SeminarPyoderma gangrenosum
Section snippets
Classical
The ulceration of classical pyoderma gangrenosum is frequently characteristic. The border is well defined and deep erythematous to violaceous in colour (figure 1). The lesion extends peripherally and the border often overhangs the ulceration as the inflammatory process spreads within the dermis, only secondarily causing necrosis of the epidermis. The lesions may be single or in crops, often beginning as a discrete pustule with a surrounding inflammatory erythema. Lesions frequently progress
Pathology
The histopathological features of pyoderma gangrenosum, though not specific, are useful in ruling out other causes of ulceration. Microscopy reveals massive neutrophilic infiltration, haemorrhage, and necrosis of the epidermis. The process may simulate an abscess or cellulitis. Some classify the initial infiltrate as a neutrophilic dermatosis2, 3 while others believe that the initial changes involve lymphocytic infiltrate, endothelial cell swelling, and fibrinoid necrosis of the vessel wall
Aetiology and associated disorders
The aetiology and pathogenesis of pyoderma gangrenosum are not understood but abnormalities of the immune system have been described. Although associated conditions are common (panel 1) between one-quarter and one-half of patients have “idiopathic” disease. The most common associated conditions are IBD, arthritis, paraproteinaemia, and haematological malignancy.
Diagnosis and differential diagnosis
The diagnostic evaluation (panel 2) of a patient presumed to have pyoderma gangrenosum has two objectives—to rule out other causes of cutaneous ulceration and to determine whether there is a treatable systemic, associated disorder. There is no specific laboratory or histopathological test. Moreover, some of the associated disease processes may be clinically silent.
The differential diagnosis of ulcerative cutaneous lesions include infectious disease, malignancy, vasculitis, insect bites, venous
Therapy
There is no specific, uniformly effective treatment for pyoderma gangrenosum,29 and no treatment listed here has been subjected to a controlled trial. So the choice of treatment rests with the clinician and the patient. The risks and the potential for benefit and how long therapy should be undertaken before the patient improves should be discussed. With associated IBD and, possibly, haematological disease, there is an opportunity to address the underlying disorder, which can affect the skin
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