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Superficial granulomatous pyoderma, recently described as a variant of pyoderma gangrenosum, would be better termed pathergic granulomatous cutaneous ulceration as the seven previously described cases, as well as our own two cases, have significant dermal involvement histologically and heal with scarring. In contrast to pyoderma gangrenosum, lesions of superficial granulomatous pyoderma respond to less toxic anti-inflammatory agents.
OBJECTIVE: To evaluate the prevalence of IgA and IgG antineutrophil cytoplasmic antibodies (ANCAs) in erythema elevatum diutinum in comparison with 2 other groups of neutrophilic dermatoses: Sweet syndrome and pyoderma gangrenosum. DESIGN: Detection of IgA and IgG ANCAs in the serum of patients with neutrophilic dermatoses and characterization of the previously known antigenic targets. SETTING: All serum was analyzed without knowledge of diagnosis in the Immunology Department, Pitié-Salpêtrière Hospital, Paris, France. PATIENTS: Ten patients with erythema elevatum diutinum, 10 with Sweet syndrome, 10 with pyoderma gangrenosum, and 10 healthy volunteers. MAIN OUTCOME MEASURES: IgA and IgG ANCAs were sought by indirect immunofluorescence with ethanol and formaldehyde-fixed human neutrophil preparations as the substrate. Enzyme-linked immunosorbent assays were further performed for antigen characterization. RESULTS: IgA ANCAs were observed in 60% and IgG ANCAs in 10 (33%) of the patients. All patients with erythema elevatum diutinum had IgA ANCAs. IgA fluorescence in formaldehyde-fixed neutrophils was restricted to those from patients with erythema elevatum diutinum. Enzyme-linked immunosorbent assays disclosed no single predominant target, and antigens remained largely undetermined in erythema elevatum diutinum. CONCLUSIONS: The ANCAs, particularly of IgA class, may prove to be a helpful paraclinical marker in erythema elevatum diutinum and an interesting perspective for understanding the pathophysiology of the disease. The nature of the unidentified targets and the pathogenicity of ANCAs, however, remain to be assessed.
OBJECTIVE: To describe unusual clinical and radiological features in patients with chronic recurrent multifocal osteomyelitis (CRMO). DESIGN AND SUBJECTS: Based on radiographic and microscopic findings, three patients were prospectively diagnosed as having chronic recurrent multifocal osteomyelitis (CRMO). They form the basis of this report because of either the unusualness of the clinical presentation, hitherto undescribed associated diseases or the unusual age of presentation and sites of lesions. RESULTS: One patient developed pyoderma gangrenosum at the site of one of the skeletal lesions and then went on to develop ulcerative proctitis. A second patient presented with a soft tissue mass, which on MRI mimicked a sarcoma. The final patient presented with lesions in the wrist and phalanges of the toes at the unusual age of 38. None of the patients was treated with steroids or antibiotics for the skeletal lesions. Steroids were administered to one patient for treatment of pyoderma gangrenosum. CONCLUSIONS: The pattern and distribution of skeletal lesions in CRMO are well recognized in the pediatric age group. The unusual clinical and/or radiological features discussed herein suggests that this is a disease that continues to evolve with a broader spectrum of features than recognized.
Originally used to treat psoriasis nearly three decades ago, mycophenolic acid, reformulated as mycophenolate mofetil (MMF), has been rediscovered by the world of dermatology. As a relatively well-tolerated immunosuppressive used in organ transplant recipients, MMF has recently been reported to show promise for several dermatologic conditions, including psoriasis, pemphigus vulgaris, pyoderma gangrenosum, bullous lichen planus, and even connective tissue diseases such as lupus erythematosus and dermatomyositis. Although not intended to be exhaustive, this review discusses MMF with regard to its basic pharmacology, its side effects, and its reported efficacy in a variety of dermatologic indications. Relevant literature was retrieved by a Medline search combining the terms "mycophenolate" or "mycophenolic acid" and "skin" or "skin disease" or a number of specific conditions ("psoriasis", "dermatitis", "eczema", "pemphigoid", "pemphigus", "vasculitis", "pyoderma gangrenosum", "Crohn's disease", "graft-versus-host disease", "lichen planus"). As MMF has only been recently re-introduced for dermatologic application, the nature of much of the literature is admittedly that of case reports or case series. Nevertheless, the results are sufficiently promising to warrant further larger, control studies.
PURPOSE: Myelodysplastic syndromes are clonal hematologic disorders, expanded from myeloid stem cells. A primitive immunologic disorder is discussed. This hypothesis could explain a non-casual association with systemic diseases. The aim of our study is to test this hypothesis. METHODS: We retrospectively investigated the data of 60 patients with myelodysplastic syndromes (group I) hospitalized in our unit from 1990 to 1999. The frequency of systemic disorders was screened and compared to controls (group II). Group II consisted of 120 patients matched for age and sex and hospitalized in the same hospital during the same period. RESULTS: Sixty patients were included (mean age: 83 years old). Myelodysplastic syndrome subtypes were refractory anemia with excessive blasts (52%), refractory anemia (43%) and sideroblastic anemia (5%). Fourteen cases of systemic manifestations were reported in group I (23%) and five in the controls (4%) (P < 0.0001). Systemic manifestations in group I included vasculitis in six cases (42%), polyarthritis in three cases (21%), systemic amyloidosis AA in two cases (14%), relapsing polychondritis in one case, pyoderma gangrenosum in one case and celiac disease associated with a systemic granulomatosis in one case. In the controls, vasculitis was present in four cases and polyarthritis in one. Median age at onset of myelodysplastic syndrome was not influenced by the association with systemic disorders which, in return, have not influenced the myelodysplastic syndromes' subtypes. Myelodysplastic syndromes succeeded to systemic manifestations in 71.4% of cases and could not be attributed to immunosuppressive therapy. CONCLUSIONS: The association of myelodysplastic syndromes with systemic manifestations seems not to be casual. It raises the hypothesis of a primitive immunological disorder in both diseases. Moreover, the description of two cases of systemic amyloidosis and one case of pyoderma gangrenosum might suggest an additional disorder of macrophages or granular cells.
OBJECTIVE: The aim of this study was to determine the prevalence of the major extraintestinal manifestations of inflammatory bowel disease (IBD) and their relation to disease diagnosis and gender. METHODS: We used the population-based University of Manitoba IBD Database, which includes longitudinal files on all subjects of all health system contacts identified by International Classification of Diseases, 9th Revision, Clinical Modification codes for visit diagnosis. We extracted a cohort from our database, which included subjects with a known diagnosis of IBD for at least 10 yr. We then determined how many contacts each subject had for each of the following extraintestinal IBD-associated immune diseases: primary sclerosing cholangitis, ankylosing spondylitis, iritis/uveitis, pyoderma gangrenosum, and erythema nodosum. We calculated the prevalence of the extraintestinal diseases using an administrative definition of having at least five health system contacts for the diagnosis in question. This administrative definition has previously been validated in Crohn's disease and ulcerative colitis (UC). RESULTS: A total of 6.2% of patients with IBD had one of six major extraintestinal diseases studied in this report. Only 0.3% of patients had multiple extraintestinal diseases. Iritis/uveitis was the most common extraintestinal disease of all assessed (2.2% of women and 1.1% of men). Iritis/uveitis was more common among women, particularly those with UC (3.8%). Primary sclerosing cholangitis was most common among men with UC (3%). Ankylosing spondylitis was more common among men, and the highest rate was seen among men with Crohn's disease (2.7%). Pyoderma gangrenosum was more common in Crohn's (1.2%) with no gender predilection. Erythema nodosum was similarly present in Crohn's and UC but was more common among women (1.9%). CONCLUSIONS: The associations of immune mediated diseases in extraintestinal sites may help us to further our understanding of IBD pathogenesis, and it may help us in developing a paradigm of disease subsets.
OBJECTIVES: To describe a family from New Zealand with the pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome, an autoinflammatory, variably expressed, erosive destructive form of arthritis. METHODS: Information was gained through medical records and interviews of the affected patients and wider family. DNA sequencing was performed at Seay Center for Musculoskeletal Research Texas Scottish Rite Hospital for Children. RESULTS: Five patients were affected over three generations with an E250Q mutation found on the CD2BP1 gene on chromosome 15. Common features included a severe, pauciarticular-onset, destructive peripheral arthritis, beginning at ages 5, 5, 2, 3 and 1(1/2) years. This combination marked cervical ankylosis (in two members), micrognathia and a more severe phenotype is unique. A third-generation family member treated early with DMARDs is following a less severe course. The skin involvement was variable, all with degrees of acne from puberty, though only one patient displayed pyoderma gangrenosum. A clear pattern of the arthritis switching off in adolescence and the triggering of skin disease was observed. CONCLUSIONS: Differing degrees of joint destruction, and cervical ankylosis in this family with the E250Q mutation demonstrate PAPA syndrome's variable expression. Further understanding of this rare condition and its pathway may allow better targeting of treatments, not just for families with this specific syndrome but also for other, more common, forms of arthritis.
This case report describes the simultaneous occurrence of pyoderma gangrenosum and subcorneal pustular dermatosis. They are both neutrophilic dermatoses that have been described associated in several instances. The association of pyoderma gangrenosum and other cutaneous diseases is also reviewed.
The authors describe three cases of refractory anaemia with an excess of myeloblasts in the bone marrow (RAEM), associated with pyoderma gangrenosum (PG) and vasculitis. The first patient was an 85-year old man whose RAEM had begun in 1979. In 1985, he developed pyoderma gangrenosum in the popliteal fossa and on the right heel. Histology confirmed the diagnosis. A direct immunofluorescence test on the biopsy specimen was negative. Apart from the haematological syndrome, there were no laboratory abnormalities. The cutaneous lesions disappeared after 7 weeks of treatment with prednisolone 20 mg per day. The blood disease remained unchanged. The second patient was a 71-year old woman with RAEM since 1982. In 1984, she presented with lesions of cutaneous vasculitis located on the anterior aspect of the upper third of her left leg. There was neither arthralgia nor fever, and no history of drug toxicity or infection. Beside RAEM, polyclonal hyperglobulinaemia was present. Histological examination of the skin showed evidence of vasculitis with fibrinoid necrosis of vascular walls and perivascular lympho-histiocytic infiltrate with granulocytes and slight leucocytoclasia. Direct immunofluorescence testing of the skin demonstrated intravascular complement deposits. There were no circulating immune complexes; measurements of complement and complement fractions gave normal values; no cryoglobulin was found. The cutaneous lesions recurred on two occasions in 6 months, although no drug toxicity or infection could be elicited and the haematological syndrome was unaffected. The third patient was a 67-year old man with RAEM since 1982. In 1983, an ulcero-necrotic lesion spontaneously developed on his right leg.(ABSTRACT TRUNCATED AT 250 WORDS)
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Pyoderma gangrenosum is a distinctive, uncommon, ulcerative condition with an associated systemic disease in most cases. The causative mechanism of this disease is still unknown. In this article, we review the pathogenesis of pyoderma gangrenosum, its clinicopathologic picture and the currently available therapeutic alternatives. Associations with systemic disease are also reviewed.
PURPOSE: Although coexistence of Takayasu's arteritis with ulcerative colitis is often reported, all these cases have been in patients whose colons were in situ. The aim of the present study was to describe a patient who developed Takayasu's arteritis after total proctocolectomy for ulcerative colitis. METHODS: The clinical course of a 42-year-old female who developed Takayasu's arteritis after total proctocolectomy for ulcerative colitis is described and the literature reviewed. RESULTS: Symptomatic arteritis developed in the left carotid artery four months after total proctocolectomy with ileal J-pouch-anal anastomosis for ulcerative colitis. Peristomal pyoderma gangrenosum, multiple ulcers in the oral cavity, and leakage at the ileoanal anastomosis were observed simultaneously. High-dose therapy with steroids proved effective for the arteritis, pyoderma gangrenosum, and multiple oral ulcers. We finally excised the ileal pouch with anus because of the intractable leakage three years after the occurrence of Takayasu's arteritis. CONCLUSIONS: This patient illustrates the possibility that Takayasu's arteritis is an extraintestinal complication of ulcerative colitis even after surgical removal of the colon.
A 40-year-old Caucasian man presented to the dermatology clinic at Baylor College of Medicine, Houston, Texas, in February 2003, for the evaluation of three nonhealing ulcers. The patient's past medical history was significant for hypothyroidism and pulmonary sarcoidosis, the diagnosis of which was made in June 2000. In March 2000, the patient had complained of cough and shortness of breath. A purified protein derivative (PPD) (Mantoux text) was negative. Computed tomography (CT) scans of the chest revealed diffuse hilar and mediastinal adenopathy and bilateral interstitial and alveolar infiltrates. Although consistent with sarcoidosis, these findings were insufficient to exclude other etiologies, including disseminated fungal infection. Cultures and stains of subsequent bronchoscopy specimens failed to reveal any organisms, and histopathologic evaluation of the specimens was nondiagnostic. Based on the imaging studies and the negative cultures, a diagnosis of sarcoidosis was made, and the patient was started on therapy with prednisone. Before coming to our clinic, the patient had been on several courses of prednisone. In May 2002, the patient had presented to a private dermatologist with a 1-year history of a nonhealing 2.4 cm x 2.0 cm ulcer on the left medial forearm. Two biopsies were reported as nondiagnostic. The patient's presentation was interpreted as most consistent with Mycobacterium marinum infection, and so he was empirically treated with minocycline. This treatment was continued for almost 3 months without improvement in the ulcer. A few months after the minocycline had been discontinued, the patient was treated empirically for 2 months with ciprofloxacin. This treatment was also unsuccessful in ameliorating the ulcer. In between the two courses of antibiotics, specimens from the lesion were sent for bacterial and fungal cultures, which revealed normal skin flora. In January 2003, the patient returned to his private dermatologist with three ulcerations. In addition to the nonhealing ulcer on his left forearm, which he had acquired several months earlier, he had also developed a 3.0 cm ulcer on his right arm and a 3.0 cm ulcer on his central back. The patient refused biopsies at this visit. Given the patient's previous diagnosis of pulmonary sarcoidosis, it was thought that the skin lesions might represent ulcerative cutaneous sarcoidosis. Pyoderma gangrenosum was also considered to be a likely diagnosis. Therefore, the patient was started on a course of oral prednisone, an effective therapy for both sarcoidosis and pyoderma gangrenosum. Despite 1 month of treatment with 60 mg/day of prednisone, the ulcers increased, and the patient was subsequently referred to our clinic. Physical examination at the time of presentation revealed steroid acne on the trunk and upper extremities and three non-tender ulcers with erythematous, undermined borders (Figs 1-3). On the left arm, there was an adjacent nodule which the patient attributed to a scar from a previously healed ulcer. Histologic examination of biopsy specimens from all three sites showed similar findings. The lesion contained diffuse, suppurative, granulomatous, inflammatory infiltrates with extensive central necrosis. The infiltrates were composed of histiocytes, multinucleated foreign-body-type giant cells, plasma cells, lymphocytes, neutrophils, and neutrophil fragments. No organisms were seen in the initial, routinely stained sections. However, periodic acid-Schiff (PAS) staining demonstrated small fungal spores (Fig. 4) morphologically consistent with sporotrichosis, within the cytoplasm of multinucleated histiocytic giant cells (Fig. 5). Additional stains for bacteria and acid-fast organisms were negative. Cultures of the biopsy specimens from all three sites grew Sporothrix schenckii. Further questioning of the patient failed to reveal an obvious source of the infection. The patient denied any history of traumatic skin inoculation and did not engage in gardening or other outdoor activities that are classically associated with sporotrichosis. The patient did admit to blackberry picking on detailed retrospective questioning. Once the diagnosis of sporotrichosis was made, the patient was given 200 mg/day of itraconazole. After 2 months, the patient's ulcers were almost completely healed. The patient's pulmonary complaints were also much improved.
OBJECTIVE: To review the data surrounding the use of tacrolimus for skin disorders. DATA SOURCES: Articles were obtained through a MEDLINE search of English-language literature (1990-May 2000); references of the retrieved publications were further reviewed for relevant literature. STUDY SELECTION: Orginal studies in humans regarding the use of tacrolimus for skin disorders were included. DATA EXTRACTION: The major outcomes extracted from the literature involved patient response to therapy and adverse effects. DATA SYNTHESIS: Tacrolimus offers an additional therapeutic approach to the treatment of immunologically based skin disorders. led trials and case reports indicates topical tacrolimus is a safe and effective alternative treatment in patients with atopic dermatitis. Case reports document efficacy in recalcitrant pyoderma gangrenosum, mucosal lichen planus, and ichthyosis linearis trolled trials and case reports indicate oral tacrolimus is effective in recalcitrant plaque psoriasis. Case reports document efficacy in recalcitrant pyoderma gangrenosum and leukocytclastic vascultis. The clinical utility of oral tacrolimus in skin disorders is limited due to potentially severe adverse effects such as infections, hypertension, hyperglcemia hyperkalemia, nephrotoxicity neurotoxicity, and increased risk of neoplasia. CONCLUSIONS: Most of the available data indicate short-term (3 wk to 3 mo) topical tacrolimus is a safe and effective treatment alternative for inflammatory skin disorders. Further study is needed to evaluate long-term safety and efficacy and to determine the best dosage regimen. Although oral tacrolimus has demonstrated efficacy in inflammatory skin disorders, the potenial for serious adverse effects limits its utility to third-line therapy for patients who are resistant to, or intolerant of, conventional therapies.
BACKGROUND/AIMS: Evaluation of clinical, demographic and epidemiologic features of ulcerative colitis and therapy response of these patients. METHODOLOGY: Retrospectively, 116 patients (64.7% female) were enrolled in this study. Mean age and mean follow-up period were 36+/-16, 5+/-2 years, respectively. RESULTS: The most common anatomic involvement was pancolitis (60.3%) and the others were as follows: left side 25%, rectum 13.8% and ileum (backwash ileitis) 0.9%. Extraintestinal manifestations were observed in 42.2% of patients (sacroiliitis 12%, primary sclerosing cholangitis 7.6%, pyoderma gangrenosum 2.4%, peripheral arthropathy 1.6%, autoimmune hepatitis 1.6%, steatosis 12.9%, gallstone 0.8%, perianal fistulas 0.8%, sagittal sinus thrombosis 0.8%, psoriasis 0.8%). Multiple extraintestinal manifestations were observed (primary sclerosing cholangitis + pyoderma gangrenosum) in 2 patients. Colonic perforation due to toxic megacolon was observed in only one patient during follow-up period. The mean period for remission was 3.7 months in 72% of patients with pancolitis by the treatment of 5-aminosalicylic acid (5-ASA) (2-3 gr/day) + methylprednisolone (1 mg/kg/day) +/- (for maintenance of remission) azathioprine (AZT) (1.5-2 mg/kg/day); 3.7 months in 72% of left-sided colitis by 5-ASA +/- corticosteroid enemas +/- methylprednisolone (1 mg/kg/day) +/- (for maintenance of remission) AZT (1.5-2 mg/kg/day) and 3.7 months in 62.5% of patients with distal colitis by 5-ASA (p.o. +/- enema) +/- corticosteroid enemas. Colectomy was performed on 7 patients refractory to these treatments. Six patients (4 of them had pancolitis) were treated with cyclosporine (Cys) (4 mg/kg/day, p.o.). Only one patient, a non-responder to Cys therapy, had colectomy. The mean remission time was 4.7 months in 80.6% of patients with extraintestinal involvement and 3.2 months in 71.2% of patients without extraintestinal involvement (P=0.002). CONCLUSIONS: Medical therapy was generally enough for the treatment. Cys and/or surgery can be another choice for the patients that do not respond to the medical therapy. Extraintestinal manifestations do not change the remission rate, but prolong the time to catch remission.
The association between ulcerative colitis and various abnormalities of the skin, especially pyoderma gangrenosum, has been accepted for many years. The underlying aberrations in either of the organ systems, as well as the basis of their association, however, remain obscure. Several large series of ulcerative colitis patients are reviewed to emphasize the important concomitant cutaneous findings, and investigations into the pathogenesis of pyoderma gangrenosum are discussed.
INTRODUCTION: Prolonged topical application of silver sulfadiazine cream can induce argyria and adverse effects of sulphonamides. We report a case of a woman with acute renal failure following repeated applications of topical silver sulfadiazine on pyoderma gangrenosum wounds. CASE REPORT: A 61 year-old woman suffering from rheumatoid arthritis, Sjogren's syndrome and scleroderma was treated with corticosteroids (1 mg/kg/day) and topical application of silver sulfadiazine cream (200 g/day) for extensive pyoderma gangrenosum wounds on the legs. Three weeks later, the patient was transferred to intensive care because of pulmonary edema, oligoanuria and disrupted consciousness. Laboratory data revealed leukopenia (1100/mm(3)) with neutropenia and renal failure (serum creatinine 316 micromol/l). Proteinuria was moderate and ultrasonography of the kidneys was normal. Silver concentration in blood was 1818 nmol/l (N < 92 nmol/l) and 1381 nmol/l (N < 9 nmol/l) in urine. Sulfadiazine concentration in blood was undetectable. All the signs regressed after withdrawal of silver sulfadiazine and after several sessions of hemodialysis. DISCUSSION: Various causes of renal failure are discussed in our patient. However, direct silver-induced renal toxicity is the most likely and is confirmed by the high concentration of silver in blood and urine and the improvement on withdrawal of the topical cream, without modification in the oral treatment. The absence of red corpuscles and crystals in the urine and undetectable concentrations of sulfadiazine in blood are not in favor of sulphonamide renal toxicity. Furthermore, the autoimmune diseases of our patient were well-controlled. Leukopenia could be secondary to silver sulfadiazine medullar toxicity. This observation confirms that this topical cream should not be used for long periods on extensive wounds.
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