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Extrapulmonary thoracic restriction (hidebound chest) complicating eosinophilic fasciitis
R B Chalker1, B F Dickey, N C Rosenthal
1Pulmonary Center, Boston University School of Medicine.
Chest
|November 1, 1991
Summary
Eosinophilic fasciitis (EF) rarely causes severe breathing problems. This case highlights a unique instance of "hidebound chest" restricting lung function in a patient with EF.
Area of Science:
- Rheumatology
- Pulmonology
- Dermatology
Background:
- Eosinophilic fasciitis (EF) is a rare connective tissue disorder.
- It typically involves inflammation of the deep fascia, leading to skin thickening and induration.
- Visceral involvement is uncommon in EF.
Observation:
- A patient with biopsy-proven EF presented with progressive respiratory limitation.
- Physical examination showed significant thoracic integument induration and limited chest wall excursion.
- Pulmonary function tests revealed reduced total lung capacity (62% of predicted).
Findings:
- Despite normal maximal inspiratory force and diffusion capacity (Dco), chest CT showed no parenchymal lung disease.
- The patient exhibited severe extrapulmonary thoracic restriction, termed "hidebound chest".
- This complication has not been previously reported in Eosinophilic fasciitis.
Implications:
- This case expands the known clinical spectrum of Eosinophilic fasciitis.
- It underscores the potential for severe thoracic restriction in EF, impacting respiratory function.
- Recognizing "hidebound chest" is crucial for managing respiratory complications in EF patients.