Pyoderma gangrenosum coexisting with a myeloproliferative disorder

Introduction

Pyoderma gangrenosum is a rare disease from the group of neutrophilic dermatoses due to the large neutrophilic infiltrations on the skin and thus leading to secondary damage of vessels. Even though neutrophilic infiltrations in pyoderma gangrenosum are present in tissues bacterial infections do not play a direct role in its pathogenesis. Culture of skin and blood are always negative. Antibiotic therapy does not influence the course of the disease. It is believed that proinflammatory cytokines and growth factors take part in the process [1]. Pyoderma gangrenosum at first takes the form of a small disorder imitating a furunculus or a cyst, a small pustule, which becomes necrotic and rapidly evolves into a large ulceration. The disorder creates ulcers rapidly and spreads in a circumferential fashion, creating a large but relatively topical ulceration with a swollen necrotic ulcer bed and heaped up ruby red edges. The edges are usually undermined, blue-erythematous or violet, irregular, heaped up with presence of fistulas, partially covered with a necrotic eschar, with presence or absence of granulation tissue. Pustules are present in the active edge as well as in the bottom of the ulceration [2, 3].
The ulceration is sometimes preceded by an inflammatory infiltration or a cystic reaction [4]. The skin disorders disappear, leaving scars; the scars created in the healing process are described as sieve-like with numerous tiny holes and pits [2, 3]. One of the symptoms of pyoderma gangrenosum is pathergy – changed reactivity, which consists of appearance of skin disorders due to injuries, often very minor. Pathergy is present in 25% of pyoderma gangrenosum. If the disease is not properly diagnosed and is radically surgically treated it will progress in circumferential fashion [5]. So in a case of a suspicion of pyoderma gangrenosum it is contraindicated to conduct any procedures because they can have a negative effect on the progress of the disease. Pyoderma gangrenosum is often accompanied by systemic diseases, such as inflammatory bowel disease, autoimmune connective tissue diseases, internal organ and hematopoietic system malignancies, especially myeloproliferative disorder (MPD), acute and chronic leukemic myelosis and multiple myeloma [6, 7]. Presence of other diseases influences the progression of pyoderma gangrenosum and the prognosis of the underlying pathology influences it as well. If the underlying disease is treatable,...


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