[Paraneoplastic sclerodermiform syndrome].

Rev Mal Respir

Service de pneumologie et unité de soins intensifs respiratoires, université de Rouen, hôpital de Bois-Guillaume, CHU de Rouen, UPRES EA 3830 (IFR MP23), institut hospitalo-universitaire de recherche biomédicale, 76031 Rouen cedex, France.

Published: March 2010

Introduction: Despite the known association between scleroderma and cancer, the current systemic sclerosis classifications do not clearly identify paraneoplastic sclerodermiform syndrome or sclerodermiform syndrome secondary to cancer treatments.

Case Report: A 56 year old man was hospitalized for severe Raynaud's phenomenon with bilateral digital necrosis and otherwise good health status. X-rays did not show any subcutaneous calcification. Levels of serum antinuclear antibodies were high but anticentromere and anti-topoisomerase 1 antibodies were negative. Chest X-ray and CT-scan identified an irregular opacity in the right upper lobe with enlarged mediastinal lymph nodes. A diagnosis of bronchial adenocarcinoma was made following mediastinoscopy and the patient was treated with neo-adjuvant chemotherapy and lobectomy. After two cycles of treatment, his skin lesions had almost disappeared.

Conclusion: The presence of a sclerodermiform syndrome may suggest the existence of an underlying neoplasm. In this case report, the skin lesions disappeared quickly after antineoplastic treatment. We suggest that sclerodermiform syndrome be included in the systemic sclerosis classification.

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.rmr.2009.09.005DOI Listing

Publication Analysis

Top Keywords

sclerodermiform syndrome
16
systemic sclerosis
8
skin lesions
8
[paraneoplastic sclerodermiform
4
sclerodermiform syndrome]
4
syndrome] introduction
4
introduction despite
4
despite association
4
association scleroderma
4
scleroderma cancer
4

Similar Publications

Anti-synthetase and myelodysplastic syndromes with deep morphea: an example of shared immunopathogenesis? A case-based review.

Rheumatol Int

November 2024

Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

Article Synopsis
  • Anti-synthetase syndrome (AS) is a type of inflammatory muscle disease marked by specific autoantibodies and is often associated with myositis and lung issues; it sometimes includes skin lesions like those in dermatomyositis.
  • A case study of a 57-year-old man revealed a rare combination of AS, deep morphea, vasculitic neuropathy, and myelodysplastic syndrome, raising questions about shared underlying mechanisms among these conditions.
  • Despite receiving aggressive treatment, the patient experienced a severe decline and ultimately died from complications, underlining the complex interplay between AS and other inflammatory disorders.
View Article and Find Full Text PDF

Phospholipidosis is a rare disorder which consists of an excessive intracellular accumulation of phospholipids and the appearance of zebra bodies or lamellar bodies when looking at them using electron microscopy. This disease is associated with certain genetic diseases or is secondary to drugs or toxins. Drug-induced phospholipidosis encompasses many types of pharmaceuticals, most notably chloroquine, amiodarone or ciprofloxacin.

View Article and Find Full Text PDF

Eosinophilic Fasciitis Illustrated by 18 F-FDG PET/CT.

Clin Nucl Med

April 2024

Nuclear Medicine, ICO Pays de la Loire, Angers, France.

Article Synopsis
  • Eosinophilic fasciitis is a rare condition that causes swelling in the limbs and is similar to scleroderma.
  • A 71-year-old woman was hospitalized due to painful swelling in her legs, with lab tests showing elevated eosinophils and signs of inflammation.
  • Imaging studies, including a PET/CT scan and MRI, indicated heightened metabolic activity in the fasciae, leading to a biopsy that confirmed the diagnosis of eosinophilic fasciitis.
View Article and Find Full Text PDF

Background: Sclerodermiform lupus erythematosus (SDLE) is a rare Type 3 overlap syndrome of morphea and cutaneous lupus diagnosed with histopathologic features of both diseases present. It was first reported in 1976 by Umbert et al with a case series of four patients. SDLE is more common in young to middle-aged female patients.

View Article and Find Full Text PDF

Background: Linear atrophoderma of Moulin (LAM) is a dermatosis that affects children and adolescents characterized by hyperpigmented and atrophic linear lesions following Blaschko lines. So far, less than 50 cases have been published. Therefore, it is a rare entity with unknown etiology and a chronic and self-limited course.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!