Pyoderma gangrenosum is a rare inflammatory condition with varying clinical presentations and severity. It is commonly seen in association with an underlying condition, most common of which is inflammatory bowel disease. We report a case of a 26-year-old male who came to the emergency department with increasing lower extremity ulcers, intermittent hematochezia, and pain in the small joints of his hands. After excluding a broad list of differentials for lower extremity ulcers, the diagnosis of pyoderma gangrenosum was made. He was also found to have erosive changes at multiple proximal interphalangeal joints and jug-like syndesmophytes at T12 and L1 on CT scan. Although there was evidence of a spondyloarthropathy, there was no evidence of inflammatory bowel disease on colonoscopy, psoriasis, or sexually transmitted infections. After multiple failed trials of medications including azathioprine and sulfasalazine, 4 weeks of Adalimumab resulted in rapid healing of pyoderma gangrenosum lesions and improvement in his synovitis. Coupled together, this suggests a diagnosis of pyoderma gangrenosum associated with undifferentiated spondyloarthropathy and erosive inflammatory arthritis. This case is suggestive of spondyloarthropathy going underdiagnosed and untreated in other patients with pyoderma gangrenosum as lower extremity ulcerations can be the primary complaint for seeking treatment. Although rare, axial spondyloarthropathy associated with pyoderma gangrenosum should be kept as an associated differential diagnosis when faced with pyoderma gangrenosum.
Pyoderma gangrenosum is a rare inflammatory condition with varying clinical presentations and severity. Typically, it presents as a papule or pustule that rapidly evolves to an ulcerative lesion with irregular, erythematous to violaceous edges. Pathergy is a characteristic feature of pyoderma gangrenosum as lesions often occur at sites of trauma. It has an estimated annual incidence of 3–10 cases per million people [
A 26-year-old Hispanic male with no significant past medical history came to the emergency department complaining of multiple wounds that gradually developed over the past 3 years. He reported a wound at the vertex of his scalp, posterior aspect of his neck, and on bilateral lower extremities. He reported the wounds initially started as small pimples, with progression in size and pain severity. The patient also reported serosanguineous drainage from his wounds, hematochezia, and pain in the small joints of his hands bilaterally, 9/10 in severity, associated with swelling and stiffness. The patient reported that his scalp lesions started 3 years prior to this admission and remained the same size since then. The lesions over his lower extremities started within 3 months of this admission and progressed so rapidly that it prompted him to go to the emergency department and seek help. He reported that his joint pain started approximately the same time as his scalp lesions and has progressively gotten worse over the past 3 years. He denied diarrhea, back pain, uveitis, iritis, sexually transmitted infections, abdominal pain, fever, or night sweats.
Physical exam was significant for a morbidly obese male in mild distress due to pain. Triage vitals were significant for mild tachycardia of 103 but otherwise were unremarkable. He had multiple wounds with serosanguineous drainage: ulcer at the vertex of the scalp measured 7 cm × 3 cm, posterior neck ulceration 5 cm × 2 cm, and multiple ulcers, of varying stages, circumferentially on bilateral lower extremities (Figure
Ulcerations on initial presentation. (a) Vertex of the scalp. (b) Base of the posterior neck. (c) Right lower extremity. (d) Left lower extremity.
Initial admission labs revealed leukocytosis of 12.0 × 103
(a) X-ray of the left hand with erosive changes at the 5th PIP joint. (b) X-ray of the right hand with erosive changes at the 2nd and 4th PIP joints.
Rheumatoid factor (RF), anticyclic citrullinated peptide (CCP), antinuclear antibodies (ANA), anti-Ro/SSA, anti-La/SSB, anti-RNP, complements levels, double-stranded DNA, anti-smith antibodies, anti-neutrophil cytoplasmic antibodies, HLA-B27, HIV, hepatitis B, hepatitis C, Quantiferon, and blood cultures were all negative. Punch biopsy of the left lower extremity edge revealed mixed dermal infiltrate with neutrophils and vasculitis. The tissue culture was positive for
CT enterography revealing jug-like syndesmophytes at T12 and L1, suggestive of spondyloarthropathy.
As there was no improvement of the ulcerative lesions with antibiotics, and in conjunction with syndesmophytes, enthesitis, and dactylitis, we started treatment for undifferentiated spondyloarthropathy associated with pyoderma gangrenosum. The patient was initially started on solumedrol 120 mg intravenously for 3 days followed by tapering doses of prednisone in combination with sulfasalazine. There was poor response after 6 weeks; thus, prednisone was discontinued and azathioprine was added to sulfasalazine. Azathioprine 100 mg daily and sulfasalazine 1000 mg twice daily were continued for 8 weeks with minimal response. In fact, the lower extremities ulcerations coalesced and increased in circumference. Due to lack of response to corticosteroids, sulfasalazine and azathioprine, he was subsequently started on adalimumab 40 mg subcutaneously every 14 days. Within four weeks, there was significant improvement (Figure
(a, b) Bilateral lower extremities prior to adalimumab initiation. (c, d) Bilateral lower extremities 4 weeks after Adalimumab treatment.
Pyoderma gangrenosum can have many different clinical presentations with varying degrees of severity. Clinically, it can present as classic or ulcerative subtype, bullous, pustular, vegetative, drug-induced, postsurgical, or peristomal types [
The exact pathophysiology of pyoderma gangrenosum is poorly understood but is believed to be a combination of genetics, irregular activation of inflammasomes, and both innate and adaptive immune systems [
The diagnosis of pyoderma gangrenosum is challenging, as it is ultimately a diagnosis of exclusion. Su et al. have proposed diagnostic criteria for pyoderma gangrenosum and require two major and two out of four minor criteria to establish the diagnosis [
Treatment of these cutaneous ulcerations is based upon the severity and the associated medical conditions. Although a direct association between severity of an associated condition and pyoderma gangrenosum has not been established, it is important that medications ideally be used that are beneficial for both conditions. Limited disease can be managed with wound care, topical, and intralesional therapy, whereas aggressive disease requires systemic immunomodulatory agents. However, no gold standard treatment exists as there are limited randomized controlled trials [
Initially, sulfasalazine was started for our patient’s pyoderma gangrenosum as there was suspicion for an associated inflammatory bowel disease, and colonoscopy had not been completed at this point. Subsequently after minimal response, azathioprine was added as a mild immunosuppressant because our patient was considered high risk for infection with his large, open wounds. However, he did not respond. Finally, adalimumab was started instead of other longer-acting biologics such as infliximab. We preferred adalimumab as it is dosed every two weeks and thus has a shorter half-life, in case the medication had to be discontinued in the future due to an infection. Our indication for an anti-TNF agent was our patient’s refractory pyoderma gangrenosum and enthesitis seen on ultrasound examination. The final diagnosis for our patient was pyoderma gangrenosum with superimposed pseudomonas infection associated with undifferentiated spondyloarthropathy.
Traditionally, pyoderma gangrenosum is commonly associated with inflammatory bowel disease, but this was not the case in our patient. He initially sought out treatment for ulcerations from pyoderma gangrenosum and then was subsequently found to have erosive inflammatory arthritis and undifferentiated axial spondyloarthropathy. This is suggestive of spondyloarthritis possibly going underdiagnosed and subsequently untreated in other patients with pyoderma gangrenosum. Thus, even though axial spondyloarthropathy may be rarely associated with pyoderma gangrenosum, it should be kept as an associated differential diagnosis when faced with pyoderma gangrenosum.
The authors declare that they have no conflicts of interest.