneous lesions typical of classic dermatomyositis, clinical evidence of antisynthetase syndrome (arthritis, Raynaud's phenomenon, mechanic's hands and interstitial lung disease with anti-Jo-1 autoantibody) and lack of muscle disease after a full muscle evaluation that included clinical, enzymatic, electromyographic, magnetic resonance imaging and histological studies. The patient did not develop myositis after 9 years of clinical disease. The association of dermatomyositis sine myositis with antisynthetase antibodies suggests that the characteristic skin lesions are closely linked with dermatomyositis on the basis of the similar clinical and serological features of both dermatomyositis sine myositis and classic dermatomyositis.     

Dermatomyosite sans atteinte musculaire dans le cadre d’un syndrome des antisynthétases

We describe a 66-year-old woman with cutaneous lesions typical of classic dermatomyositis, clinical evidence of antisynthetase syndrome (arthritis, Raynaud’s phenomenon, mechanic’s hands and interstitial lung disease with anti-Jo-1 autoantibody) and lack of muscle disease after a full muscle evaluation that included clinical, enzymatic, electromyographic, Magnetic Resonance Imaging and histological studies. The patient did not develop myositis after nine years of clinical disease. The association of dermatomyositis sine myositis with antisynthetase antibodies suggests that the characteristic skin lesions are closely linked with dermatomyositis on the basis of the similar clinical and serological features of both dermatomyositis sine myositis  and classic dermatomyositis.     

Unexpected IgA nephropathy during the treatment of a young woman with idiopathic dermatomyositis: case report and review of the literature

This article reports the unexpected discovery of IgA nephropathy in a 26-year-old Chinese woman 1.5 years after the onset of idiopathic dermatomyositis. The patient was taking immunosuppressive agents, prednisolone 25 mg and azathioprine 75 mg daily. Glomerulonephritis associated with idiopathic polymyositis/dermatomyositis is rare. A review of the medical literature indicates that the most common pattern seen in idiopathic polymyositis is mesangial proliferative glomerulonephritis. However, both membranous and mesangial proliferative glomerulonephritis are often seen in idiopathic dermatomyositis. It is still not clear, however, whether the humorally- mediated immune process in dermatomyositis and the cell-mediated immune process in polymyositis can explain the different patterns of occurrence of glomerular lesions in these two closely related disease entities.     

Multiple neoplastic lesions of the lower genital tract in a human papilloma virus-infected kidney-pancreas allograft recipient: a case report

States of immunodeficiency are associated with an increased rate of certain cancers. Immunosuppressed allograft recipients are at high risk of Human Papilloma Virus (HPV)-related de novo malignancies. A female pancreas plus kidney transplant patient developed multiple genital malignancies within 6 years. The genome of human papilloma virus type 16 was detected in malignant lesions obtained from surgical procedures. All detected lesions were removed at an early state of development.     

Identification of autoantibodies elicited in a patient with prostate cancer presenting as dermatomyositis

OBJECTIVES: Dermatomyositis is an uncommon autoimmune disease distinguished by proximal muscle weakness and a characteristic skin rash. Dermatomyositis has also frequently been associated with malignancy, typically heralding the diagnosis of ovarian, lung, gastric, or colorectal cancer. We report an unusual case of prostate adenocarcinoma preceded by a diagnosis of dermatomyositis. We hypothesized that in this particular patient, proteins produced by the neoplastic prostatic tissue, which might be normally expressed in muscle tissue, were immunologically recognized as autoantigens. METHODS: Serum from this patient was used to screen a cDNA lambda phage expression library from normal prostate tissue for prostate protein-specific IgG. RESULTS: We identified several immunoreactive plaques encoding known autoantigens, and several encoding known muscle-related proteins, including aldolase C, eukaryotic translation elongation factor 1 alpha 1, transgelin, and acetyl-coenzyme A acyltransferase 1. IgG specific for these proteins were not specifically recognized in sera from other patients with prostate cancer compared with male control blood donors, and were not specifically recognized in a small panel of sera from patients with breast or ovarian cancer and dermatomyositis. CONCLUSIONS: Our results demonstrate that this patient with prostate cancer presenting as dermatomyositis had autoantibodies to specific proteins, possibly associated with his autoimmune myopathy. Moreover, given this patient's history and the multiple treatment options for prostate cancer, the identification of dermatomyositis in men should prompt an evaluation to exclude a concurrent diagnosis of prostate cancer.     

Neuromuscular blockade using vecuronium in dermatomyositis

The significant features of neuromuscular blockade with vecuronium in a patient with dermatomyositis are described: vecuronium 0.08 mg/kg resulted in 90%, 0.12 mg/kg in 100% neuromuscular blockade. In contrast to claims made in some previous publications, dermatomyositis did not produce increased sensitivity to vecuronium. Onset time and duration of action were also within normal limits in our patient. Time of spontaneous recovery until antagonism with neostigmine was markedly prolonged, but the dermatomyositis was only one of various possible explanations. Although there are potential hazards in the use of neostigmine in patients with dermatomyositis, antagonism of the neuromuscular block with 2 mg neostigmine was without problems in our patient. Our data support recent suggestions to reconsider the implications of dermatomyositis for anesthesia.     

Widespread central nervous system cavernous malformations associated with café-au-lait skin lesions. Case report

The simultaneous presence of cavernous malformations in the brain and spinal cord is a very rare finding and is typically associated with familial cavernous malformations. Although they are uncommon, various skin lesions can manifest in patients with familial cavernous malformations. The authors report on a 60-year-old man in whom more than 100 lesions consistent in appearance with cavernous malformations, including several intramedullary spinal cord lesions, were found throughout the neuraxis. This patient also displayed prominent café-au-lait skin lesions, but had no additional signs of neurofibromatosis or other neurocutaneous disorders. Analysis of his DNA revealed a novel mutation in the KRIT1/CCM1 gene, thereby confirming the diagnosis of familial cavernous malformation. The presence of these lesions in every major compartment of this patient's central nervous system underscores their indiscriminate nature and the need to screen throughout the neuraxis in patients in whom familial cavernous malformations are suspected. The findings in this case add to the growing list of skin lesions associated with genetically confirmed familial cavernous malformations. In patients presenting with seizures, focal neurological deficits, or hemorrhagic stroke, the presence of unusual skin lesions should prompt consideration of familial cavernous malformations, and appropriate screening should be performed.     

Skin cancer and (pre)malignancies of the female genital tract in renal transplant recipients

Immunosuppressive therapy in renal transplant recipients (RTRs) is associated with an increased risk for the development of (pre)malignancies involving the skin and the female lower genital tract. We assessed whether yearly cervical screening was performed and evaluated the development of skin cancer and gynaecological (pre)malignancies in RTRs. Female RTRs (n = 224), transplanted between 1991 and 1995, were analysed retrospectively. Sociodemographic patient characteristics, frequency and results of cervical smears and prevalence of cutaneous, cervical, vaginal or vulvar (pre)malignancies were investigated and compared with that in the general population. A mean of 0.2 cervical smears per patient per year was found to have been performed in RTRs, which is significantly less than the recommended screening ratio of 1.0 for female RTRs (P < 0.001). The risk for RTRs to develop malignancies of the female lower genital tract was increased: twofold to sixfold for cervical intraepithelial neoplasia, threefold for cervical carcinoma and 50‐fold for vulvar carcinoma. Cervical screening is not performed in accordance with the advised yearly intervals, and the risk for RTRs to develop vulvar and cervical (pre)malignancies is increased. More attention should be paid to the vulvar and cervical surveillance of RTRs by both medical specialists and general physicians.     

Anaplastic Thyroid Carcinoma Associated with Granulocyte Colony-Stimulating Factor: Report of a Case

A 75-year-old woman was hospitalized due to a right axillary mass. She had undergone a resection of thyroid carcinoma 13 years earlier, followed by two subsequent operations for recurrent thyroid disease. A physical examination revealed a right axillary mass associated with skin ulceration. Persistent bleeding was observed at the skin ulcer associated with the right axillary lymph node, despite conservative treatment for the lesion. Surgery was thus performed to control persistent bleeding from the axillary ulcer, and a histopathological examination resulted in a diagnosis of poorly differentiated thyroid carcinoma. The postoperative course was uneventful, but marked leukocytosis and extensive skin metastases were recognized 30 days postoperatively. A systemic examination revealed no other lesions associated with marked leukocytosis, but elevated levels of granulocyte colony-stimulating factor were noted in a blood examination. As a result, her general condition deteriorated rapidly and the patient died 2 weeks after the onset of leukocytosis.     

Dermatomyositis associated with membranous nephropathy in a 43-year-old female

A 43-year-old woman was admitted to our hospital for myalgic pain and erythema of the face and thighs. She had been diagnosed with dermatomyositis at another hospital 8 years earlier and proteinuria had developed suddenly. Renal histological examination revealed membranous nephropathy. Membranous nephropathy associated with dermatomyositis is very rare. To date, malignant disease has not been detected in our patient, but the serum level of CA125, a tumor marker, has been elevated to 1,097 U/ml. The increased CA125 level is related to the degree of proteinuria and after prednisolone and cyclosporine therapy, proteinuria and the CA125 level gradually decreased.     

Gastric carcinoma associated with dermatomyositis. Case report

A case of gastric carcinoma associated with dermatomyositis is reported. Total gastrectomy was performed and subsequently excision of hepatic metastasis. Although the cause-effect relationship between dermatomyositis and internal malignancy remains controversial, the obvious dependence of the clinical course of dermatomyositis on that of the gastric carcinoma in this case suggests a causal involvement.     

Amyopathic dermatomyositis

Amyopathic dermatomyositis (ADM) is a rare condition characterized by skin lesions suggestive of dermatomyositis (DM) without detectable muscle abnormalities after at least 2 years of follow-up. Pulmonary fibrosis is uncommon in patients with ADM. CASE REPORT: A 64-year-old woman presented with a 2 years and 6 months history of nondestructive polyarthritis. She had skin changes suggestive of DM, including a pink rash over the face, neck, and forearms; Gottron's papules over the metacarpophalangeal joints; and heliotrope edema of the eyelids. She reported no muscle symptoms. Findings were normal from muscle enzyme assays, electromyography, and muscle biopsies. A diagnosis of ADM was given. Early lung fibrosis was found. Investigations for a tumor were negative. DISCUSSION: ADM is a rare condition that may be an abortive form of DM with a favorable outcome and a lower risk of malignancy compared to classic DM. However, the development of pulmonary fibrosis may cloud the prognosis.     

Warfarin skin necrosis: local and systemic factors.

Warfarin induced skin necrosis occurs in 0.01-0.1% of warfarin treated patients. The usual presentation is that of painful lesions developing in obese women after the initiation of warfarin treatment. The lesions usually evolve into full thickness skin necrosis within a few days. Although the exact mechanism is not totally clear, low levels of Protein C or S, either functional or inherited, are associated with many of the cases. We report the case of a 17-year-old patient treated with warfarin because of iliofemoral deep vein thrombosis post abortion. The patient developed several huge haemorrhagic blisters on the affected leg. The condition rapidly developed into full thickness skin and fat necrosis. The necrotic lesions were excised and eventually covered with skin graft. The combination of the patient tendency towards hyper-coagulation and the local factors is discussed.     

5-year qualitative results of isolated cryosurgical ablation for hepatic malignancy at Walter Reed Army Medical Center(1)

Cryosurgical ablation (CSA) is an established treatment for primary and metastatic liver malignancies. The study objective was to qualitatively define our patient CSA experience and compare it with the existing literature.A retrospective review was conducted of patients who underwent isolated CSA from September 1995 to April 2000. Data were collected on patient characteristics, tumor characteristics, sequential 12-hour laboratory data, transfusion requirements, and survival data. SPSS 9.0 (SPSS, Chicago, Illinois) was used for data analysis.Twenty-four patients (14 men, 10 women) were studied. Eighty-seven lesions (mean 3.8/patient) were treated. Six patients underwent treatment for primary liver tumors, whereas 16 were treated for metastatic disease. White blood cell count increased 1.7-fold, and platelet count decreased 2.0-fold. Aspartate aminotransferase and alanine aminotransferase increased significantly 42- and 29-fold, respectively. Seven out of 21 (33%) patients required blood transfusion. Our overall complication rate was 25%. Perioperative mortality was 0%. Kaplan-Meier survival analysis revealed an overall survival of 46% at a median follow-up of 33.7 +/- 6.8 months.CONCLUSIONS:Although isolated CSA of hepatic malignancies results in major and minor alterations in serologic parameters, they equate to little clinical significance. Blood product transfusions are necessary in 30% patients post-CSA. Significant perioperative complications occur in 25% of patients. Survival estimates suggest that nearly 50% of patients undergoing CSA can be expected to survive longer than 2 years post-CSA.