Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria

Pyoderma gangrenosum is a rare but significant cause of ulcerations. It is a diagnosis of exclusion. Herein, we suggest diagnostic criteria and some historical perspectives on the diagnosis of pyoderma gangrenosum.     

Pyoderma Gangrenosum

A 67-year-old man presented in April 1983 with three painful ulcers with bluish margins on the right shin and the lower back. The largest lesion was 6 cm in diameter. The lesions had started as painful nodules 8 months previously; these ulcerated within 2 weeks and fluctuated in size. There was no history of arthritis or bowel disease. Full blood count, erythrocyte sedimentation rate, serum electrolytes, urea, and liver enzymes were normal. Antinuclear antibody and rheumatoid factor were negative. The serum IgA level was elevated at 7.35 g/L (normal 0.8–4 g/L). Immunoelec-trophoresis revealed IgA kappa paraproteinemia. Bence Jones protein was not found in the urine. Skeletal survey, bone marrow, sigmoidoscopy, and rectal biopsy were normal. No bacterial pathogens were grown. Histology of the edge of the ulcer showed dermal abscesses, dermal granulation tissue, and chronic inflammatory infiltrate extending to the subcutis. During the next 2 years the patient developed active pyodermatous lesions on the trunk and left arm. Between April 1983 and March 1986, the patient received a combination of prednisolone 15–40 mgand azathioprine 50–100 mg daily, a 12-week course of dapsone 200 mg daily, and repeated courses of oral and topical antibiotics. During this period, although the disease activity was lessened, the lesions never healed completely. In April 1986, the pyoderma gangrenosum was more active; the ulcers enlarged and deyeloped active pustules at the margins. While the patient was still taking prednisolone 15 mg and azathioprine 50 mg, oral cyclosporin A 6 mg/kg daily (250 mg twice daily) was started; after 3 weeks, the dosage was increased to 10 mg/kg daily (400 mg twice daily) and the prednisolone was reduced to 5 mg daily over 2 months. At 3 weeks the ulcers started to heal, and at seven weeks all ulcers healed, leaving papery scars. Over a period of 4 weeks, the dosage of cyclosporin A was gradually reduced and the ulcers remained healed at a maintenance dosage of 250 mg daily. There was no change in the paraproteinemia. Weekly whole blood cyclosporin A levels were measured, and they fluctuated between 800 ng/ml and 910 ng/ml. On two occasions the levels were >1500 ng/ml. During the course of the treatment, the patient developed nausea, hypertrichosis, transient thrombocytopenia, and hypertension (blood pressure of 190/110 mmHg); creatinine clearance fell from 87 to 46 ml/min. At a maintenance dosage of 250 mg daily, the blood pressure was 180/100 mmHg and creatinine clearance was 64 ml/min.     

Gigantic Pyoderma Gangrenosum

A 71-year-old Japanese female with gigantic pyoderma gangrenosum is reported. The pyoderma lesions had been treated as an infectious condition for seventeen months and had extended to enormously large areas. The nature of the chronic type of pyoderma gangrenosum may need to be stressed, even for dermatologists.     

Pyoderma Gangrenosum in childhood

Pyoderma Gangrenosum is a rare, ulcerative, necrotizing cutaneous disorder of unknown etiology. ¹ The lesions usually present as a painful nodule or pustule that breaks down to form a progressively enlarging ulcer with a raised tender, undermined edge. ¹ Pyoderma Gangrenosum was first described by Brunsting et al. in 1930, ² but the pathogenesis is still not clear. Local infection does not appear to be an etiologic factor even though Brunsting initially suggested this may be the cause. ^1–3 Its association with various autoimmune diseases and its response to immunosuppressive therapy suggests an immunologic basis for the disease. ^4,5 The characteristic feature of pyoderma gangrenosum is the development of lesions at sites of trauma: also known as pathergy phenomenon. Most cases of pyoderma gangrenosum occur mainly between the third and fifth decades of life, though disease can occur anytime between the first and ninth decades of life. ⁶ We present a case of pyoderma gangrenosum in a 3-year-old boy, and review the features of pyoderma gangrenosum in children.     

Leukocyte Chemotaxis and Pyoderma Gangrenosum

Leukocyte chemotaxis (in five patients with pyoderma gangrenosum) was studied using a modification of the Boyden chamber method. In all patients the chemotactic response was significantly lower than in the controls. This abnormal chemotaxis was a result of an intrinsic neutrophil dysfunction. No significant difference was detected between the chemotactic response of leukocytes from patients with minimal or no skin involvement and those from patients with extensive lesions.     

Clinical Management of Pyoderma Gangrenosum

Pyoderma gangrenosum is a noninfectious neutrophilic dermatosis that usually starts with sterile pustules which rapidly progress to painful ulcers of variable depth and size with undermined violaceous borders. In 17 to 74% of cases, pyoderma gangrenosum is associated with an underlying disease, most commonly inflammatory bowel disease, rheumatological or hematological disease or malignancy. Diagnosis of pyoderma gangrenosum is based on a history of an underlying disease, typical clinical presentation and histopathology, and exclusion of other diseases that would lead to a similar appearance. Randomized, double-blinded prospective multicenter trials investigating the treatment of pyoderma gangrenosum are not available. The treatments with the best clinical evidence are systemic corticosteroids (in the initial phase usually 100 to 200 mg/day) and cyclosporine (mainly as a maintenance treatment). Combinations of corticosteroids with cytotoxic drugs such as azathioprine, cyclophosphamide or chlorambucil are used in patients with disease that is resistant to corticosteroids. The combination of corticosteroids with sulfa drugs, such as dapsone, or clofazimine, minocycline and thalidomide, has been used as a corticosteroid-sparing alternative. Limited experience has been documented with methotrexate, colchicine, nicotine, and mycophenolate mofetil, among other drugs. Alternative treatments include local application of granulocyte-macrophage colony-stimulating factor, intravenous immunoglobulins and plasmapheresis. Skin transplants (split-skin grafts or autologous keratinocyte grafts) and the application of bioengineered skin is useful in selected cases in conjunction with immunosuppression. Topical therapy with modern wound dressings is useful to minimize pain and the high risk of secondary infection. The application of topical antibacterials cannot be recommended because of their potential to sensitize and their questionable efficacy, but systemic antibacterial therapy is mandatory when infection is present. Despite recent advances in therapy, the prognosis of pyoderma gangrenosum remains unpredictable.     

Pyoderma Gangrenosum in Childhood Leukemia

A case of pyoderma gangrenosum (PG) in a 14-year-old boy with acute myelogenous leukemia (AML) is described. The onset of pyoderma gangrenosum coincided with the relapse of AML. The lesions responded dramatically to treatment with oral prednisone despite the persistence of leukemia. Pyoderma gangrenosum should be included in the differential diagnosis of any nodular, pustular, or necrotic cutaneous eruption in children with leukemia.     

Pyoderma Gangrenosum on the Finger

A case of pyoderma gangrenosum on the dorsal aspect of the right second and third fingers of a 50-year-old woman is reported. The patient had been wounded at the above-mentioned site by a cutter. This wound developed into a painful ulcer which enlarged rapidly. No bacterial infections were detected. Histologically, an inflammatory infiltrate of lymphocytes mixed with neutrophils and nucleodusts was seen throughout the entire dermis, but there was no evidence of vasculitis. Based on this data, the case was diagnosed as pyoderma gangrenosum. The patient responded to treatment with systemic corticosteroids. Pyoderma gangrenosum at this site of the fingers seems to be rare.     

坏疽性脓皮病1例

报告1例坏疽性脓皮病。患者男,49岁,全身多发结节、溃疡伴疼痛反复发作2年,加重3月。溃疡可自行愈合,形成菲薄的萎缩性瘢痕。组织病理检查符合坏疽性脓皮病表现。给予糖皮质激素及免疫调节剂获良效。     

Pyoderma Gangrenosum in Infants and Children

Abstract: Pyoderma gangrenosum is an uncommon ulcerative skin disorder that occurs in all age groups. Approximately 4% of patients are infants and children. There are several notable differences between the childhood and adult manifestations of the disease, including the distribution of lesions and associated disorders. We reviewed the childhood cases (≤18 yrs of age) of unequivocal pyoderma gangronosum in the English literature and tabulated the trends in clinical features, associated disorders, and therapy. We report our 3-week-old patient, the youngest documented case. Of the 46 patients, only 4 were less than 1 year of age. A systemic Illness was present in 74% of the older children, most commonly, ulcerative colitis. Only one Infant had an associated problem (HIV+) at the time of onset. Infants appear to have an unusual distribution of perianal and genital lesions not often described in other age groups. Our review suggests that pyoderma gangrenosum in children has a similar clinical appearance to that in adults. It Is associated with some of the same underlying disorders, but with different frequencies. The distribution of lesions in children Is similar, often involving the lower extremities, but pyoderma gangrenosum of the head and face appears to be more common in children. Infants may have ulcers in genital and perianal areas. The most frequently prescribed treatment for children is systemic corticosteroids, which generally are very effective.     

Pyoderma Gangrenosum in Childhood: Case Report

Two cases of pyoderma gangrenosum occurring in children after an exanthem are reported because of rarity and clinical interest.     

Etiology and Management of Pyoderma Gangrenosum

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful, necrotic ulceration. It typically affects patients in the third to sixth decades of life, with almost equal incidence in men and women. PG occurs most frequently on the lower extremities. Five clinical variants are currently recognized: classic, bullous, pustular, vegetative, and peristomal types. Half of PG cases are seen in association with systemic disease. Mimickers include infection, vascular insufficiency ulcers, systemic vasculitides, autoimmune disease, cancer, and exogenous tissue injury, among others. PG is often a diagnosis of exclusion, as there are no specific laboratory or histopathologic findings to confirm the diagnosis. PG thus presents many clinical challenges: it is difficult to diagnose, is frequently misdiagnosed, and often requires a work-up for underlying systemic disease. Successful management of PG typically requires multiple modalities to reduce inflammation and optimize wound healing, in addition to treatment of any underlying diseases. Prednisone and cyclosporine have been mainstays of systemic treatment for PG, although increasing evidence supports the use of biologic therapies, such as tumor necrosis factor-α inhibitors, for refractory cases of PG. Here, we review the clinical presentation and pathophysiology of PG, as well as its associated conditions, diagnostic work-up, and management.     

Pyoderma Gangrenosum with Systemic Involvement

We report a case of a patient with pyoderma gangrenosum associated with multisystemic manifestations. The patient showed liver dysfunction, respiratory failure, and aseptic meningeal reaction. Corticosteroid therapy was efficient in treating both the skin lesions and the other systemic disorders.     

茄病镰刀菌引起坏疽性脓皮病

患者男，５８岁，铁渣灼伤左上睑后，于眼睑部，左颞部及鼻中迅速出现红肿，化脓，坏死，结痂，溃烂，深达皮下及肌层。皮损区直接镜检及组织病理切片均发现大量真菌菌丝，８次真菌学培养为同一菌株ＣＺ９２１３生长。该菌株形态特征，生理测定及动物试验等真菌学研究鉴定证实为茄病镰刀菌。     

Vulvar Pyoderma Gangrenosum in a Child

Abstract:   We present a case of a 10-year-old girl with a diagnosis of vulvar pyoderma gangrenosum. Intravenous methylprednisolone was started and on tapering the steroid regimen, the lesion showed significant enlargement and purulent discharge without any remission of fever and inflammatory activity, so she was treated with oral cyclosporin A in combination with low-dose steroid. A response to treatment was achieved after 2 weeks and clinical and laboratory follow-up at 12 months did not show any disease relapse or inflammation.     

Refractory Pyoderma Gangrenosum in an Infant

We present a rare case of infantile pyoderma gangrenosum with an extended course and limited response to treatment. Despite extensive examination for an underlying disorder, the case remains idiopathic.     

Topical Therapy for Peristomal Pyoderma Gangrenosum

Background Pyoderma gangrenosum (PG) is an uncommon condition. Literature on the management of peristomal PG (PPG) is sparse. In the absence of randomized controlled trials the reporting of case series is potentially helpful to the management of this uncommon disease.Objective In this study we report our experience with topical corticosteroid therapy for 14 consecutive cases of PPG.Methods A clinical diagnosis PPG was made by a trained dermatologist using the appropriate investigations where necessary.Results The majority of the cases presented were managed with simple topical corticosteroids, occasionally in combination with a change of dressing. In 8/14 (57%) cases ulcer resolution was achieved within 3 months with topical treatment alone or topical treatment plus a change of dressing to a silicone-based product applied directly to the wound under the normal base plate of the stoma bag.Conclusion Our experience suggests that a significant proportion of PPG can be managed by topical treatment alone. The simple topical treatment allows the patient to continue use of stoma care products while minimizing the potential for side effects.

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SommaireAntécédents Pyoderma gangrenosum (PG) est une condition rare. Rares sont également les publications spécialisées traitant de la gestion de PG péristomiale (PGP). En I’absence d’essais randomisés contrôlés, I’exposition de séries de cas peut s’avérer utile dans la gestion de cette maladie fortuite.Objectif Dans la présente étude, nous relatons notre expérience avec 14 cas consécutifs de PGP traités par corticoïdes topiques.Méthodes Le diagnostic clinique de PGP a été posé par un dermatologue qualifié, au moyen des investigations appropriées au besoin.Résultats La plupart des cas ont été traités aux corticoïdes topiques, parfois avec changement du pansement. Dans huit des cas (57%), les ulcères ont été guéris dans un intervalle de trois mois, au moyen du traitement topique seul ou en combinaison avec le remplacement du pansement par un produit à base de silicone, appliqué directement sur la blessure, sous la base normale de la poche de stomieConclusion Notre expérience suggére qu’une bonne proportion des PGP peut être gérée par traitement topique seul. Ce traitement permet au patient de continuer à utiliser ses produits de stomie tout en minimisant les effets secondaires.

     

Pediatric Pyoderma Gangrenosum with Splenic and Pulmonary Involvement

An 8‐year‐old boy presented with ulcers on the lip and limbs, scattered pustules, fever, and general malaise. Further investigation revealed splenic and pulmonary lesions. A diagnosis of pyoderma gangrenosum with splenic and pulmonary involvement was made. The authors have not found a previous report of pediatric pyoderma with splenic involvement in the literature.