Familial ulcerative pyoderma gangrenosum: a report of 2 kindred

Pyoderma gangrenosum is a rare, chronic ulcerative skin disease. It is a diagnosis of exclusion, after ruling out other causes of cutaneous ulceration. The etiology of pyoderma gangrenosum is poorly understood but is likely multifactorial. We describe 2 families affected by ulcerative pyoderma gangrenosum. This familial clustering suggests a possible genetic role in the development of pyoderma gangrenosum in some cases.     

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.     

Pyoderma gangrenosum associated with severe oropharyngeal involvement and IgA paraproteinaemia

We report a patient with combined cutaneous and oropharyngeal pyoderma gangrenosum in association with an IgA lambda paraproteinaemia. The differential diagnosis of oral pyoderma gangrenosum is discussed.     

Superficial Granulomafous Pyoderma

Superficial granulomatous pyoderma, recently described as a variant of pyoderma gangrenosum, would be better termed pathergic granulomatous cutaneous ulceration as the seven previously described cases, as well as our own two cases, have significant dermal involvement histologically and heal with scarring. In contrast to pyoderma gangrenosum, lesions of superficial granulomatous pyoderma respond to less toxic anti-inflammatory agents.     

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.     

Wegener''s granulomatosis presenting as pyoderma gangrenosum

We report three cases of Wegener's granulomatosis presenting with cutaneous ulceration resembling pyoderma gangrenosum. Wegener's granulomatosis classically affects the upper and lower respiratory tracts and the kidneys. Skin involvement occurs in up to 50% of patients. Increased awareness that cutaneous involvement can take the form of pyoderma gangrenosum and that it can be a presenting sign may lead to more rapid diagnosis of Wegener's granulomatosis.     

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, ovarian carcinoma treated with Melphalan and acute myelomonocytic leukaemia: report of one case and literature review (author's transl)]

A 67-year-old woman suffered from an ovarian carcinoma with lymph nodes metastasis. During 3 years, she was treated with alkylating agents (Melphalan). At the end of therapy, no recurrence was observed. Two years later, she developed concomitantly pyoderma gangrenosum and acute myelomonocytic leukaemia. Death occurred rapidly. The association between pyoderma gangrenosum and acute leukaemia is discussed in the light of 16 cases previously reported in the literature. In this case, an induction of leukaemia by cytostatic drugs seems likely. The authors conclude that pyoderma gangrenosum may be considered as a cutaneous signs of acute leukaemia.     

Pyoderma gangrenosum in a thoracotomy wound associated with a pulmonary cavitating lesion

We present a patient with both pyoderma gangrenosum in a thoracotomy wound and a pulmonary cavitating lesion with the histological features of Wegener's granulomatosis. An ulcer with blue undermined edges developed in our patient's thoracotomy scar after a lobectomy was carried out for the cavitating lung lesion and c Antineutrophil Cytoplasmic Antibodies (cANCA), which is highly specific for active Wegener's granulomatosis but was negative at the time of her skin lesion. This suggests that our patient had cutaneous pyoderma gangrenosum rather than cutaneous Wegener's granulomatosis, although the two conditions may occasionally have similar clinical and histological features.     

Case of multiple myeloma associated with extramedullary cutaneous plasmacytoma and pyoderma gangrenosum

A variety of cutaneous manifestations has been described in multiple myeloma including extramedullary cutaneous plasmacytomas, cutaneous amyloidosis, pyoderma gangrenosum, leukocytoclastic vasculitis, necrobiotic xanthogranuloma, scleromyxedema, Sweet's syndrome, subcorneal pustular dermatosis, scleredema, and plane xanthomas etc. An 89-year-old Korean man, who had been suffering from multiple myeloma 1 year previous, presented for evaluation of two nodules on the right side of the forehead, left side of the chest (7th rib area), and multiple ulcers with papulopustules on both the thigh and the left side of the chest (2nd rib area) during 15 days, which developed at the same time. A biopsy of a lesion which manifested as a cutaneous nodule on the right side of the forehead revealed dermal infiltration by well-differentiated plasma cells, similar to those found on a bone marrow biopsy, and a biopsy of the lesion manifested as a painful ulceration on the right thigh area showing dermal neutrophilic infiltration. Histologic findings were consistent with plasmacytoma and pyoderma gangrenosum, respectively. We present a case of multiple myeloma which developed extramedullary cutaneous plasmacytoma and pyoderma gangrenosum simultaneously, which is very rare. The patient was treated with a systemic steroid and conservative therapy.     

CD8-positives,CD30-negatives kutanes T-Zell-Lymphom unter dem klinischen Bild eines Pyoderma gangraenosum

Ulcerated primary cutaneous lymphomas are not rare, but the clinical manifestation as a pyoderma gangrenosum look-alike is extraordinary. CD8-positive lymphomas are rare, unclassifiable tumours with variable prognosis. We report on a 49-year-old patient with a large ulcerated primary cutaneous lymphoma on the left chest wall presenting as pyoderma gangrenosum. With immunohistochemical staining, most lymphocytes were shown to be CD8-positive. The CD30 antigen was not expressed. After radiotherapy with complete skin irradiation, the lymphoma regressed completely. The patient has been free of relapse for 28 months so far.     

Pyoderma gangrenosum: a review

Pyoderma gangrenosum is a poorly understood disease characterized by exacerbations and remissions of morphologically unique skin ulcers. It frequently is thought to be the cutaneous manifestation of an underlying systemic disease. In this review of pyoderma gangrenosum, the characteristics of the disease are described and a differential diagnosis is formulated. Associations with systemic diseases are made through a review of the literature. The pathophysiology of pyoderma gangrenosum and probable causes are considered, and special consideration is given to the immunologic mechanisms that may be operative in the disease. Finally the currently available therapeutic alternatives are reviewed.     

Ulcerative colitis and the skin.

The association between ulcerative colitis and various abnormalities of the skin, especially pyoderma gangrenosum, has been accepted for many years. The underlying aberrations in either of the organ systems, as well as the basis of their association, however, remain obscure. Several large series of ulcerative colitis patients are reviewed to emphasize the important concomitant cutaneous findings, and investigations into the pathogenesis of pyoderma gangrenosum are discussed.     

Pyoderma gangrenosum in childhood

A review of our records disclosed eight children with pyoderma gangrenosum. All had associated inflammatory bowel disease, and four had arthritis. In three patients the bowel disease was severe and required resection of portions of the affected bowel. The cutaneous lesions had a chronic course (average duration, 2 1/2 years), and systemic therapy was required in seven cases. Previously reported cases of pyoderma gangrenosum in children are reviewed, and the management of this skin disorder in childhood is discussed.     

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.     

Histiocytosis X in an adult mimicking pyoderma gangrenosum*

A case of histiocytosis X is reported in which the presenting feature was recurrent cutaneous ulceration indistiguishable from pyoderma gangrenosum. Such a presentation of histiocytosis X has not previously been described.     

Pyoderma gangraenosum Erfolgreiche topische Therapie mit Tacrolimus (FK506)

Pyoderma gangrenosum is a distinct clinical entity characterized by chronic, recurring, destructive ulcerations. Although the pathogenesis of pyoderma gangrenosum is unknown, immunologic aberrations of neutrophil granulocytes seem to be important. Systemic steroids and macrolide lactones such as cyclosporin A and tacrolimus have been reported to be useful in the clinical management of disease. Pyoderma gangrenosum has been found to be associated with several systemic diseases.The association with chronic ulcerative colitis is well known, but the diagnosis may be complicated by early administration of systemic steroids.Therefore, local immunosuppression with topically applied agents could be an efficient therapeutic alternative especially for mild or early cutaneous lesions.We describe the successful topical treatment of a patient with multiple lesions of pyoderma gangrenosum with 0,1% tacrolimus (FK506) ointment which is known to have better dermal penetration and higher immunosuppressive potency than topical cyclosporin A. In addition, other indications for topical tacrolimus are discussed.     

Superficial granulomatous pyoderma: a case in an 11-year-old girl and review of the literature

Superficial granulomatous pyoderma is a rare entity thought to be a variant of pyoderma gangrenosum and is often mistaken for classic pyoderma gangrenosum. Superficial granulomatous pyoderma has mainly been described in adults. We describe a case of Superficial granulomatous pyoderma in an 11-year-old girl and present a review of the literature. Superficial granulomatous pyoderma maintains clinical and histopathologic characteristics distinct from classic pyoderma gangrenosum and has a more favorable prognosis. It is often responsive to conservative treatment.     

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.     

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.