Pyoderma gangrenosum in an infant: A case report and review of the literature

Pyoderma gangrenosum is a neutrophilic dermatosis that is rare in infancy, with only 20 cases reported in the literature. We present a case of infantile pyoderma gangrenosum refractory to topical steroids, tacrolimus, and dapsone as well as systemic steroids and infliximab that is currently well controlled with the addition of oral tacrolimus. To our knowledge, this is the first report of the effective, safe use of oral tacrolimus in combination with infliximab for infantile pyoderma gangrenosum. We review all current cases of infantile pyoderma gangrenosum, as well as tacrolimus and its role in the treatment of this condition.     

Topical tacrolimus therapy for pyoderma gangrenosum

Pyoderma gangrenosum (PG) is a type of neutrophilic disorder with a chronic clinical course. Immunosuppressive agents have been used for its management. Among them, corticosteroid is known as the most effective. However, other immunosuppressants including cyclosporine A have been selected for patients with PG who were refractory to systemic steroids. Herein we report a case of PG resistant to systemic steroids, who was successfully treated with topical tacrolimus. A fifty-four year-old male had a 14-year history of PG. In 2002, necrotic ulcers appeared on his right leg that were refractory to oral prednisolone (30 mg/day). The application of topical tacrolimus to the border of the ulcers hastened epithelization of the ulcers and allowed for reduction of the oral prednisolone. Topical tacrolimus therapy may be an effective alternative for PG when the lesion is poorly controlled by corticosteroid.     

Pyoderma gangrenosum—response to topical nitrogen mustard

We report a 69-year-old caucasian male patient with long-standing pyoderma gangrenosum; the lesions preceded the appearance of an IgA monoclonal gammopathy by 2 years. A number of systemic treatments, including high dose steroids and immunosuppressive agents, were poorly tolerated and resulted in serious side-effects. The skin and haematological conditions, however, were kept under control for 2 years with regular plasmapheresis. Pyoderma gangrenosum recurred as vascular access became exhausted but new lesions healed completely with topical application of 20% nitrogen mustard.     

Pyoderma gangrenosum on the breast: A case presentation and review of the published work

We present a case of pyoderma gangrenosum localized on the breast, without a preceding surgical intervention and associated systemic disorder. The ulcer had rapidly developed and covered a large portion of the breast. The patient responded well to systemic steroids and salicylazosulfapyridine and the ulcer completely healed with scarring after 3 months of treatment. Pyoderma gangrenosum rarely involves the breasts. A published work survey disclosed only 31 reported cases up to date. In most of these cases the lesions were related to previous surgical interventions, probably as the result of a pathergy phenomenon. The main differential diagnoses were skin and soft tissue infections including necrotizing fasciitis, and malignant neoplasms. Negative initial wound cultures and the relative sparing of nipple/areola complex helped to eliminate these disorders. Though an unusual site for pyoderma gangrenosum, lesions on the breast showed the characteristic clinical features of the disease. The types of associated disorders were also similar to those of the cases with classical pyoderma gangrenosum. As most of the lesions healed with significant scarring, early recognition and treatment of pyoderma gangrenosum located on the breast is important to prevent serious physical and psychological morbidity.     

Topical tacrolimus in dermatology

Tacrolimus and cyclosporin A are potent immunosuppressants that are used systemically to treat several inflammatory skin conditions successfully. They differ in their structure and tacrolimus is 10-100 times more potent than cyclosporin A. They have similar side-effects. They have been used topically in various clinical studies. Topical cyclosporin A is largely ineffective whereas topical tacrolimus is effective in treating atopic dermatitis. Topical tacrolimus has not been studied sufficiently in treating psoriasis although it has been used successfully in allergic contact dermatitis, erosive mucosal lichen planus and pyoderma gangrenosum.     

Genital pyoderma gangrenosum: Report of two cases and published work review of Japanese cases

Pyoderma gangrenosum is an ulcerative skin disorder showing characteristic non‐infectious ulcers and affects the lower extremities in approximately 70% of cases. Pyoderma gangrenosum is commonly associated with systemic diseases such as inflammatory bowel disease, rheumatoid arthritis and hematological malignancies. Herein, we report two cases of Japanese patients diagnosed with genital pyoderma gangrenosum. Case 1 was a 74‐year‐old woman without associated systemic complications, whose skin lesion resembled a squamous cell carcinoma and was limited to the vulva. Case 2 is an 89‐year‐old man, who suffered from myelodysplastic syndrome and acute myeloid leukemia, and presented with penile and leg ulcers mimicking pressure sores. Both cases responded well to systemic steroids. We review 13 genital pyoderma gangrenosum cases (76.9% male; aged 30–89 years) from 1996 to 2012 in Japan, including 11 previously reported cases and the present study's two cases. Four of the 13 genital pyoderma gangrenosum cases had associated systemic diseases and their skin lesions spread to the extragenital areas. Eight of the remaining nine genitalia‐localized pyoderma gangrenosum cases had no associated systemic diseases. In conclusion, genital pyoderma gangrenosum is rare and may be misdiagnosed. It should therefore be considered in cases of refractory genital ulcers. In addition, genitalia‐localized pyoderma gangrenosum tends to be without systemic complications.     

Pyoderma gangrenosum associated with biphenotypic acute leukemia

Pyoderma gangrenosum is a neutrophilic dermatosis that may be associated with myeloid malignancies. Less information is available about the association of pyoderma gangrenosum with lymphoid malignancies. We present, to our knowledge, the first case of pyoderma gangrenosum associated with biphenotypic acute leukemia wherein the malignant cells show a phenotype specific for myelogenic and lymphocytic leukemia. Histopathologic examination revealed rather nonspecific features without involvement of leukemic cells in the skin lesions. Treatment with systemic steroids was followed by characteristically rapid healing of the skin lesion.     

Successful treatment of pyoderma gangrenosum with topical 0.5% nicotine cream

Pyoderma gangrenosum is potentially a devastating and destructive disorder. There is no uniformly effective or specific therapy for pyoderma gangrenosum. Previous reports of nicotine therapy for pyoderma gangrenosum have suggested it to be efficacious. Unfortunately, previous reports were restricted by the use of commercially available preparations of nicotine, either as a gum or patch formulation. We have used topical nicotine 0.5% w/w cetamacrogol formula A cream that enables direct application onto the lesion, as well as dose and concentration variation. Two patients with pyoderma gangrenosum treated with topical nicotine 0.5% w/w cetamacrogol formula A cream are described here, both of whom had dramatic clinical resolution of their pyoderma gangrenosum.     

Multiple cavitary pulmonary nodules in association with pyoderma gangrenosum: case report

Pyoderma gangrenosum is a rare neutrophilic disease of unknown origin that is associated with systemic diseases in 50% of cases. It is characterized by erythematous-violaceous nodular lesions that quickly progress to painful ulcers, with undermined edges, necrotic-hemorrhagic, varying in size and depth, located mainly in the lower limbs. Extracutaneous locations of pyoderma gangrenosum are rare, usually involving the lungs; the main differential diagnosis in these cases is Wegener granulomatosis. We report a case of pyoderma gangrenosum, which showed multiple cavitary lung nodules, with good response to high doses of steroids. Once excluded the possibility of Wegener granulomatosis, the authors concluded that it was the manifestation of systemic pyoderma gangrenosum with pulmonary involvement.     

Management des Pyoderma gangraenosum

Pyoderma gangrenosum is a rare neutrophilic inflammatory skin disease, mostly observed in middle-aged adults. Etiology and pathogenesis remain unclear. Autoimmune mechanisms including immune complex-mediated neutrophilic vascular reactions have been suggested. The hallmark finding in pyoderma gangrenosum is painful ulcers with sharply circumscribed and demarcated, frequently undermined, livid borders and a necrotic base. Pyoderma gangrenosum has been described in association with a great variety of systemic disorders, ranging from inflammatory bowel diseases to myeloproliferative disorders. The diagnosis of pyoderma gangrenosum is based primarily on the clinical presentation and course. It is usually a diagnosis of exclusion. Histopathological and laboratory findings in pyoderma gangrenosum are nonspecific. The aims of therapy are the complete suppression of inflammatory disease activity, promotion of wound healing and control of pain. Frequently, successful treatment of associated diseases leads to an improvement or complete remission of pyoderma gangrenosum. Surgical interventions, including aggressive ulcer excision, recipient site preparation and autologous skin grafting have to be avoided during the active phase of the disease because the likely occurrence of pathergy inducing new lesions at surgical sites and causing a worsening the original lesions.     

Factitious panniculitis masquerading as pyoderma gangrenosum

We report a case of factitious panniculitis masquerading as florid pyoderma gangrenosum in a 35-year-old woman. At presentation, she had tender, ecchymotic plaques over the lower trunk and limbs, and several biopsies showed active lobular panniculitis. However, the extensive ulceration that ensued was clinically persuasive for pyoderma gangrenosum. We elected to treat the inflammatory element symptomatically with a range of topical and systemic medications including clobetasol propionate, tacrolimus 0.1% ointment, prednisolone, dapsone, cyclosporin A and mycophenolate mofetil, none of which effected an improvement. The possibility of a factitious aetiology had been suspected from the outset, and when signs of clinical depression emerged, antidepressant therapy was initiated and the ulcers were encased in fibreglass casts. Within a short period, healing commenced and slowly progressed with scar formation. In retrospect, we consider the diagnosis to have been factitious panniculitis on the basis of strong circumstantial evidence and the disparity between the histological and clinical features.     

Pyoderma Gangrenosum in Association with Autoimmune Neutropenia of Infancy

Abstract: We report the case of a 10‐month‐old girl who presented with a spontaneous ulcer on the left buttock which failed to heal despite antibiotic therapy. Histology showed changes consistent with pyoderma gangrenosum and the ulcer resolved rapidly with super‐potent topical steroids under occlusion. Blood tests revealed a persistent neutropenia. Immunoglobulin G (IgG) antineutrophil antibodies were detected in the serum, directed against human neutrophil antigen (HNA)‐1a. Bone marrow studies showed normocellular marrow with no evidence of dysplasia. T and B cell subsets and karotype analysis were normal. Autoimmune neutropenia is an uncommon self‐limiting condition in young children. Pyoderma gangrenosum is rare in infants, although the buttocks are a common site of involvement in this age group. Pyoderma gangrenosum in infancy can be associated with systemic disease as in adults, particularly myelodysplasia and leukemia, arthritis and inflammatory bowel disease. However, the association of pyoderma gangrenosum and autoimmune neutropenia of infancy has not previously been reported.     

Management of pyoderma gangrenosum--an update

Pyoderma gangrenosum is a neutrophilic dermatosis with distinctive clinical manifestations. It is frequently associated with systemic diseases like inflammatory bowel disease, rheumatoid arthritis and myeloproliferative diseases. The etiopathogenesis of pyoderma gangrenosum is still not well understood. Clinically it is classified into ulcerative, pustular, bullous and vegetative types. The diagnosis mainly depends on the recognition of evolving clinical features as there are no specific investigations for the diagnosis. It is essential to exclude other infectious diseases before therapy is initiated as corticosteroids and immunosuppressant therapy are the mainstays in the treatment of this disease. Recently, drugs like tacrolimus, mycophenolate mofetil and infliximab have shown promising results in this condition. Recent concepts regarding the various types of pyoderma gangrenosum and its management are reviewed.     

Strategies for optimizing treatment with efalizumab in moderate to severe psoriasis

BACKGROUND: Diagnosis of pyoderma gangrenosum can be difficult, leading to overdiagnosis or underdiagnosis. OBJECTIVE: To identify clinical features helpful in establishing a diagnosis of pyoderma gangrenosum and to compare the characteristics of patients with pyoderma gangrenosum with those of patients with chronic venous ulcers. METHOD: A retrospective chart review was performed in 28 patients with typical pyoderma gangrenosum and compared with the clinical features in 28 patients with chronic venous ulcers. RESULTS: (1) Even when other body sites are affected, pyoderma gangrenosum usually affects the upper and lower legs and feet or peristomal sites compared with chronic venous ulcers that are limited to the lower legs and feet. (2) Pyoderma gangrenosum can be associated with systemic diseases, especially inflammatory bowel disease. (3) Pustules and purulent discharge are features of pyoderma gangrenosum but not of chronic venous ulcers. (4) Crater-like holes or cribriform scarring is commonly seen in pyoderma gangrenosum but not in chronic venous ulcers. (5) Pathergy is a specific but not sensitive finding of pyoderma gangrenosum. It does not occur in patients with chronic venous ulcers. CONCLUSIONS: Diagnosis of pyoderma gangrenosum should be considered in patients with purulent ulcers affecting the legs or peristomal sites. To confirm the diagnosis, specific features should be sought, including pathergy, crater-like holes or cribriform scarring, and association with inflammatory bowel disease. Other causes of ulceration should be excluded.     

Neutrophilic Dermatoses

Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis are neutrophilic dermatoses – conditions that have an inflammatory infiltrate consisting of mature polymorphonuclear leukocytes. The neutrophils are usually located within the dermis in Sweet syndrome and pyoderma gangrenosum; however, in subcorneal pustular dermatosis, they are found in the upper layers of the epidermis. Sweet syndrome, also referred to as acute febrile neutrophilic dermatosis, is characterized by pyrexia, elevated neutrophil count, painful erythematous cutaneous lesions that have an infiltrate of mature neutrophils typically located in the upper dermis, and prompt clinical improvement following the initiation of systemic corticosteroid therapy. Classical, malignancy-associated, and drug-induced variants of Sweet syndrome exist. Pyoderma gangrenosum is characterized by painful, enlarging necrotic ulcers with bluish undermined borders surrounded by advancing zones of erythema; its clinical variants include: ulcerative or classic, pustular, bullous or atypical, vegetative, peristomal, and drug-induced. Subcorneal pustular dermatosis is an uncommon relapsing symmetric pustular eruption that involves flexural and intertriginous areas; it can be idiopathic or associated with cancer, infections, medications, and systemic diseases. Since Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis share not only the same inflammatory cell but also similar associated systemic diseases, it is not surprising that the concurrent or sequential development of these neutrophilic dermatoses has been observed in the same individual. Also, it is not unexpected that several of the effective therapeutic interventions – including systemic drugs, topical agents, and other treatment modalities – for the management of these dermatoses are the same. The treatment of choice for Sweet syndrome and idiopathic pyoderma gangrenosum is systemic corticosteroids; however, for subcorneal pustular dermatosis, dapsone is the drug of choice. Yet, tumor necrosis factor-α antagonists are becoming the preferred choice when pyoderma gangrenosum is accompanied by inflammatory bowel disease or rheumatoid arthritis. Potassium iodide and colchicine are alternative first-line therapies for Sweet syndrome and indomethacin (indometacin), clofazimine, cyclosporine (ciclosporin), and dapsone are second-line treatments. Cyclosporine is effective in the acute management of pyoderma gangrenosum; however, when tapering the drug, additional systemic agents are necessary for maintaining the clinical response. In some patients with subcorneal pustular dermatosis, systemic corticosteroids may be effective; yet, systemic retinoids (such as etretinate and acitretin) have effectively been used for treating this neutrophilic dermatosis – either as monotherapy or in combination with dapsone or as a component of phototherapy with psoralen andUVAradiation. Topical agents can have an adjuvant role in themanagement of these neutrophilic dermatoses; however, high-potency topical corticosteroids may successfully treat localized manifestations of Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis. Intralesional corticosteroid therapy for patients with Sweet syndrome and pyoderma gangrenosum, hyperbaric oxygen and plasmapheresis for patients with pyoderma grangrenosum, and phototherapy for patients with subcorneal pustular dermatosis are other modalities that have been used effectively for treating individuals with these neutrophilic dermatoses.     

A case of vulvar pyoderma gangrenosum associated with collagenous colitis

Pyoderma gangrenosum is a reactive inflammatory dermatosis which belongs to the spectrum of neutrophilic dermatoses. Due to a lack of diagnostic criteria, pyoderma gangrenosum is mainly a diagnosis of exclusion. It is rarely observed on the perineum, and vulvar involvement is even less frequent. Collagenous colitis is an idiopathic inflammatory colonic disease that is included in the microscopic colitides. The colonic mucosa and the crypt architecture are preserved but histologic alterations are found. We describe a case of collagenous colitis associated with vulvar pyoderma gangrenosum that improved spectacularly with cyclosporine 3 mg/kg/day and the twice-daily application of topical tacrolimus 0.1%.     

Pyoderma gangrenosum associated with hidradenitis suppurativa: a case report and review of the literature

Pyoderma gangrenosum is an inflammatory disease that has been found to be associated with many systemic illnesses. The case presented here is of a man with a 20-year history of hidradenitis suppurativa who developed pyoderma gangrenosum. The pyoderma lesions appeared as a single outbreak which resolved totally after immunosuppressive treatment. This association has been reported only rarely in the literature. Furthermore, in the cases reported, no relationship was apparent between the activity of both diseases. In all cases the clinical course appeared independent, with no apparent overlap in inflammatory activity or response to the drugs administered.     

Pyoderma gangrenosum with liver, spleen and bone involvement in a patient with chronic myelomonocytic leukaemia

Pyoderma gangrenosum is a neutrophilic dermatosis of unknown aetiology. Visceral involvement by pyoderma gangrenosum is rare, the lung being the most frequent site of extracutaneous disease. We describe a 73-year-old man with pyoderma gangrenosum and chronic myelomonocytic leukaemia in whom aseptic hepatosplenic abscesses and bony lesions were associated.     

Systemic ciclosporin and tacrolimus in dermatology

Ciclosporin and tacrolimus are calcineurin inhibiting immunosuppressant agents useful in the treatment of immune-mediated inflammatory dermatoses. Available data and clinical experience demonstrate ciclosporin's efficacy in treating psoriasis, atopic dermatitis, pyoderma gangrenosum, lichen planus, autoimmune bullous disease (in combination with corticosteroids), recalcitrant chronic idiopathic urticaria, and chronic dermatitis of the hands and feet. Although the role of topical tacrolimus in atopic dermatitis is well established, such experience with the oral formulation of tacrolimus has been limited. However, there are several case studies and anecdotal reports of the successful use of oral tacrolimus in various dermatoses. In this article we discuss the utility of systemic ciclosporin and tacrolimus in dermatology.     

Pyoderma gangrenosum associated with ulcerative colitis and subcutaneous and splenic abscesses

Pyoderma gangrenosum is a rare, chronic, inflammatory ulcerative skin disease of unknown etiology and pathogenesis. It is often associated with systemic disease. We describe a patient with pyoderma gangrenosum associated with ulcerative colitis and aseptic abscesses of the subcutis and spleen, which have been rarely reported previously. These manifestations were cleared by combined therapy with minocycline hydrochloride and diaphenylsulfone.