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A case of propylthiouracil-induced pyoderma gangrenosum associated with antineutrophil cytoplasmic antibody.

A 27-year-old woman who had been receiving propylthiouracil for 2 years for Graves' disease presented with painful ulceration on the lower limbs which had first appeared 2 weeks previously. Well-circumscribed hemorrhagic ulcerations with ragged borders were noted on both legs. Skin biopsy demonstrated a florid neutrophilic infiltrate and evidence of leukocytoclasis around small blood vessels in the papillary dermis compatible with the diagnosis of pyoderma gangrenosum. A highly positive perinuclear pattern of antineutrophil cytoplasmic antibody with specificities for IgM myeloperoxidase was observed. The authors think that propylthiouracil is associated with the occurrence of pyoderma gangrenosum in this patient.

Adult↗

Infantile Crohn disease presenting with diarrhea and pyoderma gangrenosum.

Cutaneous erosions and ulcerations in the diaper area are common in infancy and usually result from local irritation. We describe an infant with chronic diarrhea and failure to thrive who developed extensive ulcerations in the inguinal folds and perineum that were initially thought to be exclusively caused by local irritation. A cutaneous examination found signs consistent with those of pyoderma gangrenosum, leading to a diagnosis of infantile Crohn disease. Cutaneous signs can lead to the diagnosis of an underlying systemic disease in infants with chronic diarrhea and rash. Prompt diagnosis is especially important in infantile Crohn disease, since many infants require surgical resection of affected bowel, and 60% die from disease complications. This article reports a rare instance of an infant who developed pyoderma gangrenosum due to Crohn disease and reviews cutaneous signs of systemic disease in infants presenting with chronic diarrhea and rash.

Biopsy, Needle↗

Pyoderma gangrenosum in association with undifferentiated seronegative spondylarthropathy.

The cases of 2 women with pyoderma gangrenosum (PG) and undifferentiated seronegative spondylarthropathy (SpA) are described. These 2 cases, together with the recently reported case of PG and B27-positive psoriatic spondylarthropathy, suggest that PG may also occur in association with forms of seronegative SpA that are different from primary ankylosing spondylitis (AS) and AS associated with inflammatory bowel disease.

Adult↗

Recalcitrant oral pyoderma gangrenosum in a child responsive to cyclosporine.

We report a case of recalcitrant pyoderma gangrenosum which developed on the tongue of an 8-year-old, previously healthy boy without any systemic associated disease. The lesion was confined to the tongue and was not responsive to conventional treatment with prednisolone and sulfa drug, but it showed dramatic improvement with oral cyclosporine therapy. In addition, the histopathology of the lesion included many eosinophils, which caused difficulties in diagnosis to differentiate this condition from other oral ulcerative diseases showing tissue eosinophilia including eosinophilic ulcer of the tongue.

Child↗

Granulomatous pyoderma gangrenosum: two unusual cases showing necrotizing granulomatous inflammation.

We present two cases of pyoderma gangrenosum (PG) with unusual histopathological findings. The main histopathological feature of PG is usually massive neutrophilic infiltration; the neutrophil is thus the cytologic hallmark of PG. The occurrence of vasculitis is controversial. In our patients, in contrast, biopsy specimens revealed extensive granulomatous inflammation with massive tissue necrosis throughout the entire dermis and subcutaneous tissue and vascular involvement simulating many other granulomatous diseases. However, there was no evidence of systemic disease. Our cases may therefore represent a histopathologically distinct subset of PG.

Adult↗

Cephalic pyoderma gangrenosum (PG)-like lesions as a presenting sign of Wegener's granulomatosis.

BACKGROUND: Pyoderma gangrenosum (PG)-like lesions can be associated with Wegener's granulomatosis (WG); in rare instances, they could be the presenting sign of WG. The purpose of this study was to elucidate the relationship between these two disorders. METHODS: The clinical and histologic data from a case of WG with primary ulcerative lesions of the face were compared with five other cases previously reported in the literature. RESULTS: A predominance among young men was noted. In three patients the histology of the skin ulcerations was suggestive of WG, in the remaining three, including the case described, it was nonspecific. Two cases developed pulmonary WG after the skin lesions cleared, showing that the extent and severity of skin lesions do not necessarily correlate with the systemic disease. CONCLUSIONS: When the histology of the skin is nonspecific, the diagnosis of PG or PG-like lesions of the head and neck should not be considered definitive and the patients should be carefully monitored to rule out the possible presence of WG.

Adolescent↗

Improvement of Pyoderma gangrenosum and psoriasis associated with Crohn disease with anti-tumor necrosis factor alpha monoclonal antibody.

BACKGROUND: Infliximab is an anti-tumor necrosis factor alpha monoclonal antibody IgG effective in the treatment and maintenance of remission of active refractory Crohn disease and associated draining enterocutaneous fistulae. Multiple infusions of infliximab show promising results in patients with rheumatoid arthritis. Currently, there is limited clinical experience with infliximab, and no published reports exist on its use in cutaneous disorders. OBSERVATIONS: We describe 2 patients with Crohn disease and pyoderma gangrenosum and 1 patient with Crohn disease and psoriasis who were treated with infliximab for recalcitrant Crohn fistulae, with concurrent improvement in their skin diseases. CONCLUSIONS: These cases suggest that infliximab, a promising therapeutic agent for refractory Crohn disease and fistulae, may also be effective in the treatment of pyoderma gangrenosum and psoriasis associated with Crohn disease.

Adult↗

[Cutaneous manifestations of internal diseases: purpura, livedo, pyoderma gangrenosum].

Dermatology and internal medicine have in common that many systemic diseases manifest with skin symptoms that are easily accessible for both diagnostic and investigative procedures. Even if modern practice of medicine tends towards organ specific particularity of specialists, dermatology traditionally strives for interdisciplinary communication. Cutaneous manifestations of internal diseases present in various forms. In a syndrome, a number of symptoms present in an identical pattern, and pattern recognition is diagnostic. Vice versa, a distinct skin sign can be a clue to various internal disorders, that have to be considered in the differential diagnosis. Purpura and livedo due to either inflammatory (immune complex mediated or associated with ANCA) or vasoocclusive microthrombotic vascular disease are presented as examples. Finally, subtile variations in key symptoms of the skin, such as of pyoderma gangrenosum, may give a clue to a specific underlying disease, such as rheumatoid arthritis, inflammatory bowel disease, monoclonal gammopathy, or hematologic disease, as exemplified by the variants of pyoderma gangrenosum.

Adult↗

[Pyoderma gangrenosum, subcorneal IgA pustulosis and recurrent neutrophilic pleural and pulmonary diseases in a patient with IgA gammopathy].

INTRODUCTION: The term "neutrophilic dermatosis" is normally used to cover a number of dermatoses characterized by their response to corticotherapy or to drugs effective on the neutrophilic polynuclear. Neutrophilic dermatosis was frequently associated with hematologic disease. We study the case of a neutrophilic dermatosis accompanied by a pulmonary involvement and then review the literature on the reported cases of neutrophilic dermatosis with extra-cutaneous localisations. CASE STUDY: A woman aged 54 displayed a pyoderma gangrenosum successively followed by a pustular dermatosis (related to subcorneal pustular dermatosis of Sneddon-Wilkinson) and by a pulmonary involvement not caused by any underlying infection. The laboratory results revealed a benign IgA gammaglobulin. The use of corticotherapy and a relay by dapsone led to the gradual disappearance of the cutaneous and pulmonary lesions. DISCUSSION: Pulmonary involvement have been observed in the Sweet syndrome and in the pyoderma gangrenosum. The neutrophilic lung is diagnosed once a tumorous or infectious cause has been eliminated and on the basis of the response to the cortisone treatment. CONCLUSION: The link between pulmonary involvement and neutrophilic dermatosis is sometimes mentioned but is in fact often difficult to confirm. The positive reaction to corticosteroids could be a diagnostic test.

Aged↗

Pyoderma gangrenosum: successful treatment with intralesional steroids.

A case of pyoderma gangrenosum (PG) responsive to intralesional injection of corticosteroid is reported. Improvement was detected by 48 hours, and complete healing had occurred by 6 weeks. The association with various underlying systemic diseases, pathogenesis, and modalities of therapy are discussed.

Humans↗

Pyoderma gangrenosum in Papua New Guinea.

A severe case of pyoderma gangrenosum in a Papua New Guinean girl aged 11 years in reported. Multiple lesions of the arms, legs, buttocks and mouth were present. There was no associated disease. She was treated with very high doses of prednisolone and slow resolution over 3 months in hospital was observed.

Acute Disease↗

Twenty cases of peristomal pyoderma gangrenosum: diagnostic implications and management.

HYPOTHESIS: Our experience with peristomal ulcers suggested that peristomal pyoderma gangrenosum (PPG) is an infrequent and usually unrecognized complication of inflammatory bowel disease. We hypothesized that a review of our experience with PPG would clarify the essentials of its diagnosis, evaluation, and treatment. DESIGN: A case series of 20 consecutive patients with PPG complicating inflammatory bowel disease were treated at our institution between 1986 and 1999. There were 15 women and 5 men. At the time of development of peristomal pyoderma, 10 of 20 patients had a diagnosis of Crohn disease (CD), while 9 had a diagnosis of ulcerative colitis (UC). One patient was diagnosed as having CD only after first developing PPG. MAIN OUTCOME MEASURE: Healing of PPG. INTERVENTIONS: All patients had failed local enterostomal care prior to referral. Debridements and/or stomal revisions were uniformly unsuccessful. Biopsies, when performed, did not provide clinically important information. Treatment was directed toward inflammatory bowel disease, with variable clinical responses to corticosteroids, metronidazole, cyclosporine, sulfasalazine, and infliximab. RESULTS: Ultimately, 13 patients had a diagnosis of CD. Of these patients, 12 (92%) of 13 developed PPG coincident with recurrent disease. Two patients had a remote history of proctocolectomy for UC and subsequent evaluation revealed CD. One patient developed PPG adjacent to a urinary Kock pouch after cystectomy; ultimately, a diagnosis of CD was made. No patients were lost to follow-up, but in 1 case of UC, no evaluation for latent CD was carried out. The final diagnosis was CD disease in 13 (65%) of 20 and UC in 7 (35%) of 20 patients. All PPG ulcers healed completely, within an average of 11.4 months (median, 8 months; range, 1-41 months). Ulcer resolution was achieved with medical therapy alone in 14 (70%) of 20 cases. Resection of active gastrointestinal CD resulted in healing in 5 (83%) of 6 cases. One case healed 2 months after conservative therapy only. CONCLUSIONS: This review of the largest reported series of PPG suggests the following: (1) PPG complicating inflammatory bowel disease is uncommon and often misdiagnosed by clinicians; (2) local wound care measures have little role in the healing of PPG; (3) PPG usually heralds active CD; (4) in patients with prior history of UC, PPG indicates CD until proven otherwise; (5) prolonged medical therapy (11 months), usually with immunosupression, is required for healing of PPG; and (6) if feasible, surgical resection of all active CD leads to the healing of PPG ulcers.

Adult↗

[Low dosage cyclosporin A therapy in pyoderma gangrenosum. Experiences with 6 patients].

Pyoderma gangraenosum can cause great therapeutic problems. High dosed corticosteroids are the treatment of choice. However, recalcitrant pyoderma gangraenosum or side effects from corticosteroid treatment may require therapeutic alternatives. Pyoderma gangraenosum responds excellently to treatment with cyclosporine A. Because of side effects and drug interactions, the patients must be selected and carefully and closely monitored. Six patients with pyoderma gangraenosum, unresponsive to various topical and systemic therapies, were treated with oral cyclosporine A at mean daily doses of approximately 3 mg/kg. Marked improvement of the skin lesions and complete healing occurred in all patients over a period of 3-6 months. Only one patient suffered a relapse after discontinuation of the treatment. No severe irreversible side effects occurred. The results show that low-dose cyclosporine A treatment can be considered a first-line treatment of pyoderma gangraenosum.

Adult↗

The association of pyoderma gangrenosum with ulcerative colitis in Japan.

A patient with pyoderma gangrenosum (PG) and ulcerative colitis (UC) is described. He had melena and systemic skin lesions. The skin lesions consisted of small discrete ulcers on the back and head and large punched-out ulcers on the legs. He was successfully treated with prednisolone and salazosulfapyridine. He became asymptomatic after two weeks' treatment. Although the association of PG with UC is well documented among Caucasians, it is very rare among Japanese.

Adult↗

Cutaneous cryptococcosis simulating pyoderma gangrenosum.

A 33-year-old white man with a history of chronic ulcerative colitis presented with multiple cutaneous ulcers and an indurated cellulitic area on his right thigh. Clinically, the ulcers were considered to represent pyoderma gangrenosum. However, tissue biopsy revealed copious yeast forms that were identified as Cryptococcus neoformans. The indurated area on the right thigh later ulcerated, and tissue culture of this area also revealed Cryptococcus. This case illustrates that when pyoderma gangrenosum is diagnosed, the possibility of deep fungal infection should be considered and excluded by appropriate studies.

Adult↗