Bullous lung disease suny downstate medical center. In patients with severe bullous lung disease ct figure 2 will differentiate emphysematous bullae from pneumothorax and save the patient an unnecessary and potentially dangerous procedure. By direct immunofluorescence, linear staining of iga at the dermalepidermal junction. Severe bullous lung disease due to marginalzonelymphomaassociated amyloidosis. A 12 year old boy suffering from p67 phox deficient chronic granulomatous disease presented with a bullous skin disease and a lung infection with paecilomyces species. Loss of alveolar surface area due to bullous disease contributes to functional and physiological impairment of lung function in sarcoidosis. Marked compression of adjacent lung tissue demonstrated by ct scan fev1 emphysema treatment trial for elective lvrs operative mortality up to 6% pulmonary morbidity 30% high risk for death with little functional benefit fev1 dacvs. These pockets ultimately form cysts bullae that are airfilled and surrounded by normal lung tissue.
Martinez definition a bulla is an aircontaining space within the lung parenchyma that arises from destruction, dilatation, and confluence of airspaces distal to terminal bronchioles and is larger than 1 cm in diameter fig. Serum angiotensinconverting enzyme activity was normal. Bright, dvm, ms, dacvs bullous lung disease basic information description bullous lung disease is the formation of contained or confined pockets of air and fluid in one or more portions of the lungs. Demeester, md departments of surgery and pulmonary medicine, creighton university school of medicine, omaha, nebraska one of the most difficult problems facing clinicians is the evaluation and management of patients with dyspnea whose chest roentgenogram shows single or multiple large. Patients who have bullous disease in the presence of diffuse lung disease emphysematous or nonemphysematous should be evaluated on an individual basis and surgery should be performed on patients. Bullae are thinwalled, airfilled cystic spaces that exceed 1 cm in diameter and are found within the lungs. The histopathology of a bullous lesion showed subepidermal blister formation and microabcesses containing eosinophils in the dermal papillae. Giant bullous emphysema gbe was first described in 1937 by burke in a young male cigarette smoker with a large bullae in the upper lobe associated with paraseptal emphysema in 1937. Treatment considerations include therapy for underlying disease, ventilation strategies to prevent progression of the bullous lung disease, and indications for surgical or bronchoscopic intervention. The majority of patients with labd present with pruritic vesicles and papules, localized primarily to the extensor. Aug 10, 2016 bullous pemphigoid is a skin disorder characterized by large blisters.
In subjects who smoke marijuana, these pathological changes occur at a younger age approximately 20 years earlier than in tobacco smokers. Nov 01, 2008 severe bullous lung disease due to marginalzonelymphomaassociated amyloidosis christopher s king, aaron b holley, john h sherner respiratory care nov 2008, 53 11 14951498. The lung loses its capability to expand and contract completely. Bullous lung disease is the formation of contained or confined pockets of air and fluid in one or more portions of the lungs. However, the cxr was normal in four patients and lung function was normal in five.
This is condition which is characterized by abnormal and enlarged air spaces within the lungs. Bp is the most common autoimmune bullous disease and is the prototypic subepidermal blistering disorder. Interstitial lung disease is an umbrella term that covers more than 100 different types of pulmonary disorders that deal with the absorption of oxygen into the lungs. Coronal chest computed tomography demonstrating extensive bullous disease of the lungs with a prominent bulla in the right upper lobe containing an air fluid level. Chronic bullous disease of childhood and a paecilomyces lung. In very severe bullous emphysema, visceral pleura may be so thin that no lung sliding is detectable. As the symptom expression of these subsidiary respiratory diseases can range widely, what unites these ranges of conditions is their symptoms. In some people, the mouth or genitals are also affected. Interstitial pneumonia associated with bullous pemphigoid. A chest ct scan revealed bullous lung disease, increased interstitial markings, and bilateral mediastinal adenopathy. Radiographic findings typical of vaping related lung disease include ggos, heterogeneous consolidation, crazy paving, micronodules alone or in combination. The blisters are usually located on the arms, legs, or middle of the body. Bullous lung disease the western journal of emergency.
Atelectasis due to compression of surrounding lung by giant bullae is also seen in the posterior subpleural regions of the lungs bilaterally c. Highresolution computed tomography of the chest with intravenous contrast demonstrates extensive bilateral giant bullae, which are most pronounced in the bilateral upper lobes a and b. Ritesh agarwal and ashutosh n aggarwal introduction bullous lung disease is an entity characterized by the presence of bullae in one or both the lung fields, with normal intervening lung. Bulla is a blister more than 5 mm about 316 inch in diameter with thin walls that is full of fluid or air. Most patients with bullae have a significant cigarette smoking. Linear iga bullous disease labd is a clinically heterogeneous blistering disease in which direct immunofluorescence of perilesional skin biopsies reveals the presence of a linear band of iga at the basement membrane zone. A coronal view d demonstrates extensive bullous disease. Bullous lung disease mimicking pneumothorax in focused.
Lung disorders associated with bullous pemphigoid are extremely rare, and, to our knowledge, this is the first report of an immunologically confirmed case of interstitial pneumonia. In patients with severe emphysema, discrete emphysematous bullae have been shown to functionally impair pulmonary mechanics and result in diminished exercise capacity and even acute respiratory distress. Demeester, md departments of surgery and pulmonary medicine, creighton university school of medicine, omaha, nebraska one of the most difficult problems facing clinicians is the evaluation and management of patients with dyspnea. Teaching case of the month joshua o benditt md, section editor bullous lung disease or bullous emphysema. Each had a history of significant exposure to marijuana. Bullous disease of the lung fishmans pulmonary diseases. In patients with severe emphysema, discrete emphysematous bullae have been shown to functionally impair pulmonary mechanics and result in diminished exercise capacity and even acute respiratory distress 14.
Bullous lung disease is a lung disorder, which causes a patient to suffer from shortage of breath. The patient had progressive pulmonary symptoms over 14 years. Marijuana smoking leads to asymmetrical bullous disease, often in the setting of normal cxr and lung function. Bullous lung disease, or bullous emphysema, is a longterm progressive disease of the lungs characterized by single or multiple bullae. In the first case a diagnosis of cutis laxathat is, ehlersdanlos syndromewas made in view of elastic skin, past history of being operated for bilateral inguinal hernia, aphakic eyes, and presence of mitral valve prolapse. Nonsurgical management of giant lung bullae during. Apr 18, 2019 in very severe bullous emphysema, visceral pleura may be so thin that no lung sliding is detectable. Bullous lung disease the western journal of emergency medicine. In three of the four cases the tobacco smoking load had been relatively small, suggesting a possible causal role for marijuana in the pathogenesis of this unusual pattern of bullous emphysema. The differential diagnoses of cystic bullous disease of. Christopher s king, aaron b holley and john h sherner.
Bullous pemphigoid is a skin disorder characterized by large blisters. The case histories are presented of four men with multiple large upper zone lung bullae but otherwise relatively preserved lung parenchyma. This emphysema also includes deterioration of alveolar tissue and this is basically lung tissue that makes up the walls between air sacs. Bullous pemphigoid is an acute or chronic autoimmune skin disease causing the formation of blisters bullae in the epidermal and dermal junction. Severe bullous lung disease due to marginalzonelymphoma. Chronic bullous disease of childhood and a paecilomyces. Bullous lung disease is the most common form of emphysema. These pockets ultimately form cysts bullae that are airfilled and sur rounded by normal lung tissue. Effect of cannabis smoking on lung function and respiratory. Although there are some treatment recommendations for copdrelated lung bullae, 3, 4 the role and timing of bullectomy for newonset lung bullae. Idiopathic giant bullous emphysema, also known as vanishing lung syndrome vls, is characterized by giant emphysematous bullae, which commonly develop in the upper lobes and occupy at least onethird of a hemithorax. A chest radiograph showed hyperinflation with increased interstitial markings in the apical and mid lung zones.
It results due to the formation of bullae in the lungs of an individual. Aside from the abovementioned study, 7 case series and 10 case reports were also found, including a total of 56 marijuana smokers presenting with bullous lung disease often with pneumothorax. Oct 25, 20 1 greenberg ja, singhal s, kaiser lr giant bullous lung disease. Pdf on apr 1, 20, gilbert berdine and others published bullous lung disease find, read and cite all the research you need on researchgate. Although considered to be rare, it is not inconceivable that bullous sarcoidosis may in fact be underrecognised for a variety of reasons. Outcome of the surgical treatment of bullous lung disease. In the second case the diagnosis of marfans syndrome was made in view of cardiac and skeletal abnormalities. Bullae on the pleura, the membrane that covers the lung, are also called blebs.
Copd patients with dyspnoea on exertion, forced expiratory volume in 1 s fev 1 bullous lung disease, comorbid conditions that may worsen hypoxaemia like cardiac disease and significant symptoms during previous air travel are considered as high risk and. American journal of respiratory and critical care medicine. Oct 20, 2016 aside from the abovementioned study, 7 case series and 10 case reports were also found, including a total of 56 marijuana smokers presenting with bullous lung disease often with pneumothorax. The first page of the pdf of this article appears above. Cystic and bullous lung disease the annals of thoracic. There was a history of global initiative for chronic obstructive lung disease gold 1 stage iv chronic obstructive pulmonary disease copd with severe emphysematous changes on a regimen that included 02 liters of oxygen via nasal cannula initially. Bullous disease of the lungsconventional radiograph and ct. Bullous emphysema with mycetoma journal of copd foundation.
Copd patients with dyspnoea on exertion, forced expiratory volume in 1 s fev 1 bullous lung disease lidie lajoie, md suny downstate surgery grand rounds. Idiopathic giant bullous emphysema radiology reference. Chest radiology showed bilateral bullae predominantly of both upper and the left lower lobes and mild bronchiectasis. By the west jem article submission agreement, all authors are required to disclose all affiliations, funding sources and financial or management relationships that could be perceived as potential sources of bias. Bullous lung disease is a spectrum of disease with multiple causes, most commonly smoking. Paraseptal emphysema, characterized by multiple contiguous subpleural blebs, is seen along the subpleural space bd, arrows.