Pyoderma gangrenosum in childhood

We have treated 4 cases of pyoderma gangrenosum in infancy and childhood in past 2 years. The ages at onset were 6 months, 8 months, 6 months and 11.5 years respectively. Initial lesions were papulopustular in 3 and nodular in 1 patients but later on all of them developed ulcerative lesions with erythematous, violaceous, infiltrated or undermined edges. The lesions were numerous in 3 patients but few in 1 patient. The first patient had very high leucocyte count. In the second patient skin lesions of PG followed measles vaccinations. We could not reveal any association in the other two patients. None of the patient responded to antibiotic therapy. Three patients were treated successfully with dapsone and one with a combination of dapsone and prednisolone. We conclude that pyoderma gangrenosum is not so rare in infancy and it is more likely to occur without associated systemic diseases.     

Pustular pyoderma gangrenosum

A 79-year-old woman was admitted to our hospital with pustular pyoderma gangrenosum and an associated IgG kappa monoclonal gammopathy. The patient is currently being evaluated for possible multiple myeloma. IgG multiple myeloma and IgG monoclonal gammopathies are very rare in patients with pyoderma gangrenosum. The skin lesions are improving with the use of prednisone.     

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.     

Rare association of pyoderma gangrenosum and palmoplantar pustulosis: A case report and review of the previous works

Pyoderma gangrenosum is a rare inflammatory, ulcerative skin disease that mainly involves the lower extremities. It frequently occurs in association with systemic diseases such as inflammatory bowel disease, myeloproliferative disorders and rheumatoid arthritis. Palmoplantar pustulosis is also an inflammatory dermatosis characterized by recurrent sterile pustules localized on the palms and soles. These two dermatoses are histologically characterized by neutrophilic infiltration into the lesional skin. Co‐occurrence of pyoderma gangrenosum and palmoplantar pustulosis in a single patient is extremely rare. We report a case of pyoderma gangrenosum occurred in a patient with palmoplantar pustulosis, with a review of the previously reported cases. A 68‐year‐old Japanese woman with a 10‐year history of palmoplantar pustulosis developed a skin ulcer on the left lower leg. The ulcer was diagnosed as pyoderma gangrenosum based on the clinical and histological findings, and rapidly improved in response to oral prednisolone. In addition to our case, five cases with palmoplantar pustulosis who developed pyoderma gangrenosum have been reported. These cases were thought to have some characteristics in common, such as marked female predominance, no association with inflammatory bowel disease and myeloproliferative disorders, and good response to less aggressive therapy. The co‐occurrence of pyoderma gangrenosum and palmoplantar pustulosis in our case may have an etiological link, rather than being a coincidental complication.     

坏疽性脓皮病11例临床分析报告

报告１１例坏疽性脓皮病，均有不同程度皮肤损害，伴发热等全身症状和其它脏器累及，其中以肺部累及较为突出。对本病的治疗应在了解全身病情的基础上以全身使用皮质类固醇激素为主，控制剂量相当于强地松０．５￣１ｍｇ（ｋｇ．ｄ），必要时辅助应用免疫抑制剂，及时处理并发症。全身用抗生素只有辅助作用，局部治疗有利于控制感染和促进皮损愈合。     

Pyoderma gangrenosum following cesarean delivery

We describe the case of a 30-year-old woman who, 5 days after giving birth to her first child by cesarean section, presented with dehiscence of one end of the surgical wound and a lesion on her leg that developed into a well-defined ulcer; both lesions were very painful. The patient was initially diagnosed with a skin infection and later with superficial pyoderma gangrenosum. The lesions were treated with topical corticosteroids and a good response was observed. No evidence was found of underlying disease. Isolated cases of pyoderma gangrenosum associated with pregnancy or cesarean delivery have been reported in the literature. The etiology of pyoderma gangrenosum is currently unknown, but some theories suggest an immunologic mechanism. Gestation is known to generate a state of immune tolerance that could play a role in the development of the disease and future studies may help to clarify the significance of this association.     

Pyoderma gangrenosum or cutaneous lymphoma: a difficult clinical diagnosis

Pyoderma gangrenosum is frequently associated with an underlying condition such as ulcerative colitis or a myeloproliferative syndrome. However, lymphoproliferative malignancies have only rarely been seen concurrently with pyoderma gangrenosum. We present the case of a patient with small lymphocytic lymphoma who noted a slowly enlarging skin ulcer that was clinically consistent with pyoderma gangrenosum. Examination of a biopsy specimen showed infiltration of the skin with lymphoma cells as well as deeper necrotic material and thrombosis of vessels that were infiltrated by lymphoma. This case illustrates the difficulty of differentiating pyoderma gangrenosum from cutaneous lymphoma clinically.     

Pyoderma gangrenosum coexisting with acute myelogenous leukaemia

The frequency of occurrence of malignant neoplasms in the cases of pyoderma gangrenosum is not exactly determined, but it can be assessed to be at 7%. The aim of the study was to report a 26-year-old male patient with pyoderma gangrenosum coexisting with acute myelogenous leukaemia. The first skin lesions on both tibia occurred in June 2001. Prior to the proper diagnosis of pyoderma gangrenosum, the patient was treated surgically. Because of the dramatic dermatological and general condition in November 2001, the patient was admitted to the Dermatological Department of the Silesian Medical Academy in Katowice where the diagnosis of pyoderma gangrenosum was established. On the clinical and biochemical picture, the diagnosis of pyoderma gangrenosum within acute myelogenous leukaemia was made. Initially, cyclosporin A 200 mg orally per day in the therapy of pyoderma gangrenosum was administered to achieve a slight clinical improvement. Although chemotherapy leukaemia was performed, the patient died after 4 months of the confirmation of the acute myelogenous leukaemia diagnosis.     

Minimizing the risk of post-operative pyoderma gangrenosum

A 61-year-old woman with seropositive rheumatoid arthritis developed numerous ulcers due to pyoderma gangrenosum at suture entry/exit sites following an arthroplasty of the right hip when interrupted silk sutures were used to close the skin. When a subsequent arthroplasty was performed on the left hip and subcuticular Dexon sutures were used to close the skin only two small ulcers developed. Sixteen cases of pyoderma gangrenosum developing in surgical wounds have previously been reported. We recommend that surgery in patients with a history of pyoderma gangrenosum is performed when the pyoderma is clinically quiescent, and that subcuticular sutures are used for skin closure, thus avoiding puncturing the skin surface.     

Lung involvement in pyoderma gangrenosum: a case report and review of the literature

Pyoderma gangrenosum is a neutrophilic dermatosis that, in addition to its characteristic skin manifestations, can cause visceral alterations. Our patient was a 34-year-old woman with pyoderma gangrenosum that was exacerbated by iodine and that also affected the lungs. Other published cases of lung involvement in pyoderma gangrenosum are reviewed and we discuss the possible exacerbation of this disease by iodine. Lung involvement is the most common extracutaneous manifestation of pyoderma gangrenosum and the main differential diagnosis is then with Wegener granulomatosis. It is important to remember the possible presentation of extracutaneous manifestations of pyoderma gangrenosum, including particularly lung involvement, in order to avoid subjecting a patient to aggressive diagnostic tests, at least in the initial stages.     

Treatment of idiopathic pyoderma gangrenosum with infliximab: induction dosing regimen or on-demand therapy?

Pyoderma gangrenosum is an ulcerative skin disease of unknown origin. Recently tumor necrosis factor alpha inhibitors have been gaining attention in the treatment of recalcitrant pyoderma gangrenosum. However, there is a lack of an optimal dosing strategy in the published literature about the treatment of idiopathic pyoderma gangrenosum, and this is responsible for substantially different treatment strategies. Therefore, it is necessary to report experiences in this patient population in order to develop an optimal dosing strategy and to clarify whether regularly scheduled or on-demand therapy is suitable for idiopathic pyoderma gangrenosum. Herein we report a recalcitrant case of an idiopathic pyoderma gangrenosum treated successfully with a dosing regimen similar to the one that has been used in psoriasis. By doing so, we were capable of controlling the disease and improving the quality of life in our patient.     

Blastomycosis-like pyoderma.

Seven patients with blastomycosis-like pyoderma had skin lesions of four months' to six years' duration. The criteria for the diagnosis of blastomycosis-like pyoderma include the clinical presentation of large verrucous plaques with multiple pustules and elevated border, pseudoepitheliomatous hyperplasia with abscess histologically, and the growth of at least one pathogenic bacteria from the culture of a tissue-biopsy specimen. The differential diagnosis includes deep fungus infection (especially North American blastomycosis), bromoderma, pyoderma gangrenosum, mycobacterial infections, giant keratoacanthoma, and squamous cell carcinoma. Generally, the patients had one or more conditions that could have affected their systemic or local immunologic competence to infection. We believe that the clinical and histologic features in these cases of blastomycosis-like pyoderma were produced by an unusual, exaggerated, vegetating-tissue reaction to a primary or secondary bacterial infection.     

Minocycline Hydrochloride Hyperpigmentation Complicating Treatment of Pyoderma Gangrenosum

Minocycline-associated hyperpigmentation is an uncommon side effect We report the case of a patient with pyoderma gangrenosum successfully treated with oral minocycline but complicated by marked hyperpigmentation in his pyoderma gangrenosum and acne scars. One of the clinical forms of minocycline hyperpigmentation includes dark-blue or black macules in depressed acne scars or other sites of skin inflammation; this pattern seems to be independent of the total cumulative dose and the skin process.     

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.     

Pyoderma Gangrenosum and Sterile Multifocal Osteomyelitis Preceding the Appearance of Takayasu Arteritis

Abstract: A 9-month-old infant had pyoderma gangrenosum (PG) and sterile osteomyelitis. Three years later the patient developed Takayasu arteritis (TA). Sterile osteomyelitis was reported in approxitnately 30 patients with different skin lesions, but never together with pyoderma gangrenosum. The association of PG and TA has been reported rarely, and then maiitly in adults or in children over age 9 years. This rare association should alert physicians with a case of infantile PG of unknown etiology to consider the possible diagnosis of TA.     

Portable negative‐pressure wound therapy for pyoderma gangrenosum: Report of two cases

Pyoderma gangrenosum is a chronic non‐infectious neutrophilic dermatosis that causes undermining ulcers. Topical therapies for the deep ulcers of pyoderma gangrenosum have not been established. To investigate whether negative‐pressure wound therapy is effective for a pyoderma gangrenosum ulcer, we used the PICO single use negative‐pressure wound therapy system (Smith & Nephew, London, UK) for two pyoderma gangrenosum patients. In these cases, the ulcers decreased in size and necrolytic tissue was removed notably. Moreover, there were no secondary infections nor was there Koebner phenomena. Our cases suggest that portable negative‐pressure wound therapy can be a treatment option for deep, intractable ulcers caused by pyoderma gangrenosum. Because portable negative‐pressure wound therapy devices afford increased mobility to patients, they can give the patient a better quality of life than standard negative‐pressure wound therapy systems do.     

Superficial granulomatous pyoderma: a localized vegetative form of pyoderma gangrenosum

Twenty-five patients had superficial ulcerative and vegetative pyoderma with granulomatous histologic findings. Healing occurred without systemic corticosteroid therapy in all but three patients. All patients had clinical pyoderma gangrenosum. In five patients the lesion occurred after surgery of the skin. Histopathologic study of 40 biopsy specimens showed focal neutrophilic abscesses of the papillary dermis, often with peripheral palisading histiocytes and foreign-body giant cells. Pseudoepitheliomatous, vegetative hyperplasia and sinus tract formation were observed frequently. All patient had massive areas of plasmacytosis, and 13 had eosinophils. Granulation tissue, hemorrhage, and fibrosis were additional features in some areas. Foreign material in the lesions was considered unimportant, except in one patient with a starch granuloma. Therapy with local corticosteroids, minocycline, tetracycline, or sulfa drugs resulted in healing in 15 patients. We believe that we have identified a localized, limited form of chronic superficial pyoderma gangrenosum with verrucous and ulcerative lesions and a granulomatous histologic appearance that represents a unique pattern of this disease in some patients.     

A distinctive vesiculopustular eruption associated with hepatobiliary disease

Background Cutaneous manifestations of hepatobiliary disease may be a clue to the clinical diagnosis. We encountered a form of vesiculopustular eruption in five patients with hepatobiliary disease. A similar eruption has been described in the setting of ulcerative colitis, and has received the designation of vesiculopustular pyoderma gangrenosum. Methods Skin biopsy specimens were examined by conventional histopathologic methods and, in three cases, fresh tissue was available for direct immunofluorescence examination. A diagnosis of hepatobiliary disease was known in three of the patients and in the other two it was established by the skin biopsies. Results All skin biopsies of the five patients showed a neutrophilic dermatosis that had two morphologies. One was characterised by superficial neutrophilic dermolysis with subepidermal bulla formation and the other by a nonulcerating destructive suppurative folliculitis and perifollicular neutrophilic dermolysis. Both were frequently accompanied by a neutrophilic and lymphotic eccrine hidradermitis and a Sweet's syndrome-like vascular reaction. The lesions were held to represent a novel vesiculopustuiar eruption with features reminiscent of pyoderma gangrenosum. Conclusions We propose the term vesiculopustular eruption of hepatobiliary disease for this distinctive clinicopathologic entity, which bears a strong histologic resemblance to pyoderma gangrenosum. Patients with a known hepatopathy who present with necrotizing vesicles and pustules may have such an eruption. The onset may be the first clue to or herald an exacerbation of underlying liver disease.     

Free flap coverage of pyoderma gangrenosum leg ulcers

Background: Pyoderma gangrenosum is a necrotizing skin disorder of unknown cause. Treatment of pyoderma gangrenosum usually entails medical treatments. Surgical treatment is generally not successful. Objective: Two patients with pyoderma gangrenosum of their lower extremities are presented. The application and utility of microvascular free flap coverage of their ulcers is discussed. Results: Both patients achieved successful healing with microvascular free flaps. Conclusion: Microvascular free flap coverage may be a surgical treatment option for selected patients with pyoderma gangrenosum.     

Pyoderma gangrenosum in a patient with essential thrombocythemia

BACKGROUND: Pyoderma gangrenosum is an uncommon ulcerative condition associated with inflammatory bowel disease, arthritis, and hematologic disease. We report a patient with essential thrombocythemia and pyoderma gangrenosum. OBJECTIVE: This article is a review of the associations between pyoderma gangrenosum and other diseases. RESULTS: There have been two previous reports of patients with pyoderma gangrenosum and essential thrombocythemia. CONCLUSION: There may be a possible association between pyoderma gangrenosum and essential thrombocythemia. The diagnosis of pyoderma gangrenosum should be considered in patients with essential thrombocythemia and cutaneous ulcers.