Pyoderma gangrenosum with spleen involvement and monoclonal IgA gammopathy

Mijušković, Z̆p; Zecević, R; Pavlović,

doi:10.1111/j.1468-3083.2004.01031.x

Cited by 18 publications

(21 citation statements)

References 12 publications

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Section: -14contrasting

confidence: 48%

“…The reported cases of severe disseminated multilocular PG in association with UC with concomitant painful oral involvement are extremely rare. [15][16][17][18] There have been some reports linking oral PG with IBD and hematological disorders, 19,20 however this was not the case in our patient where no hematological disorder was present.Clinically, the oral lesions are usually painful, appear as irregular shaped ulcers 15 mm to 20 mm in diameter with rolled out margins and a grayish colored base 6,21 with most common sites of involvement being the tongue, palate, lips, buccal mucosa, gingiva and tonsillar fauces. 20,21 In our case, both tongue and buccal mucosa were severely affected.…”

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confidence: 48%

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Disseminated refractory pyoderma gangraenosum during an ulcerative colitis flare. Treatment with infliximab.

Zampeli¹,

Lippert²,

Nikolakis³

et al. 2015

J Dermatol Case Rep

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Background: Pyoderma gangraenosum is an immune-mediated, inflammatory, neutrophilic dermatosis of unknown etiology, which represents one of the extraintestinal manifestations of inflammatory bowel disease. It is a rare disease that occurs in less than 1% of patients with inflammatory bowel disease and with the same ratio in patients with Crohn's disease and ulcerative colitis. Main observations:A 36-year-old woman was diagnosed with ulcerative colitis 6 years before admission to our dermatology department with an acute disseminated pyoderma gangraenosum with mucosal involvement, during a flare of ulcerative colitis. Disease progression was interrupted by intravenous administration of the tumor necrosis factor-α inhibitor infliximab at 5mg/kg at weeks 0, 2, and 6 (1st cycle) and every 8 weeks thereafter. Improvement of intestinal, skin and oral manifestations was evident already after the 1st cycle of treatment and has been maintained since (at least 16 months). Conclusions:This case report is one of very few on disseminated pyoderma gangraenosum with oral involvement complicating ulcerative colitis, where infliximab was shown to have a rapid efficacy on skin, mucosal and bowel symptoms. (J Dermatol Case Rep. 2015; 9(3): 62-66) Disseminated refractory pyoderma gangraenosum during an ulcerative colitis flare. Treatment with infliximab. IntroductionPyoderma gangraenosum (PG) is a severe ulcerating, primarily sterile, neutrophilic dermatosis. It can occur in association with systemic diseases in adults, the most common of which is ulcerative colitis (UC). 1 PG has been reported in 1-10% of patients with UC, 2 affecting equally men and women with a peak age incidence between 25 and 54 years. Approximately 50-78% of patients with PG have an underlying systemic disease 3 such as inflammatory bowel disease (IBD), myeloproliferative and rheumatological diseases. 4 PG has been reported to occur before, during, after or even independently of the onset of an underlying IBD. 5Four clinical and histological variants of PG have been described: ulcerative, pustular, bullous, and vegetative. 4 We present here a female patient with severe, disseminated PG, refractory to conventional treatment, during a clinical flare of a previously diagnosed UC. Both entities responded to infliximab, a chimeric monoclonal antibody that inhibits tumor necrosis factor-α (TNF-α). Case report Medical HistoryA 36-year-old female was diagnosed with UC in 2005. The leading symptom was bloody diarrhea. Under treatment with mesalazine, remission was maintained for nearly 5 years. In October 2010, the patient relapsed with bloody diarrhea (at DOI: http://dx.doi.org/10.3315/jdcr.2015DOI: http://dx.doi.org/10.3315/jdcr. .1206 62 least eight stools per day), fever and diffuse abdominal pain with increasing severity of the symptoms. She was admitted to an internal medicine department, where she was treated with antibiotics (levofloxacin and metronidazol), mesalazine, sulfasalazin and low-dose corticosteroids (20 mg prednisone daily). After tw...

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Section: -14contrasting

confidence: 48%

contrasting

confidence: 48%

Disseminated refractory pyoderma gangraenosum during an ulcerative colitis flare. Treatment with infliximab.

Zampeli¹,

Lippert²,

Nikolakis³

et al. 2015

J Dermatol Case Rep

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“…Such is the case with pulmonary involvement, which is the most common. We found only 5 such patients in the literature with features consistent with abscess-like lesions (Patients [39][40][41][42][43]. Other kinds of neutrophilic dermatosis may be concerned: 1 patient with PG-associated AA also had a subcorneal pustular dermatosis (Patient 36).…”

Section: Close/overlap Conditionsmentioning

confidence: 87%

Aseptic Abscesses

André¹,

et al. 2007

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Aseptic abscesses (AA) are characterized by deep, sterile, round lesions consisting of neutrophil that do not respond to antibiotics but improve dramatically with corticosteroids. We report the clinical, laboratory, and radiologic characteristics and the associated conditions of 29 patients from the French Register on AA plus 1 patient from the Netherlands.The mean age of patients at AA diagnosis was 29 years (SD = 14). The main clinical manifestations of AA were fever (90%), abdominal pain (67%), and weight loss (50%). Duration of symptoms was 4.7 months on average until abscesses were discovered. The abscesses involved the spleen in 27/29 patients (93%; the thirtieth patient had a personal history of splenectomy after a trauma). In 7 they were unifocal and in the others they were multifocal, involving in addition the abdominal lymph nodes in 14 (48%), liver in 12 (40%), lung in 5 (17%), pancreas in 2 (7%), and brain in 2 (7%). They were not splenic in 3, including 2 with abdominal lymph nodes and 1 with superficial lymph nodes and testicle and lung involvement. Twenty-two patients (70%) had elevated white blood cell and neutrophil count; antineutrophil cytoplasmic autoantibodies with a perinuclear, cytoplasmic or atypical pattern (negative for antiproteinase 3 and negative for antimyeloperoxidase except for 1) were positive in 21% of the 24 patients tested. Twenty-one patients had inflammatory bowel disease (IBD), which preceded the occurrence of abscesses in 7, was concomitant in 7, and appeared secondarily in 7. Six patients had neutrophilic dermatosis (20%), 3 had relapsing polychondritis as an associated condition, and 3 others had monoclonal gammopathy of undetermined significance. Three patients had no associated condition. Splenectomy was performed in 15 (52%) patients. All patients received steroid therapy. Thirteen (43%) were given additional immunosuppressive therapy, 1 immediately and the others after a relapse, of whom 3 were also treated by anti-tumor necrosis factor-alpha agents. Mean follow-up was 7 years. Eighteen (60%) patients experienced 1 or several relapses, but there was no death related to AA. Relapses occurred on immunosuppressive therapy in 2 patients and off immunosuppressive therapy in the others while corticosteroids were being tapered. We surveyed the literature and analyzed 19 additional cases. AA is an emergent and probably underrecognized entity. It represents an apparently noninfectious inflammatory disorder involving neutrophils that responds to corticosteroid therapy. AA mainly affects patients with IBD but also affects those with other conditions, or with no other apparent disease.

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“…3 &4]. Extracutaneous manifestations include involvement of upper airway mucosa, eye [9-12], genital mucosa [13], sterile pulmonary neutrophilic infiltrates [14] or spleen infiltrates [15], and neutrophilic myositis [16]. Sterile cortical osteolysis has been observed adjacent to PG ulcers as another extracutaneous manifestation of the disease [17].…”

Section: Clinical Presentationmentioning

confidence: 99%

Pyoderma gangrenosum – a review

Wollina

2007

Orphanet J Rare Dis

240

246

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Pyoderma gangrenosum (PG) is a rare noninfectious neutrophilic dermatosis. Clinically it starts with sterile pustules that rapidly progress and turn into painful ulcers of variable depth and size with undermined violaceous borders. The legs are most commonly affected but other parts of the skin and mucous membranes may also be involved. Course can be mild or malignant, chronic or relapsing with remarkable morbidity. In many cases PG is associated with an underlying disease, most commonly inflammatory bowel disease, rheumatic or haematological disease and malignancy. Diagnosis of PG is based on history of an underlying disease, typical clinical presentation, histopathology, and exclusion of other diseases that would lead to a similar appearance. The peak of incidence occurs between the ages of 20 to 50 years with women being more often affected than men. Aetiology has not been clearly determined yet.The treatment of PG is a challenge. Randomized, double-blinded prospective multicenter trials for PG are not available. The best documented treatments are systemic corticosteroids and ciclosporin A. Combinations of steroids with cytotoxic drugs are used in resistant cases. The combination of steroids with sulfa drugs or immunosuppressants has been used as steroid-sparing modalities. Antitumor necrosis alpha therapy in Crohn's disease showed a rapid response of PG. Skin transplants and the application of bioengineered skin is useful in selected cases as a complement to the immunosuppressive treatment. Topical therapy with modern wound dressings is useful to minimize pain and the risk of secondary infections. Despite recent advances in therapy, the prognosis of PG remains unpredictable.

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Pyoderma gangrenosum with spleen involvement and monoclonal IgA gammopathy

Cited by 18 publications

References 12 publications

Disseminated refractory pyoderma gangraenosum during an ulcerative colitis flare. Treatment with infliximab.

Disseminated refractory pyoderma gangraenosum during an ulcerative colitis flare. Treatment with infliximab.

Aseptic Abscesses

Pyoderma gangrenosum – a review

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