Active chronic hepatitis and pyoderma gangrenosum: report of a case

An association of pyoderma gangrenosum and active chronic hepatitis is reported. In view of the well known occurrence of pyoderma gangrenosum in ulcerative colitis and in other diseases based on a disturbance of the immune system, the absence of the expected combination of pyoderma gangrenosum and active chronic hepatitis from previously reported series was surprising. This further report of such an association is therefore both expected and significant.     

Pyoderma gangrenosum involving the head and neck

In six patients with pyoderma gangrenosum, the head and neck region was a major site of ulcerative skin disease. In two patients, the disease was limited to this anatomic site. Corticosteroids were effective therapy in five cases. In one case, occurring in association with ulcerative colitis, total proctocolectomy was required to control ulcerative scalp disease. Detailed histologic examination of a primary lesion in one case with 0.5-micron sections demonstrated morphologic evidence of mast cell activation, suggesting that mast cells may contribute to the pathogenesis of the inflammatory process in pyoderma gangrenosum.     

Topical disodium cromoglycate in the management of pyoderma gangrenosum

A 52-year-old woman who had had ulcerative colitis for more than ten years experienced a third relapse of pyoderma gangrenosum of her lower extremities. She was treated with topical disodium cromoglycate. The dosage of salicylazosulfapyridine and steroids she was taking, and by which the ulcerative colitis, but not the pyoderma gangrenosum could be controlled, was not increased. Because pyoderma gangrenosum is often very difficult to treat and may require an aggressive approach using drugs that can cause serious side effects, we believe that a topical therapy is worth trying.     

PsAPASH: a rare and recent autoinflammatory syndrome associated with hidradenitis suppurativa

Hidradenitis suppurativa is a chronic inflammatory skin disease, which affects 1% of the population, being more common in young, obese and smokers, and mainly affects armpits and groin, with formation of pustules, nodules, abscesses, scars and fistulas. Recently, its association with other autoimmune diseases such as psoriasis, psoriatic arthritis, pyoderma gangrenosum, pyogenic arthritis and ulcerative colitis have been reported. These associated forms are usually resistant to standard treatment, with immunobiologicals as promising therapy. The case of a rare form of association is reported, with only one case previously described in the literature: psoriasis arthritis, pyoderma gangrenosum, acne and hidradenitis suppurativa.     

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.     

A continuum of neutrophilic disease occurring in a patient with ulcerative colitis

A case of Sweet's syndrome (acute febrile neutrophilic dermatosis) occurring concurrently with bullous pyoderma gangrenosum is reported to emphasize the close relationship between these two disorders, Aiypical pyoderma gangrenosum and Sweet's syndrome have been described as occurring simultaneously in haematological) dyscrasias but not. to our knowledge, in ulcerative colitis. It has been proposed that pyoderma gangrenosum,; Sweet's syndrome, erythema elevatum diutinum and. subcorneal pustular dermatosis may represent manifestations along a continuum of neutrophilic dermatoses.     

Peristomal pyoderma gangrenosum in a patient with ulcero-hemorrhagic rectocolitis

Cutaneous manifestations of chronic ulcerative colitis are numerous and present in 20 p. 100 of the patients. The case of a 49-year old man with peristomal pyoderma gangrenosum (PG) is presented. In November, 1983, one year after the onset of chronic ulcerative colitis, the patient underwent total colectomy. The formation of an ileal reservoir resulted in a pelvic abscess which necessitated right lower ileostomy. Because of severe ulcerations around the stoma, the ileostomy was transferred to the left lower abdomen. Similar inflammatory ulcerations developed, and the diagnosis of pyoderma gangrenosum was made. The patient responded very well to dapsone combined with prednisone for only 6 months. The possibility of PG being an autoimmune condition is discussed, as well as the differential diagnosis. The several therapeutic approaches are summarized. The peristomal localization of PG in this patient suffering from chronic ulcerative colitis seems to be rather unique. No other case has been reported so far, to our knowledge.     

Pustular pyoderma gangrenosum: an uncommon variant which is easily misdiagnosed

Pustular pyoderma gangrenosum is a relatively uncommon clinical form of pyoderma gangrenosum; it presents with vesiculo-pustular lesions that do not develop into frank ulceration. We report a case of a 44-year-old man with associated ulcerative colitis, who was misdiagnosed as having necrotizing fasciitis. He underwent multiple debridements and a subsequent skin grafting procedure, but without improvement. The diagnosis of pyoderma gangrenosum is often challenging because there is no defining diagnostic clinical, laboratory, or histopathological feature. A high index of suspicion is, therefore, essential to diagnose pyoderma gangrenosum clinically because failure to do so in the early stages of the disease can lead to disfigurement and even unnecessary and detrimental surgery.     

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 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.     

Sweet's syndrome and pyoderma gangrenosum associated with ulcerative colitis

A young woman with ulcerative colitis developed pyoderma gangrenosum during the active phase of the disease and Sweet's syndrome (acute febrile neutrophilic dermatosis) three months after panproctocolectomy.     

Pyoderma gangrenosum associated with ulceratlve colitis-first case report from Nepal

A case of pyoderma gangrenosum with ulcerative colitis from Nepal is reported. The patient responded well to a combination of oral steroids, clofazimine. dapsone and salazopyrine within a period of 3 months.     

Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne

Autoinflammatory syndromes associated with hidradenitis suppurativa (HS) and/or acne are rare but potentially debilitating disorders if not diagnosed and treated correctly. They share a common pathogenesis involving a dysregulated innate immune system with abnormal interleukin (IL)‐1 signaling leading to sterile neutrophilic inflammation. The clinical features are recurrent episodes of fever, painful arthritis, and skin lesions consistent with HS, acne, and pyoderma gangrenosum (PG) accompanied by elevated systemic inflammatory markers in blood. So far, several clinically different syndromes have been reported in the literature including pyoderma gangrenosum, acne, and pyogenic arthritis (PAPA), pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH), pyoderma gangrenosum, acne, and spondyloarthritis (PASS), pyoderma gangrenosum, acne, pyogenic arthritis, and hidradenitis suppurativa (PAPASH), psoriatic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PsAPASH), and pyoderma gangrenosum, acne, and ulcerative colitis (PAC). The rarity of the syndromes complicates the establishment of evidence‐based treatment guidelines. Furthermore, treatment can be challenging due to lack of response to standard treatment modalities. Therefore, it is important to increase the awareness about these diseases in order to optimize disease management and ultimately improve the quality of life of patients.     

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: a report of 15 cases and review of the literature

BackgroundPyoderma gangrenosum is a condition that is included among the neutrophilic dermatoses. Given its low incidence, few studies have addressed its epidemiology or treatment.ObjectiveTo describe the epidemiological and clinical characteristics of patients with pyoderma gangrenosum along with our experience of treating the condition in a referral hospital in Malaga, Spain.Material and methodsA retrospective, observational study was undertaken in the Department of Dermatology at Hospital Clínico Universitario Virgen de la Victoria in Malaga, Spain between January 2000 and December 2009 and included all patients diagnosed with pyoderma gangrenosum.ResultsThe incidence of pyoderma gangrenosum in our reference population is 3.26 cases per million inhabitants per year. The most frequent concomitant systemic disease was ulcerative colitis (5 cases, 33%). In 4 patients with that disease, pyoderma gangrenosum appeared during a flare-up. In 80% of cases, patients were not referred to a dermatologist during the initial phase of pyoderma gangrenosum, and most referrals were from gastroenterology or general surgery (4 patients each, 52%).ConclusionsPatients with pyoderma gangrenosum are often referred to dermatologists by other specialists after a varying period of time has elapsed without achieving an accurate diagnosis. In these patients, especially those between 20 and 40 years of age, it is essential to rule out concomitant disease. Adalimumab is a good treatment option for pyoderma gangrenosum.     

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.     

TREATMENT OF PYODERMA GANGRENOSUM WITH CLOFAZIMINE

We report a case of pyoderma gangrenosum in a 80-year-old woman suffering from ulcerative colitis and treated with clofazimine. Significant improvement was evident within 5 days following commencement of therapy, complete healing occurred after only 4 weeks of treatment.     

Chronic vegetating pyodermia in cellular immune deficiency]

The simultaneous occurrence of pyoderma gangrenosum together with other diseases, most frequently ulcerative colitis, and with disorders of the humoral immune system is known. The etiology of the individual case remains nevertheless unclear in most instances. We report on an otherwise normal patient with normal immunoglobulin levels, who showed, by use of lymphocyte differentiation and functional tests, a nearly complete lack of T-cell reactivity. A substitution with transfusions of fresh blood lead to rapid healing of the clinical picture, which so far had been resistant to long term application of antibiotics, azathioprine and topical steroids. Therefore it is recommended to look for functional defects of the T-cell system as a cause of pyoderma gangrenosum.