Pyoderma gangrenosum

Abstract:  We present a case of a 10-year-old girl with a diagnosis of vulvar pyoderma gangrenosum. Intravenous methylprednisolone was started and on tapering the steroid regimen, the lesion showed significant enlargement and purulent discharge without any remission of fever and inflammatory activity, so she was treated with oral cyclosporin A in combination with low-dose steroid. A response to treatment was achieved after 2 weeks and clinical and laboratory follow-up at 12 months did not show any disease relapse or inflammation.

Pyoderma gangrenosum (PG) is a chronic skin disease with an incidence of 3-10 per million, and it is often associated with underlying systemic disease. A case of PG of the left leg successfully treated with local debridement and advanced and compression dressings, without systemic treatment is reported. Progression and healing of the ulcer was incredibly rapid. The treatment of PG usually consists of systemic administration of corticosteroids. But wherever the administration of systemic immunosuppressive therapy is impractical, a topical treatment can be used. This type of treatment does not represent a mere palliative, but could be significant for healing, as in the case of our patient.

Pyoderma gangrenosum-like ulcers and cellulitis of the lower extremities associated with recurrent fevers in patients with X-linked (Bruton) agammaglobulinemia have been reported to be caused by Helicobacter bilis (formerly classified as Flexispira rappini and then Helicobacter strain flexispira taxon 8). Consistent themes in these reports are the difficulty in recovering this organism in blood and wound cultures and in maintaining isolates in vitro. We confirmed the presence of this organism in a patient's culture by using a novel application of gene amplification polymerase chain reaction and electrospray ionization time-of-flight mass spectrometry. An adolescent boy with X-linked agammaglobulinemia presented with indurated plaques and a chronic leg ulcer whose origin was strongly suspected to be an H bilis organism. Histologic analysis demonstrated positive Warthin-Starry staining of curvilinear rods, which grew in culture but failed to grow when subcultured. They could not be identified by conventional techniques. A combination of gene amplification by polymerase chain reaction and electrospray ionization time-of-flight mass spectrometry confirmed the identity of this organism. This novel technology was useful in the identification of a difficult-to-grow Helicobacter organism, the cause of pyoderma gangrenosum-like leg ulcers in patients with X-linked agammaglobulinemia. Correct identification of this organism as the cause of pyoderma gangrenosum-like ulcers in patients with X-linked agammaglobulinemia is of great importance for the early initiation of appropriate and curative antibiotic therapy.

Auto-inflammatory syndromes are a new group of distinct hereditable disorders characterized by episodes of seemingly unprovoked inflammation (most commonly in skin, joints, gut, and eye), the absence of a high titer of auto-antibodies or auto-reactive T cells, and an inborn error of innate immunity. A narrative literature review was carried out of studies related to auto-inflammatory syndromes to discuss the pathogenesis and clinical manifestation of these syndromes. This review showed that the main monogenic auto-inflammatory syndromes are familial Mediterranean fever (FMF), mevalonate kinase deficiency (MKD), Blau syndrome, TNF receptor-associated periodic syndrome (TRAPS), cryopyrin-associated periodic syndrome (CAPS), and pyogenic arthritis with pyoderma gangrenosum and acne (PAPA). The data suggest that correct diagnosis and treatment of monogenic auto-inflammatory diseases relies on the physicians' awareness. Therefore, understanding of the underlying pathogenic mechanisms...

Pyoderma gangrenosum (PG) is a rare chronic inflammatory skin disease characterized by the recurring development of necrotizing and painful ulcers and therefore, often misinterpreted. This condition can simulate fulminant infection, particularly after surgery. The presentation is often impressive in extensive ulcerations and scarring and lead to significant sequelae. A rapid initial management avoids a dramatic evolution. We report the case of three PG for patients followed for breast reconstruction after breast cancer. PG is a neutrophilic dermatosis that can occur after injuries of varying intensity. Its shape after surgery is a rare disease whose presentation loud and fast pace "infectious" contrasts with the absence of inflammatory lymphangitis or lymphadenopathy. Be aware of prescribing high doses of corticosteroids. These three cases illustrate the importance for early diagnosis and treatment of PG, who can allow spontaneous healing without complex surgery, always feared in this context.

Abstract but mostly he was fed with his aunt's expressed milk and with formula by bottle. At 9 weeks postpartum relactation began. Techniques used were a supplemental nursing support system device; frequent suckle at the breast, supplemented by formula given by bottle; breast pumping; domperidone; and support from an International Board Certified Lactation Consultant and family. Problems that appeared during relactation were that at 12 postpartum weeks the baby refused the supplemental nursing system device, and at 12 weeks and 3 days he refused the breast; after 3 weeks of relactation, the milk supply was still low, needing supplementation; and in the first week of exclusive breastfeeding, the baby stopped growing, then he gained weight slowly, and his gain fell down to the 15(th) percentile. Solutions and interventions used to solve the problems were usage of an artificial nipple during breast strike for 3 days and cessation of supplementary formula and frequent suckling at th...

We present a 24-year-old female referred with non-healing wound of a few days duration on anterior aspect of her right foreleg. Biopsy of the wound was reported to be pyoderma gangrenosum on pathologic report. Further work up of the patient for high grade fever and occasional leukopenias revealed the diagnosis of cyclic neutropenia. Treatment with granulocyte colony-stimulating factor (G-CSF) resulted in patient's neutrophil counts correction and dramatic improvement in healing of her lower extremity wound.

We report a case of the superficial granulomatous (vegetating) form of pyoderma gangrenosum, involving the forehead and the left temporal area in a 44-year-old woman. No association with other pathologies could be found. Lesions responded dramatically to systemic ciclosporine (5 mg/kg/day), and complete healing was reached after 6 months. Doses were tapered progressively. Treatment was discontinued after 4.5 years. Discontinuation was not followed by recurrence of the disease. Healing is maintained after another 4.5 years of follow-up.