Author: Dr Kate Dear, Senior House Officer, St Mary’s Hospital, London, United Kingdom. DermNet New Zealand Editor in Chief: A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell, November 2017.
A respiratory disease refers to any condition that affects the upper respiratory tract, trachea, lungs, and the nerves and muscles associated with breathing. The correct recognition of cutaneous signs of respiratory disease can aid clinicians in diagnosing and treating these potentially life-threatening diseases.
What are the skin signs of respiratory disease?
Cyanosis
Cyanosis is a blue discolouration of the skin and mucous membranes. It is seen in patients with more than 5 g/dL of desaturated haemoglobin. Cyanosis may be:
Central — on the lips and tongue; relates to a circulatory or respiratory problem associated with poor blood oxygenation in the lungs
Peripheral — on the extremities or fingers; due to oxygen-depleted peripheral blood.
Cyanosis
Cyanosis and clubbing
Nail clubbing
Nailclubbing is a deformity of the nails characterised by:
Loss of the Lovibond angle (the normal < 165° angle between the nail bed and the nail fold)
Thickening of the end of the finger
An increase in nail fold convexity
A soft boggy nail texture [1].
Clubbing is associated with a wide range of diseases, including the following respiratory diseases:
Lung cancer
Interstitial lung disease; most commonly, fibrosing alveolitis (inflammation of the alveoli in the lungs)
Suppurative (pus-forming) lung disease, such as lung abscess, empyema (pus buildup in the pleural cavity), and bronchiectasis (damage to the lungs due to infections)
Mesothelioma (a tumour of the lining of the lung)
Arteriovenous fistula or malformation (abnormal formation or connection between arteries and veins).
Periostitis (inflammation of the layer of connective tissue around a bone)
Arthritis (inflammation of a joint).
Symptoms of hypertrophic osteoarthropathy may precede the diagnosis of lung cancer and are most commonly associated with non-small-cell lung cancer, particularly squamous cell carcinoma and adenocarcinoma [2]. Hypertrophic osteoarthropathy can also be secondary to a lung abscess, mesothelioma, and other disorders [1].
Finger clubbing
Clubbing of the nails
Lung cancer
Cutaneous metastases in lung cancer are found in 2–8% of cases of lung cancer [1]. The most common sites are the chest and abdominal wall, neck, and scalp. They are usually rapidly growing, hard, painless and mobile nodules. A punch biopsy of a nodule may be nonspecific or may establish the diagnosis of lung cancer.
Monitoring changes in the size of skin nodules can help assess the patient's response to chemotherapy [1]. Other cutaneous manifestations of lung cancer include:
Superior vena cava syndrome is due to obstruction of the superior vena cava, the vein that takes deoxygenated blood from the upper body to the heart. The majority of cases are due to malignancy; most commonly, non-small-cell lung cancer, small-cell lung cancer, lymphoma, and metastatictumours [4]. Superior vena cava syndrome presents with markedly dilated veins or venules in the upper chest [5]. These occur as a result of increased collateral flow through the superficial vasculature in the chest wall.
Sarcoidosis
Sarcoidosis can be divided into two separate conditions:
A multisystem disease, with 30% of patients having cutaneous involvement [6]
Cutaneous sarcoidosis, in which there is no systemic involvement [7].
Cutaneous involvement is classified as either specific, in which there are non-caseatinggranulomas (mixed chronicinflammatory cells) on histopathology, or nonspecific, in which granulomas are absent.
Papules and nodules may become necrotic, starting with a central black dimple.
Vasculitis in eosinophilic granulomatosis
Ulcer
Erythema + eschar
Purpura
Pulmonary arteriovenous malformations
Pulmonary arteriovenous malformations are abnormal communications between the arteries and veins that supply the lungs. The majority of these malformations are congenital. Approximately 70% of patients with pulmonary arteriovenous malformations have associated hereditary haemorrhagic telangiectasia (also known as Osler–Weber–Rendu syndrome) in which telangiectasia is near the surface of the skin. The face, hands, feet, chest, lips, tongue, oral mucosa, and nasal mucosa are most commonly affected [1].
Alpha-1-antitrypsin deficiency
Alpha-1-antitrypsin deficiency is a genetic disorder where there is defective production of the alpha-1-antitrypsin enzyme. It primarily affects the lungs (with emphysema), liver, and skin. Cutaneous findings include recurrent ulcerative, necrotising panniculitis (inflamed subcutaneous fat with crops of painful, red subcutaneous nodules), which most noticeably occurs on the trunk and proximal extremities. These nodules typically ulcerate, releasing a clear to yellow oily fluid [1].
Fat embolism syndrome
Fat embolism syndrome typically follows fractures and trauma to the long bones or pelvis. Fat particles released into the circulation may cause:
Respiratory distress
Altered mental status
Thrombocytopenia.
The classic cutaneous manifestation of fat embolism syndrome is a petechialeruption with 2–3 mm purpuricmacules on non-dependent portions of the body (upper chest, neck, axillae, and conjunctivae). This finding often disappears within 5–7 days, but may be key for diagnosis [10,11].
Lymphoedema (swelling caused by blockage of the lymphatic system)
Pleural effusions (fluid in the space surrounding the lungs) [8].
Nail changes that are usually the first features of yellow nail syndrome include:
Onycholysis (separation of the nail from the nail bed)
Transverse ridging on a smooth base
Loss of the cuticle
Slow growth (less than half the rate of healthy nails) [7].
The nails are occasionally green in colour. Lymphoedema associated with yellow nail syndrome typically affects the legs and is symmetrical and non-pitting.
Yellow nail syndrome
Yellow nail syndrome
Yellow nail syndrome
Birt–Hogg–Dubé syndrome
Birt–Hogg–Dubé syndrome is an autosomal-dominant inherited disease characterised by skin tumours, pneumothorax (lung collapse), kidney tumours, and lung cysts [12].
Fibrofolliculomas and trichodiscomas are most frequently found on the head, face, and upper body.
They are asymptomatic, pale yellow, slightly raised, dome-shaped papules measuring 2–4 mm in diameter.
There may be few or many hundreds of lesions.
They usually develop at around 30–40 years of age [1].
Trichofolliculomas in Birt-Hogg-Dubé syndrome
Fibrofolliculomas in Birt Hogg Dube syndrome
Fibrofolliculomas in Birt Hogg Dube syndrome
Fibrofolliculomas in Birt Hogg Dube syndrome
References
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