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PubMed2006

Pyoderma gangraenosum, a rare, but potentially fatal complication in paediatric oncology patients.

Pyoderma gangraenosum (PG) is a serious chronic, ulcerative skin disorder afflicting both adults and children. As PG is often associated with systemic diseases (>50%) such as inflammatory bowel disease, rheumatoid arthritis or haematological disorders, it requires a multidisciplinary approach. This disorder is not commonly reported in paediatrics; therefore children with PG represent a particularly difficult diagnostic challenge. Clinical diagnosis is often delayed and PG is only considered after eliminating other causes of cutaneous ulcers. We report a 4-year-old boy with secondary myelodysplastic syndrome following treatment for acute lymphoblastic leukaemia who presented with a massive inflammatory, ulcerative proliferation of the lower lip which was diagnosed as PG. We have reviewed the literature with reference to diagnostic criteria and treatment options of this disorder that is particularly rare in childhood.

Administration, Topical↗
PubMed1999

[Pyoderma gangrenosum and portal vein thrombosis in a 33-year-old female patient].

HISTORY AND CLINICAL FINDINGS: The diagnosis of pyoderma gangraenosum (PG) was made in a 33-year-old woman with ulcerative (palm-sized) skin changes and pain of the lower leg that had developed over two weeks and was accompanied by fever (39 degrees C). Treatment with prednisolone and azathioprine was initiated. As soon as the medication was reduced new skin changes developed. Two months after onset of the illness she had to be hospitalized because of fever, epigastric pain on pressure and deteriorating general condition. Physical examination provided no significant further information. LABORATORY RESULTS: The differential count demonstrated leucocytosis (15.5 Gpt/l) with a marked monocytosis (25%) as well as anaemia (haemoglobin concentration 5.2 mmol/l). C-reactive protein was elevated (120.20 mg/l). Thromboplastin time was 60%, D-dimer 1000 micrograms/l, thrombin-antithrombin-III complex 9.7 micrograms/l. ADDITIONAL INVESTIGATIONS: Sonography and computed tomography of the upper abdomen revealed splenomegaly, ascites, thrombosis of the portal, splenic and superior mesenteric veins. Bone marrow puncture showed marked increase in blasts (14%) and monocytes (10%). TREATMENT AND COURSE: The findings indicated chronic myelomonocytic leukaemia with PG and the described venous thromboses. The cutaneous changes completely receded on administration of hydroxyurea (1.0 g/d). Other causes of the skin eruption were excluded. Phenprocoumon (INR between 2 and 3) was given in treatment of the thromboses. CONCLUSION: When PG is diagnosed, intensive search for an underlying cause must be undertaken, because of its frequent association with serious systemic disease. Only early specific treatment will improve the skin condition.

Adult↗
PubMed1989

German shepherd dog pyoderma: a genetic disorder.

In this study the pedigrees of 42 German Shepherd dogs with German Shepherd dog Pyoderma (GSP) were analysed. Parents, littermates and offspring of the affected dogs were traced and their owners were questioned on characteristic skin lesions. Evidence suggesting an autosomal recessive trait was found. Breeders are advised to exclude affected animals and their relatives from further breeding.

Animals↗
PubMed1990

Investigations on the role of flea antigen in the pathogenesis of German shepherd dog pyoderma (GSP).

Skin reaction patterns to the intradermal injection of a whole-body flea extract were examined in five physically healthy dogs and in 24 dogs with German Shepherd dog Pyoderma (GSP) at 15 and 30 minutes and at 1, 2, 4, 8, 24, 48 and 72 hours after the injection. In 10 out of 24 GSP dogs a positive skin reaction was observed macroscopically after 15 minutes. Delayed reactions at 24 or 48 hours were not observed. In the control group neither immediate nor delayed reactions were observed. The histopathologic skin changes were basically the same in both groups: an initial polymorphonuclear reaction followed by a mononuclear cell reaction. In the GSP dogs, however, these changes occurred earlier and were more prolonged than in the normal dogs. No flea-antigen-specific IgGd antibodies could be demonstrated by enzyme-linked immunosorbent assay. It is concluded that delayed type hypersensitivity to flea antigen does not play a role in the pathogenesis of GSP. Immediate type hypersensitivity may contribute to the disease in some cases.

Animals↗
PubMed1990

Investigations on the role of staphylococci in the pathogenesis of German shepherd dog pyoderma (GSP).

Skin reaction patterns to intradermal injections of a Staphylococcus intermedius antigen were examined in physically healthy dogs and in dogs with German Shepherd dog Pyoderma (GSP) at 15 and 30 minutes and at 1, 2, 4, 6, 8, 24, 48 and 72 hours after the injection. In both groups the skin histopathology revealed an aspecific inflammatory response of an early polymorphonuclear reaction, followed by a mononuclear cell reaction at 24 and 48 hours. It is concluded that hypersensitivity to staphylococcal antigens does not play a role in the pathogenesis of GSP.

Animals↗
PubMed2004

Review of 26 cases of classical pyoderma gangrenosum: clinical and therapeutic features.

BACKGROUND: Classical pyoderma gangrenosum (CPG) is an uncommon, idiopathic, neutrophilic skin disease, generally associated with other skin conditions and systemic diseases, and with several treatment options. OBJECTIVE: The objective of this review was to know the clinical and therapeutic features of our cases of CPG. METHODS: Twenty-six cases of CPG were included; 65 episodes of PG and 120 treatment courses were analysed; and the mean follow-up was 4.6 years. RESULTS: 21 cases (80%) had lesions on the lower extremities, 16 (61%) had recurrences, 22 (84%) had some disease associated, and 13 (50%) had autoimmune inflammatory conditions. Cyclosporin A (CSA) was used in 22 (84%) patients and 51 (78%) episodes, and prednisone (PDN) was used in 15 (57%) patients and 26 (40%) episodes. CONCLUSIONS: CSA efficacy was excellent, with an early response and acceptable toxicity, although it did not seem to have any impact on the incidence of PG recurrences.

Adolescent↗
PubMed2005

Use of etanercept in treatment of pyoderma gangrenosum in a patient with autoimmune hepatitis.

A 30-year-old female with past medical history of autoimmune hepatitis was sent to our clinic for consultation and treatment of refractory pyoderma gangrenosum (PG) of bilateral lower extremities. At the time of presentation, she had failed treatment with prednisone, azathioprine and local wound care. Treatment options were limited by the patient's autoimmune hepatitis. This patient was treated with etanercept and a taper of oral prednisone, which resulted in a complete clinical cure of her PG. More studies are needed to evaluate the safety and efficacy of etanercept for PG in patients with liver disease.

Adult↗
PubMed2001

Management of pyoderma gangrenosum.

Management of pyoderma gangrenosum continues to be a therapeutic challenge, both because the low incidence of the disease makes it difficult to conduct large-scale randomized studies and because pathogenic mechanisms are not yet well defined. The selection of drugs and dosing schedules is therefore mainly guided by clinical experience.

Cromolyn Sodium↗
PubMed2002

A nursery outbreak of Staphylococcus aureus pyoderma originating from a nurse with paronychia.

An outbreak of methicillin-susceptible Staphylococcus aureus pyoderma occurred in the nursery of a tertiary-care referral center. All strains retrieved from the outbreak, including one from a nurse's infected finger, were typed by arbitrarily primed polymerase chain reaction and automated ribotyping. The results indicated that the spread of the outbreak was probably facilitated by contamination of the nurse with paronychia.

Base Sequence↗
PubMed1995

Pyoderma fistulans sinifica (fox den disease): a distinctive soft-tissue infection.

Pyoderma fistulans sinifica (PFS, also referred to as fox den disease because its multiple fistulae and sinuses resemble the structure of a fox den) is a distinct chronic infectious disease in which epithelialized tracts form within the subdermal fatty tissue. PFS, which has not been previously described in the English-language literature, must be differentiated from hidradenitis suppurativa, pilonidal sinus, and perianal fistula. The fistulous tracts of PFS are always lined by stratified squamous-cell epithelium but, unlike those of hidradenitis, reach deep into the subcutaneous fat, run epifascially for long distances, and have no relation to skin appendices. We report on 10 men (mean age +/- SD, 36 +/- 5 years) with PFS (mean duration +/- SD, 11 +/- 7 years). Bacterial cultures of affected tissue from these patients yielded a total of 14 facultative and 31 obligate anaerobic species. Treatment consisted of wide en-bloc excision down to the fascia, including all fistulae. Antibiotic therapy temporarily reduced purulent discharge but did not eradicate the infection. Two patients who underwent fistulotomy without wide en-bloc excision developed recurrences.

Abscess↗
PubMed2006

Pyoderma gangrenosum complicating pacemaker implant.

A 70-year-old lady with diabetes and monoclonal gammopathy underwent pacemaker implant for 2:1 atrioventricular block. Within 7 days, a painful, infiltrating, necrotic lesion involved the implant area. Biopsy was compatible with pyoderma gangrenosum and corticosteroid treatment led to healing in 3 weeks.

Aged↗
PubMed1990

Squamous syringometaplasia in lobular panniculitis and pyoderma gangrenosum.

Squamous metaplasia of eccrine sweat glands has been most frequently described in chronic cutaneous ulcerations with associated epidermal hyperplasia. We found examples of the process in skin biopsy specimens from five patients: three had associated lobular panniculitis and two had lesions of pyoderma gangrenosum. The metaplasia was located in the mid-to-deep reticular dermis in all five patients and extended into the superficial subcutis in one. Immunohistochemical stains for CEA and S-100 protein were used to accentuate the relationship of the metaplastic islands with eccrine ducts. It is postulated that necrosis of a portion of the eccrine duct is the stimulus for this process.

Adenoma↗
PubMed1992

Case report: severe pyoderma associated with familial Mediterranean fever--favorable response to colchicine in three patients.

Familial Mediterranean fever (FMF) is an autosomal recessive disorder that occurs most frequently among Sephardic Jews and Armenians. It is characterized by recurrent episodes of fever, peritonitis, pleuritis, and arthritis. Skin lesions are seen in some patients. Diagnosis of FMF usually is made on clinical grounds only, typically when recurrent attacks of abdominal pain, fever, and arthritis are observed in a patient with an appropriate ethnic background and family history. To date, there are no specific diagnostic laboratory tests for FMF. Three patients with severe recurrent Pyoderma are covered in this report. In all three cases, the cutaneous lesions were associated with clinical manifestations of FMF and responded to colchicine therapy favorably. The importance of such an association and its therapeutic consequences are emphasized.

Adolescent↗
PubMed2001

A rationale for adjuvant surgical intervention in pyoderma gangrenosum.

Medical specialists who care for patients with pyoderma gangrenosum have been reluctant traditionally to consult plastic surgeons. This is the result of previous negative experience with debridement and skin grafting. However, a new understanding of the pathophysiology of the disease process, and better therapeutic options for control have emerged. Very few studies report the results of surgical therapy of this disease, and fewer yet report successful outcome. The authors present their limited experience at the American University of Beirut with 4 patients who were controlled medically followed by skin grafting their large wounds, thus decreasing their morbidity and hospital stay. A review of the literature together with insights on the rationale and timing of surgery in this particular small group of patients are discussed.

Adolescent↗
PubMed2001

Management strategies for pyoderma gangrenosum: case studies and review of literature.

Pyoderma gangrenosum (PG) is a systemic disease with cutaneous manifestations consisting of necrotizing ulceration. The etiology of PG is controversial, and optimal management strategies have not been established. Current management is primarily medical to control the systemic inflammatory process, with occasional surgical intervention at the ulcer site. Based on the current literature and on the authors' clinical experience, the optimal outcome depends on early diagnoses and a combination of medical and surgical therapy. Initial management is directed toward medical control of the inflammatory process and local wound care. Surgical strategies involve recipient site preparation via local wound care and serial allograft followed by autologous skin graft or muscle flap coverage when necessary. Long-term wound stabilization is obtained only through control of the systemic and local inflammatory process.

Aged↗
PubMed2002

Pyoderma gangrenosum after reduction mammaplasty in an otherwise healthy patient.

Pyoderma gangrenosum (PG) is a rare postoperative complication of plastic surgery of the breast. Initial signs and symptoms resemble those of infection, and antimicrobial therapy is usually initiated and fails before considering PG as a diagnosis. Therapy consists of immune modulators, and use of corticosteroids is frequent, as is local wound care. Sufficiently small wounds are allowed to heal secondarily, but larger wounds require coverage with either skin grafts or flaps. Long-term (1 year or more) postoperative surveillance is necessary because late failure of the graft or flap can occur.

Adult↗