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PubMed2005

Therapy Insight: pyoderma gangrenosum-old disease, new management.

Well-designed studies that help guide physicians to apply the optimal therapeutic strategy for the management of pyoderma gangrenosum are lacking in the literature. A multidisciplinary approach is paramount for the effective management of this condition, with close involvement of a wound-care specialist and a microbiologist. Treatment should be stepwise in nature. Local wound care, avoidance of trauma and the application of local steroid or tacrolimus ointment are the first-line treatments. Steroid therapy is the most widely published effective therapy for achieving resolution of pyoderma gangrenosum, although there is growing evidence for the efficacy of infliximab in refractory cases. Other therapies such as dapsone and clofazamine should be left as third-line agents for refractory pyoderma gangrenosum, while novel treatments such as granulocyte apheresis should only be used under trial conditions, to gain an objective evaluation of their efficacy. This article reviews the published treatment strategies in current use, and aims to guide the effective management of pyoderma gangrenosum.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2001

Suspected induction of a pyoderma gangrenosum-like eruption due to sulpiride treatment.

A case of a pyoderma gangrenosum (PG)-like eruption due to the antipsychotic drug sulpiride, a form of risperidone, is described. The contribution of sulpiride to the etiology of the PG-like lesion is based on the reduction and healing of the ulcer upon cessation of the drug and the formation of a bulla following the drug's re-administration. The literature on drug-induced PG or PG-like eruptions is discussed. The selectivity of sulpiride for dopamine receptors and its limited effect on other neuronal pathways differentiates sulpiride from other types of antispychotic drugs commonly used in Israel, including phenothiazine, butyrophenone, and thioxanthene. Adverse systemic and cutaneous reactions to sulpiride and to risperidone are described. To our knowledge, this is the first report of a PG-like eruption due to the former.

Antipsychotic Agents↗
PubMed2002

Mycosis fungoides presenting with extensive pyoderma gangrenosum-like ulcers.

Mycosis fungoides (MF) may present with atypical clinical manifestations. Usually it mimics various chronic dermatoses, with the appearance of ulcers during the tumour stage. Infrequently, cutaneous ulcers are the main or initial sign of lymphoma. We report the case of a man who presented multiple skin lesions that clinically appeared to be pyoderma gangrenosum (PG). However, histological and immunohistochemical examination revealed MF. This case illustrates that PG-like ulcers maybe atypical cutaneous manifestations of MF and exceptionally the presenting sign of this disease.

Biopsy, Needle↗
PubMed2001

Disseminated histoplasmosis presenting as pyoderma gangrenosum-like lesions in a patient with acquired immunodeficiency syndrome.

A 33-year-old Hispanic woman with newly diagnosed human immunodeficiency virus (HIV) infection, a CD4 T-lymphocyte count of 2, viral load of 730,000 copies/mL, candidal esophagitis, seizure disorder, a history of bacterial pneumonia, and recent weight loss was admitted with tonic clonic seizure. On admission, her vital signs were: pulse of 88, respiration rate of 18, temperature of 37.7 degrees C, and blood pressure of 126/76. Her only medication was phenytoin. On examination, the patient was found to have multiple umbilicated papules on her face, as well as painful, erythematous, large, punched-out ulcers on the nose, face, trunk, and extremities of 3 months' duration (Fig. 1). The borders of the ulcers were irregular, raised, boggy, and undermined, while the base contained hemorrhagic exudate partially covered with necrotic eschar. The largest ulcer on the left mandible was 4 cm in diameter. The oral cavity was clear. Because of her subtherapeutic phenytoin level, the medication dose was adjusted, and she was empirically treated with Unasyn for presumptive bacterial infection. Chest radiograph and head computed tomography (CT) scan were within normal limits. Sputum for acid-fast bacilli (AFB) smear was negative. Serologic studies, including Histoplasma antibodies, toxoplasmosis immunoglobulin M (IgM), rapid plasma reagin (RPR), hepatitis C virus (HCV), and hepatitis B virus (HBV) antibodies were all negative. Examination of the cerebrospinal fluid was within normal limits without the presence of cryptococcal antigen. Blood and cerebrospinal cultures for bacteria, mycobacteria, and fungi were all negative. Viral culture from one of the lesions was also negative. The analysis of her complete blood count showed: white blood count, 2300/microl; hemoglobin, 8.5 g/dL; hematocrit, 25.7%; and platelets, 114,000/microl. Two days after admission, the dermatology service was asked to evaluate the patient. Although the umbilicated papules on the patient's face resembled lesions of molluscum contagiosum, other infectious processes considered in the differential diagnosis included histoplasmosis, cryptococcosis, and Penicillium marnefei. In addition, the morphology of the ulcers, particularly that on the left mandible, resembled lesions of pyoderma gangrenosum. A skin biopsy was performed on an ulcer on the chest. Histopathologic examination revealed granulomatous dermatitis with multiple budding yeast forms, predominantly within histiocytes, with few organisms residing extracellularly. Methenamine silver stain confirmed the presence of 2-4 microm fungal spores suggestive of Histoplasma capsulatum (Fig. 2). Because of the patient's deteriorating condition, intravenous amphotericin B was initiated after tissue culture was obtained. Within the first week of treatment, the skin lesions started to resolve. Histoplasma capsulatum was later isolated by culture, confirming the diagnosis. The patient was continued on amphotericin B for a total of 10 weeks, and was started on lamivudine, stavudine, and nelfinavir for her HIV infection during hospitalization. After amphotericin B therapy, the patient was placed on life-long suppressive therapy with itraconazole. Follow-up at 9 months after the initial presentation revealed no evidence of relapse of histoplasmosis.

Adult↗
PubMed1984

Hydralazine-induced systemic lupus erythematosus presenting as pyoderma gangrenosum-like ulcers.

A 48-year-old white woman who for 3 years had been taking hydralazine, 100 mg three times a day, propranolol, 160 mg twice a day, and chlorothiazide, 500 mg/day, for hypertension suddenly developed rapidly expanding ulcers that looked like pyoderma gangrenosum. Arthralgias, fevers, and occasional shortness of breath were also noted. A pericardial effusion was diagnosed by echocardiography. The antinuclear antibody (ANA) titer on routine mouse liver substrate was initially negative, but the ANA titer was positive (1:1,920) on human epithelioid cell substrate. Antibodies to histones and single-stranded DNA were also elevated. After discontinuing hydralazine, all signs and symptoms cleared over a 4-week period. At the time of discharge the ANA titer had decreased to 1:480.

Antibodies, Antinuclear↗
PubMed2006

[Differential diagnosis of "sterile" phlegmonous hand infections].

INTRODUCTION: Bacterial infections represent a large proportion of emergencies in hand surgery. In some cases, pyoderma gangrenosum and mycobacterial infection may present with the same symptoms of swelling, pain, and purulent secretion. In these cases, operative treatment would be harmful. Therefore two cases-pyoderma gangrenosum and tuberculosis-are presented here in relation to common bacterial hand infection. METHODS: Using two case reports of diseases that only rarely affect the hands, their relevance to differential diagnosis is shown with reference to the literature. RESULTS: In both cases, we found clinical symptoms of bacterial hand infection with negative bacterial smear tests. After several debridements, pyoderma gangrenosum of the dorsum of the hand was diagnosed in one patient after pyodermiform lesions at the thigh and the nasal septum were detected and pre-existing colitis ulcerosa was taken into consideration. Corticoid therapy induced complete remission. The second patient with similar clinical symptoms had been operated on at another hospital several times before being transferred to our institution. The presumptive diagnosis of pyoderma gangrenosum was made, and under treatment with prednisone the symptoms quickly improved. After 2 weeks, the wound conditions and the patient's condition rapidly worsened. Following amputation at the upper arm level, the patient died of septic multiple organ failure. Autopsy studies revealed tuberculous sepsis originating from the hand. DISCUSSION: Patient history should be evaluated carefully because of its value to correct diagnosis. In case of negative smear tests, especially from immunocompromised, elderly patients and in patients with a history of pulmonary tuberculosis, Ziehl-Neelsen staining should be obtained. In case of multilocular affection or pre-existing chronic inflammatory bowel disease, the presumptive diagnosis of pyoderma gangrenosum can be confirmed by biopsies from the lesions margin. In both cases, unnecessary traumatizing operations could thus be avoided and treatment optimized.

Adult↗
PubMed1996

Acne fulminans coexisting with pyoderma gangrenosum-like eruptions and posterior scleritis.

A 30-year-old Japanese man presented with an erythematous, papulonodular eruption on his face and upper trunk, which rapidly progressed into severely exuberant, ulcerative lesions with a confluent tendency. His other symptoms included a high fever and general malaise. Complete blood cell counts showed neutrophilia with increased percentages of myelocytes and metayelocytes. The systemic administration of corticosteroids improved his skin lesions, leaving scars and milia. During tapering of corticosteroids, however, the patient developed posterior scleritis of his eyes and pyoderma gangrenosum-like eruptions on the lower legs. Our patient's history suggests that pyoderma gangrenosum-like eruptions and posterior scleritis are disorders associated with acne fulminans.

Acne Vulgaris↗
PubMed1992

Pyoderma gangrenosum-like ulcer in a patient with large granular lymphocytic leukemia.

Large granular lymphocytic leukemia refers to a clonal expansion of lymphocytes that have abundant cytoplasm and azurophilic granules. The disease is characterized clinically by chronic neutropenia and it may be associated with recurrent pyogenic infections. Except for these infections, cutaneous manifestations of this disease have not been well characterized. We describe a patient with large granular lymphocytic leukemia, which was confirmed by molecular genetics studies, who had a pyoderma gangrenosum-like ulcer on his leg. Results of an evaluation of the histologic characteristics and the leukocytic immunophenotype of a skin biopsy specimen from the ulcer demonstrated large granular lymphocytes within the blood vessels. Cutaneous ulceration may be a manifestation of large granular lymphocytic leukemia, and this disease should be considered when diagnosing patients with otherwise unexplained pyoderma gangrenosum-like ulcers of the skin.

Complement C3↗
PubMed1998

A continuum of neutrophilic disease occurring in a patient with ulcerative colitis.

A case of Sweet's syndrome (acute febrile neutrophilic dermatosis) occurring concurrently with bullous pyoderma gangrenosum is reported to emphasize the close relationship between these two disorders. Atypical pyoderma gangrenosum and Sweet's syndrome have been described as occurring simultaneously in haematological dyscrasias but not, to our knowledge, in ulcerative colitis. It has been proposed that pyoderma gangrenosum, Sweet's syndrome, erythema elevatum diutinum and subcorneal pustular dermatosis may represent manifestations along a continuum of neutrophilic dermatoses.

Biopsy, Needle↗