Paraneoplastic Dermatomyositis Research Articles

Letter to the Editor

Letter to the Editor

Paraneoplastic motor neuropathy and inflammatory myopathy associated with nasopharyngeal carcinoma

Nasopharyngeal carcinoma (NPC) is common in Southern Chinese and relatively young patients are affected [1]. Despite being potentially treatable by irradiation, local recurrence and metastases are lifethreatening. Dermatomyositis (DM), urticarial vasculitis, pulmonary osteoarthropathy, clubbing of fingers and myelemia are known paraneoplastic conditions associated with NPC [1]. Paraneoplastic DM occur before, simultaneously or follow the diagnosis of NPC in 0.9–3.9% patients. Up to 25% of malignancy detected in paraneoplastic DM was NPC. Diagnosis of DM trigger aggressive search for underlying malignancy including NPC. We reported a patient who developed paraneoplastic subacute predominantly motor polyneuropathy/polyradiculoneuropathy and inflammatory myopathy without skin rash simultaneously upon recurrence of NPC. Case report

Ulcerative Paraneoplastic Dermatomyositis Secondary to Metastatic Breast Cancer

A 40-year-old Chinese-American woman with breast carcinoma metastatic to her lungs presented with a 3-month history of erosions on her inner thighs (Figure 1) and medial left shoulder. Faint livedo reticularis was evident on her legs as well. She had difficulty in walking and raising her shoulders. Her cutaneous examination was also notable for cuticular erythema (Figure 2) and mild periorbital erythema and edema. She had no systemic or rheumatologic complaints other than some difficulty in swallowing. Her blood chemistry values were notable for a creatinine kinase of 564 IU/L (5-200 IU/L), alanine aminotransferase 161 U/L (0-40 U/L) and aspartate aminotransferase 93 U/L (0-40 U/L), and an antinuclear antibody titer of 1:2560. Other blood chemistries and antibody serologies (anti-Jo-1, anti-Mi-2 and other anti-tRNA synthetase, anti-Ro/SSA, anti-U1RNP, anti-PM/Scl, and anti-Ku) were within normal limits. A biopsy specimen was obtained from an area of intact skin close to a right thigh ulceration that showed subtle vacuolar alteration at the dermo-epidermal junction with occasional necrotic keratinocyte (Figure 3). Melanophages and telangiectases were present. Within the subcutis there was fibrin deposition and neutrophils. A diagnosis of dermatomyositis was made. The patient received oral prednisone 20 mg three times a day, and her ulcerations resolved. Her creatinine kinase, alanine aminotransferase, and aspartate aminotransferase values returned to normal over the course of 3 weeks, but her antinuclear antibody was unchanged. Radiographic studies concurrently noted that her breast cancer had recurred in her lungs; plans were made to treat her with chemotherapy. The patient was lost to close follow-up, but it was learned that her erosions had reoccurred while her prednisone was tapered and resolved when her dosage of prednisone was increased.

A Small Cell Lung Cancer Concurrently Diagnosed with Paraneoplastic Dermatomyositis

Dermatomyositis is an inflammatory myopathy, of unknown etiology, which is characterized by cutaneous rashes, accompanied by progressive and symmetric proximal muscle weakness. Especially in older people, the incidences of malignant conditions appear to be increased in dermatomyositis patients, and the prognosis is very poor. Because dermatomyositis may occur as a paraneoplastic syndrome, extensive screening tests, for an occult malignant neoplasm, should be conducted in older dermatomyositis patients. We experienced a case of small cell lung cancer, which had a very rapid and catastrophic clinical course, in a 63-year-old male patient with dermatomyositis. We report this case, and review the literature on the relationship of dermatomyositis and malignancy.

Dermatomyositis in association with transitional cell carcinoma of the bladder

We report a case of dermatomyositis occurring in association with transitional cell carcinoma of the bladder. The case illustrates the importance of a thorough search for neoplasms in elderly patients with dermatomyositis and is a reminder that bladder cancer may be a rare cause of dermatomyositis. The case also shows that successful treatment of an underlying tumour may lead to resolution of paraneoplastic dermatomyositis, and relapse of cutaneous and muscle symptoms and signs may indicate recurrence of tumour.

Dermatomyositis and drugs.

Dermatomyositis (DM) is an idiopathic inflammatory disorder consisting of skin and skeletal muscle involvement. Patients with skeletal muscle involvement have polymyositis (PM), and those unresponsive to therapy and with characteristic findings on muscle biopsy have inclusion body myositis. Patients without muscle damage and typical skin lesions have amyopathic dermatomyositis. Disease in children (juvenile dermatomyositis) is not associated with malignancy as it may be in adults (paraneoplastic dermatomyositis). Overlap syndrome (OS) is mixed connective tissue disease combining some features of DM, SS and LES. Scleromyositis is overlap syndrome associated with anti-PM-Sci antibodies. Patients with PM, DM or OS with "interstitial lung disease" and anti-synthetase antibodies have an "anti-synthetase syndrome". Various drugs, including d-penicillamine, NSAIDs, anti-infectious agents, as well as lipid lowering drugs, the HMG-CoA reductase inhibitors may cause myopathy and skin lesions (drug induced dermatomyositis). "Dermatomyositis" occurring as adverse reactions of drugs are rare, irregular and impossible to predict in individual patients. They are very interesting in that they may be keys for explaining the pathogenic mechanisms of the disease.

Paraneoplastic dermatomyositis presenting as erythroderma

Erythroderma is idiopathic in one third of patients. Its most common causes include a variety of pre-existing skin diseases such as atopic dermatitis and psoriasis, drug reactions, malignancies, and congenital dermatoses.1 To our knowledge there are only 4 well-documented cases in the literature of dermatomyositis presenting with erythroderma.2-5 Because none of these cases was associated with malignancy, this is the first report of paraneoplastic dermatomyositis presenting with erythroderma.

Microvascular changes in skeletal muscle in idiopathic inflammatory myopathy

Open deltoid muscle biopsy specimens from patients with idiopathic adult dermatomyositis, paraneoplastic dermatomyositis, childhood dermatomyositis, and idiopathic polymyositis, and from control patients were studied. Qualitative and morphometric capillary analysis by phase and electron microscopy was carried out. In the morphologic analysis the most striking difference was the presence of capillary damage and a higher capillary depletion in dermatomyositis as well as a higher capillary density in polymyositis. By electron microscopy, capillaries from patients with dermatomyositis showed mainly microtubuloreticular structures, loss of endothelial plasma membranes, and the appearance of abnormal cytoplasmic organelles. In contrast, capillaries from patients with polymyositis exhibited only minimal changes. By morphometric analysis, muscle capillaries in dermatomyositis had a significantly higher mean endothelial thickness than those in polymyositis. Finally, a significant topographic association between capillary damage and muscle fiber changes was observed only in patients with dermatomyositis. On the other hand, paraneoplastic dermatomyositis showed fewer structural and morphometric capillary changes than the other forms of dermatomyositis. We conclude that dermatomyositis is characterized by microvascular alterations that are absent in polymyositis. The topographic proximity of capillary changes to muscle fiber injury suggests that capillary damage may play a role in the pathogenesis of the muscle lesions observed in patients with dermatomyositis.

Pulmonary nocardiosis concurrent with Pneumocystis carinii pneumonia in two patients undergoing immunosuppressive therapy

Two cases with concomitant pulmonary nocardiosis and Pneumocystis carinii pneumonia are described. The first patient developed pneumonia 3 months after heart transplantation while undergoing standard immunosuppressive therapy with cyclosporin, azathioprine and prednisone. The second patient was treated with chemotherapy and subsequent radiotherapy of the mediastinum for a malignant epithelial tumour. He also received prednisone for paraneoplastic dermatomyositis. Chest X-rays of both patients showed a bilateral interstitial pattern and broncho-alveolar lavage revealed P. carinii. Additional dense and localised pulmonary infiltrates led to suspicion of a further infectious agent, namely, Nocardia asteroides, which was isolated from both patients. Since nocardiosis calls for prolonged treatment, extensive diagnostic measures are needed for its early detection.

Paraneoplastic Dermatomyositis in an Adolescent

An 18-year-old adolescent with previous nasopharynx cancer developed dermatomyositis. The evolution was fatal because of metastases. In spite of the young age, we believe this to be a case of paraneoplastic dermatomyositis.