Pyoderma Gangrenosum Associated With Primary Biliary Cirrhosis

Maturi, Mary F.

doi:10.1001/archinte.1983.00350060193034

Cited by 13 publications

(6 citation statements)

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“…20,21 Important associations with some immunodeficiency or autoimmune disorders are also known, including hypogammaglobulinemia, 3,14,19 IgA deficiency, 7 congenital deficiency of complement factors C2 and C4, 22 human immunodeficiency virus (HIV) infection and impaired leukocyte function, Takayasu's arteritis, primary biliary cirrhosis, and systemic lupus erythematosus. [23][24][25][26][27] PG may be associated with a monoclonal gammopathy, 28 as demonstrated in our patient. IgA gammopathies are the most common type encountered, although IgG and IgM types have been documented.…”

Section: Discussionmentioning

confidence: 69%

“…Hematologic malignancies, such as myelodysplasia, acute myeloblastic leukemia and chronic myeloid leukemia, are sometimes associated with PG 20,21 . Important associations with some immunodeficiency or autoimmune disorders are also known, including hypogammaglobulinemia, 3,14,19 IgA deficiency, 7 congenital deficiency of complement factors C2 and C4, 22 human immunodeficiency virus (HIV) infection and impaired leukocyte function, Takayasu's arteritis, primary biliary cirrhosis, and systemic lupus erythematosus 23–27 . PG may be associated with a monoclonal gammopathy, 28 as demonstrated in our patient.…”

Section: Discussionmentioning

confidence: 99%

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Pyoderma gangrenosum, acne conglobata, and lgA gammopathy

et al. 2003

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A 62‐year‐old man was evaluated for chronic recurrent pyoderma gangrenosum (PG) of 8 years’ duration. He had a long history of acne conglobata beginning in his early twenties. Although periods of inactivity lasted for months, renewed cyst formation recurred on the face, trunk, and groin. Minocycline, 100 mg twice daily, was useful for the control of acne. Severe periodontal disease developed 25 years prior to presentation. He had coronary bypass surgery 12 years ago. His family history was noncontributory and negative for PG, acne conglobata, or follicular occlusion (FO) triad. The patient presented with an 8‐year history of small hemorrhagic pustules on the legs, progressing over the subsequent few months to large ulcers with raised borders. Spikes in temperature and general malaise for 3 or 4 days sometimes heralded new PG eruptions. On examination, large inflammatory, scarring cysts were present, predominantly on the face, lateral neck, back, and inguinal areas. Both lower legs showed small hemorrhagic pustules and scattered ulcers with raised, irregular borders (Figs 1–3). The larger ulcers often began as painful nodules, with central necrosis; peripheral expansion led to the formation of a central mucopurulent base surrounded by an undermined, ragged, erythematous or dusky, edematous ulcer rim. A vague, serpiginous halo of erythema surrounded these lesions. 1 Multiple necrotic ulcerations of pyoderma gangrenosum on the leg, demonstrating raised, inflammatory borders, a mucopurulent base, and a surrounding halo of erythema. Cribriform scarring is also apparent 2 Characteristic ulcer of pyoderma gangrenosum consists of a boggy, necrotic base with ragged, undetermined, violaceous edges surrounded by a halo of erythema 3 Close up of leg ulcer in Fig. 2 A biopsy specimen taken from the erythematous border of a leg ulcer showed a massive neutrophilic infiltrate with necrotizing suppurative inflammation and limited necrotizing vasculitis; direct immunofluorescence examination gave nonspecific results. Thrombosis of the small vessels, edema, and extravasation of erythrocytes were also noted. These histologic findings support the clinical diagnosis of PG. Serum protein electrophoresis revealed a monoclonal, immunoglobulin A (lgA) kappa‐type gammopathy. Bone marrow evaluation showed a slight elevation of plasma cells, suggesting a benign monoclonal gammopathy; urine analysis did not demonstrate Bence‐Jones protein. Evaluation for gastrointestinal, liver, and hematologic disease was negative. A complete blood cell count and blood chemistries gave results within normal limits. Oral prednisone, 60 mg daily, dapsone, 200 mg daily, and trimethoprim/sulfamethoxazole DS, twice daily, were administered. Prednisone was reduced to 30 mg every other day when the large ulcers of the legs ceased to expand, and regression was noted. Cribriform scarring was seen in areas of healing. Systemic prednisone eventually produced noticeable side‐effects, including bilateral cataract formation and the development of tachycardia. Iso...

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Section: Discussionmentioning

confidence: 69%

Section: Discussionmentioning

confidence: 99%

Pyoderma gangrenosum, acne conglobata, and lgA gammopathy

et al. 2003

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“…13 PG occurs in 0.5%-5.0% of patients with UC. 1,2 Approximately 30% of women with colitis in quiescence at the time of conception will relapse in preg-eicosanoids.…”

Section: Discussionmentioning

confidence: 99%

Pyoderma gangrenosum complicating ulcerative colitis: Successful treatment with methylprednisolone pulse therapy and cyclosporine

Futami

Kodaira

Furuta

et al. 1998

Journal of Gastroenterology

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may not be effective; in such cases, total colectomy is mandatory.We present a patient with extensive PG complicating UC who responded once to methylprednisolone pulse therapy, but relapsed during the tapering of prednisolone. Eventually, with pulse therapy plus cyclosporine, prednisolone was tapered without recurrence of PG. Case reportA 32-year-old woman was admitted because of severe PG and bloody stool on April 13, 1994. Six years previously, she had been diagnosed with UC that involved the rectum and the sigmoid and descending colon. Although PG occurred 2 years later, it and the UC had been well controlled until 1 year before admission, with maintenance therapy of 5 mg/day oral prednisolone. At 10 weeks' gestation, she had abdominal discomfort and passed bloody loose stools once or twice per day. The dose of prednisolone was increased to 10 mg, and the hematochezia and loose stool gradually decreased in frequency but still occurred occasionally. She gave birth normally on December 20, 1993 (Fig. 1). Four weeks after the delivery, active skin lesions developed on her face, neck, upper arm, and thigh (Fig. 2). The dose of prednisolone was increased to 20 mg/day but this failed to ameliorate the skin lesions. She was then hospitalized.On admission, she was well-nourished (56 kg, 154 cm) with cushingoid features. Body temperature was 37.3°C. Hemoglobin was 14.2 g/dl, white blood cell count 10 400 per mm 3 , erythrocyte sedimentation rate 34 mm/h, serum protein, 7.0 g/dl; serum albumin, 3.8 g/dl; aspartate aminotransferase (AST), 30 IU/l (11-30); alanine aminotransferase (ALT), 32 IU/l (5-42); lactic dehydrogenase (LDH), 259 IU/l (101-193); alkaline phosphatase, 34 IU/l; total bilirubin, 0.4 mg/dl; and C-reactive protein (CRP), 2.9 mg/dl (Ͻ0.15). Abstract:A 32-year-old woman with ulcerative colitis had a relapsed of pyoderma gangrenosum during puerperium. Both the pyoderma gangrenosum and ulcerative colitis had been well controlled with oral prednisolone, but ulcerative colitis relapsed in pregnancy, and pyoderma gangrenosum relapsed in the puerperium. The pyoderma gangrenosum responded to methylprednisolone pulse therapy initially, but relapsed when prednisolone was tapered. A second trial of pulse therapy combined with cyclosporine resulted in complete remission of the pyoderma gangrenosum, and no recurrence was recognized after prednisolone was tapered. This is a very rare case of successful treatment with methylprednisolone pulse therapy combined with cyclosporine for pyoderma gangrenosum complicating ulcerative colitis.

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“…The development of PG in a patient with primary biliary cirrhosis, a condition well known to be associated with alterations in delayed hypersensitivity and complement metabo¬ lism, was also recently described. 23 Striking increases in the numbers of patients with Crohn's disease and PG have been noted, with reports of eight and 14 cases in two large series.2425 The association is less common in patients with Crohn's disease limited to the small intestine or colon, usually occurring in patients with combined small-and large-bowel involvement.25 Most of these latter cases show other extraintestinal manifestations affecting the skin, joint, eyes, and oral cavity. Parastomal PG in patients with Crohn's disease has been recognized as a special problem,26 and in a case I recently observed, it appeared that even minor trauma (pathergy) at the ileostomy site contributed to the persistence and extension of ulcération.…”

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confidence: 99%

“…Pathology 1971;3:62-65. 23. Ackerman AB, Su WP: The histology of cutaneous malignant melanoma, in Kopf AW, Bart 25-36. 24.…”

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confidence: 99%