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Pyoderma vegetans and ulcerative colitis.

Pyoderma vegetans (PV) is a chronic, vegetating pustular disorder characterized clinically by erythematous vesiculopustular vegetating cutaneous plaques. Marked epidermal hyperplasia, intraepidermal and subepidermal neutrophilic microabscesses and a dermal inflammatory infiltrate are the prominent histopathological findings. We describe a patient with PV associated with ulcerative colitis and mammary Paget's disease. Pustular eruptions associated with ulcerative colitis are reviewed.

Breast Neoplasms↗

Vegetative pyoderma gangrenosum in chronic renal failure.

We report the case of a 58-year-old woman with painful recurrent erythematous lesions on both legs of 6 months' duration. The patient had been treated with haemodialysis for chronic renal failure for the previous 10 years. Physical examination revealed pea-sized erythematous nodules with sinus formation and discharge of bloody pus, which yielded neither bacterial nor fungal cells in culture. These lesions healed leaving cribriform scars, after bed rest, topical disinfection and systemic and in some areas intralesional corticosteroids. Cases of pyoderma gangrenosum are usually classified as one of four clinical variants: ulcerative, pustular, bullous and vegetative. Our case might correspond to a vegetative form in view of the development of sinuses, neutrophilic abscesses and palisading granuloma, even though no ulceration was observed.

Anti-Inflammatory Agents↗

Pyoderma gangrenosum complicating Cogan's syndrome.

Cogan's syndrome is a rare clinical entity defined by the association of a nonsyphilitic interstitial keratitis and vestibuloauditory dysfunction, typically Menière's disease-like; the condition has been reported in association with a variety of cutaneous diseases. We now report a case of pyoderma gangrenosum complicating Cogan's syndrome in a 57-year-old woman, which then healed dramatically, as more interestingly did the associated uveitis with minocycline therapy.

Ataxia↗

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↗

Pyoderma gangrenosum of the eyelid and acute rhinosinusitis.

Pyoderma gangrenosum (PG) is a destructive, necrotizing, noninfective ulceration of the skin. Periorbital PG is extremely rare, and may progress, with eventual loss of the eye. We report a 47-year-old woman who presented with a right periorbital swelling of 8 days duration and concurrent acute rhinosinusitis. A clinical diagnosis of right periorbital PG was made, and treatment instituted with intravenous methylprednisolone and antibiotics, resulting in rapid resolution of the condition. The clinical features in this case, together with the past history of histologically confirmed PG, enabled a rapid diagnosis to be made, with early administration of treatment resulting in minimal scarring. The clinical picture could easily be confused with periorbital cellulitis, and this case demonstrates the importance of considering the differential diagnoses of periorbital swelling.

Acute Disease↗

Pustular pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is an idiopathic inflammatory disease of unknown aetiology, frequently associated with an underlying systemic condition such as inflammatory bowel disease or haematological malignancy. Its occurrence tends to parallel exacerbations of the underlying disease. Four clinical variants of PG have been described and these include ulcerative, pustular, bullous and vegetative types. We report two cases of the pustular form, which is an uncommon variant of PG, where the pustules do not progress to form ulcers. Both our patients suffered with inflammatory bowel disease which remained quiescent as the pustular PG developed.

Adrenal Cortex Hormones↗

Antimicrobial activity of enrofloxacin against Staphylococcus intermedius strains isolated from canine pyodermas.

This study examined and compared the minimal inhibition concentrations (MICs) of enrofloxacin against 393 Staphylococcus intermedius strains isolated in France from canine pyodermas during three different years, 1995 (174 isolates), 1997 (101 isolates) and 1999 (118 isolates). The MICs of enrofloxacin against these strains ranged from 0.063 to 64 mg L-1, with MIC50 and MIC90 equal to 0.125 and 0.25 mg L-1, respectively. Two resistant strains were found, but only among isolates collected in 1999. The data show that resistance to enrofloxacin among S. intermedius strains is still rare in dogs, but the selection in vitro of variants in which the MICs were increased 4-16-fold after 10 serial passages in subinhibitory concentrations of enrofloxacin suggests that inappropriate use might favour the development of resistant strains in vivo.

Animals↗

Atypical hemorrhagic bullous pyoderma gangrenosum.

A 55-year-old woman was seen in the Plastic and Reconstructive Surgery Clinic because of a nonhealing wound on her left leg of approximately 2 months' duration. She had an 8-month history of multiple wounds appearing on her arms and legs. The patient noted that the majority of the wounds had been treated and healed with the use of topical medications. One wound on her left leg had continued to enlarge rapidly despite topical treatments. Therefore, hyperbaric oxygen therapy had been administered for 15 sessions. No additional healing had occurred with this treatment. Skin grafting was performed on the affected area. She developed ulcers and blistering lesions at surgical and nonsurgical sites after skin grafting. The patient was referred to the Dermatology Department. Dermatologic examination revealed a deep, necrotic ulcer, 30 cm x 10 cm, with surrounding violaceous erythema on the donor area, ulceration (18 cm x 8 cm) on the graft area, a hemorrhagic bullous plaque (5 cm x 15 cm) over the right malleolus, scattered ecchymotic lesions and small hemorrhagic bullae on both legs, and small pustules around the staplers (Fig. 1a,b). Cutaneous biopsy of a new lesion revealed a focal, dense neutrophilic infiltrate, liquefaction degeneration in the center, lymphocytic and mild plasmacytic infiltration around the venules, and fibrinoid deposits in the walls and lumen of the vessels (Fig. 2a). In addition, excessive polymorphonuclear leukocytes and extravasated erythrocytes were present in the papillary and reticular dermis (Fig. 2b). The patient had a 14-year history of asthma bronchiale. Physical examination did not reveal any abnormality, except for crackling rales at the base of each lung. Laboratory examinations were within normal limits, except for the sedimentation rate (55 mm/h). Protein electrophoresis, peripheral blood smear, abdominal ultrasound, and thorax and abdominopelvic computed tomography scans were all normal. Swab cultures from the ulcers were negative. Bullous pyoderma gangrenosum was diagnosed on clinical and histopathologic grounds. Prednisolone 80 mg/day was started. Rapid epithelialization was observed within 2 months of treatment. The dose of prednisolone was gradually decreased to 20 mg/day, and was used as a maintenance dose for an additional 6 months. Complete improvement was achieved in 8 months. The patient has been followed up for approximately 1 year. There were no side effects observed during the treatment and in addition no new lesions developed at the follow-up.

Anti-Inflammatory Agents↗

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↗

Pyoderma gangrenosum: a report of 21 cases.

BACKGROUND: Pyoderma gangrenosum (PG) is an uncommon, destructive, cutaneous ulceration, belonging to the neutrophilic disease spectrum. It is associated with systemic disease in 50% of cases. METHODS: We report a retrospective study of 21 cases of PG. All cases studied fulfilled the following criteria: (i) clinical features of PG; (ii) histopathology consistent with a diagnosis of PG, and excluding other specific dermatoses. RESULTS: The average age of our patients was 41.8 years. The male to female ratio was 1.1. The typical ulcerative variant was found in 17 patients, bullous PG in two patients, and the granulomatous variant in two patients. Sixty-two per cent of our patients had lesions on their lower legs. Two patients had neutrophilic pulmonary involvement concurrent with the ulcers. An association with other internal diseases was noted in 12 patients. Histopathologic study showed vasculitis in 13 patients. Of these, 11 were leukocytoclastic and the others predominantly lymphocytic. CONCLUSIONS: PG is a rare disease, with the ulcerative variant being most frequent. The lower legs are the most commonly affected sites. The recurrence rate in our study was about 46% regardless of the treatment prescribed. Pulmonary involvement was fatal in two patients.

Adolescent↗

Case reports and a review of the literature on ulcers mimicking pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is a rare, ulcerative skin disease. Diseases with cutaneous manifestations resembling PG may lead to the misdiagnosis of PG. We discuss two patients presenting with cutaneous ulcers originally thought to be PG, and review the literature on the clinical and pathologic characteristics of PG ulcers compared to PG-like ulcers in order to determine possible distinguishing features.

Adolescent↗

Cutaneous Wegener's granulomatosis (malignant pyoderma) in a patient with Crohn's disease.

We report a case of an unusual presentation of Wegener's granulomatosis (WG) in a patient with Crohn's disease (CD). She presented to our Wound Care Center with 7th cranial nerve palsy and facial pyoderma-like ulcerations. Although WG has a predilection for the lung, kidney, and eyes, cutaneous involvement can be seen in 50% of the cases, and it can be the presenting sign in 9-14%. Because of the lethality of WG if not properly treated, the diagnosis is imperative.

Aged↗

Wegener's granulomatosis presenting as pyoderma gangrenosum.

A 59-year-old male patient developed a necrotizing ulceration on the right shin. Both clinical and histopathologic examinations suggested pyoderma gangrenosum. After temporary improvement of skin symptoms under peroral glucocorticoid treatment, a hemorrhagic-purulent discharge started from the nose, he began to have fever, malaise, cough, and a chest X-ray revealed inflammation in the lung. Cerebral CT and MRI disclosed midline bone loss within the nasal septum and granulomatosus tissue masses protruding into the right orbit. The c-ANCA test was positive, serum IgA was elevated, and he had microhaematuria and proteinuria. In this severe case of Wegener's granulomatosis prolonged methylprednisone and cyclophosphamide treatment was initiated. Both the skin symptoms and the granulomatosus infiltrations resolved.

Granulomatosis with Polyangiitis↗

Pyoderma gangrenosum associated with sclerosing cholangitis, type 1 diabetes mellitus and ulcerative colitis.

We describe the case of a 22-year-old black female with type 1 diabetes mellitus diagnosed when she was 12 years old. She first presented (March 1994) with pustules and ulcerations on the upper and lower limbs, trunk and scalp at the age 17. The diagnosis of pyoderma gangrenosum was made. Since presentation, changes in liver function were detected and subsequent study led to the diagnosis of sclerosing cholangitis. The diagnosis of ulcerative colitis was made after colonoscopy. Partial response was obtained with minocycline and clofazimine, but treatment with 5-aminosalicylic acid achieved no improvement of the ulcerations. Liver transplantation, followed by immunosuppressive therapy led to complete regression of the cutaneous lesions.

Adult↗

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↗

Aberrant integrin (CR4; alpha(x)beta2; CD11c/CD18) oscillations on neutrophils in a mild form of pyoderma gangrenosum.

We have previously shown that the beta2 integrins CR3 and CR4 physically and functionally interact with urokinase receptors (uPAR) on neutrophil plasma membranes in an oscillatory fashion. In this study we have analyzed neutrophils from patient SC, a 34 y old African American female, with aberrant skin window results and recurrent perianal abscesses and pretibial lesions diagnosed as pyoderma gangrenosum. Although untreated migrating normal neutrophils exhibited 20 s sinusoidal oscillations in CR4-uPAR proximity, neutrophils from SC demonstrated a faster oscillation (10 s) in the form of a flyback sawtooth wave. This waveform mimicked that observed for normal neutrophils treated with subsaturating doses of the kinase inhibitors staurosporine, genistein, and erbstatin. As beta2 integrins are regulated by phosphorylation, we tested the hypothesis that the aberrant CR4-uPAR proximity oscillations seen in SC's neutrophils are due to defective kinase activity that might be balanced by a decrease in phosphatase activity. When SC's cells are exposed to subsaturating concentrations of the phosphatase inhibitor pervanadate, this caused the CR4-uPAR oscillations to become sinusoidal in shape with a 20 s period, as seen in normal migrating neutrophils. Although SC's neutrophils were deficient in spontaneous and N-formyl-methionyl-leucyl-phenylalanine-induced polarization, 0.5 microM pervanadate returned cell polarization to nearly normal levels, thus paralleling the acquisition of normal receptor interactions. Inasmuch as SC's cellular phenotype is mimicked by kinase inhibitors and corrected by phosphatase inhibitors, we suggest that a mutation(s) affecting the kinetics of intracellular signaling enzymes, but not blocking the pathway per se, may be responsible for this clinical state.

Adult↗

Pyoderma gangrenosum in two siblings: a familial predisposition.

We report pyoderma gangrenosum in two siblings with onset during childhood and no associated systemic abnormalities. The patients were born of nonconsanguineous, healthy parents. Treatment with oral corticosteroids produced an excellent clinical response, followed by recurrence after cessation of therapy. Steroids were restarted in combination with dapsone to prevent further recurrence.

Administration, Oral↗