Pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that presents with rapidly developing, painful skin ulcers hallmarked by undermined borders and peripheral erythema. Epidemiological studies indicate that the average age of PG onset is in the mid-40s, with an incidence of a few cases per million person-years. PG is often associated with a variety of other immune-mediated diseases, most commonly inflammatory bowel disease and rheumatoid arthritis. The cause of PG is not well understood, but PG is generally considered an autoinflammatory disorder. Studies have focused on the role of T cells, especially at the wound margin; these cells may support the destructive autoinflammatory response by the innate immune system. PG is difficult to diagnose as several differential diagnoses are possible; in addition to clinical examination, laboratory tests of biopsied wound tissue are required for an accurate diagnosis, and new validated diagnostic criteria will facilitate the process. Treatment of PG typically starts with fast-acting immunosuppressive drugs (corticosteroids and/or cyclosporine) to reduce inflammation followed by the addition of more slowly acting immunosuppressive drugs with superior adverse event profiles, including biologics (in particular, anti-tumour necrosis factor (TNF) agents). Appropriate wound care is also essential. Future research should focus on PG-specific outcome measures and PG quality-of-life studies.

ARTERIAL INSUFFICIENCIES: Hyperbaric Oxygen Therapy for Selected Problem Wounds.

The use of hyperbaric oxygen (HBO2) for the treatment of selected problem wounds has focused almost entirely on the diabetic foot ulcer (DFU) in recent years. The prevalence of DFUs in today's patient population and the reimbursement available for the treatment of DFUs have given it priority status in discussions about problem wounds, but there are sound fundamental reasons why additional oxygen may have benefits in the treatment of non-DFU wounds.

Clinical and histological patterns and treatment of pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis for which accurate epidemiological data are limited and therapy remains a challenge. The primary study's aim was to examine all cases of PG observed in our department over a 6-year period in order to describe the relevant characteristics and outcome under therapy. Fourteen patients were included (5 women, 9 men). The average age of our patients was 40,15 years. The classical, ulcerative form was found in 10 cases (71.42%), the pustular form in 4 cases (27.57%) and PG was multifocal in 4 cases. The PG was located preferentially to the lower limbs. Histological examination was realized in all patients and objectified inflammatory infiltrate composed of polymorphonuclear neutrophils in all cases with vasculitis in 4 cases. Six patients (42.85%) had associated disease at diagnosis of PG, including inflammatory bowel disease in two cases (14.28%), a blood disease in 2 cases (14.28%), lymph node tuberculosis and inflammatory arthritis in 1 case (7%). The most frequent first-line treatments were oral corticosteroids (7 cases) and other treatments used were colchicine in 2 cases, topical corticosteroids in 3 cases with good clinical evolution. Our study confirms that PG is a rare disease, associated in almost half of cases with systemic disease already present at diagnosis; in our Moroccan background, it is most often inflammatory bowel disease, hematological or solid cancer and tuberculosis.

PAPA spectrum disorders.

Pyogenic arthritis, pyoderma gangrenosum (PG) and acne (PAPA) syndrome is an autosomal dominant autoinflammatory syndrome due to mutations in proline-serine-threonine phosphatase interacting protein 1 (PSTPIP1) gene and presenting with cutaneous and articular manifestations. Other autoinflammatory syndromes caused by mutations in PSTPIP1 gene or characterized by clinical findings overlapping with those found in PAPA syndrome have been recently included in the group of PAPA spectrum disorders. These disorders are PASH (PG, acne and hidradenitis suppurativa [HS]), PAPASH (PASH associated with pyogenic sterile arthritis), PsAPASH (PASH combined with psoriatic arthritis [PsA], PASS (PG, acne, ankylosing spondylitis, with or without HS), PAC (PG, acne and ulcerative colitis [UC]) and PAMI syndrome (PSTPIP1-associated myeloid-related-proteinemia inflammatory syndrome). Except for PAPA and PAMI, no specific pathogenetic mutations have been identified in these syndromes. Dermatologists should be aware that PG, acne and HS may represent cutaneous signs hiding the presence of these rare entities. Systemic corticosteroids, a number of immunosuppressants and biologics, such as interleukin (IL)-1 antagonists and tumour necrosis factor (TNF) α inhibitors, are nowadays therapy for these diseases. A pathogenesis-driven treatment is the near future in the management of these conditions.

Paraneoplastic Dermatoses: A Brief General Review and an Extensive Analysis of Paraneoplastic Pemphigus and Paraneoplastic Dermatomyositis.

Skin manifestations of systemic disease and malignancy are extremely polymorphous. Clinicians should be familiarized with paraneoplastic dermatoses in order to perform an early diagnosis of the underlying neoplasm. Lack of familiarity with cutaneous clues of internal malignancy may delay diagnosis and treatment of cancer. In this review, we described several paraneoplastic dermatoses and discussed extensively two paradigmatic ones, namely paraneoplastic pemphigus and paraneoplastic dermatomyositis.

The 5 P's of Pyoderma Gangrenosum

The diagnosis of pyoderma gangrenosum (PG) is often difficult to establish based on a clinical presentation, which can mimic other dermatologic conditions. The formation of a mnemonic that incorporates the most prevalent clinical features of PG could aid in accuracy and speed of diagnosis. The 5 P's of PG: Painful, Progressive, Purple, Pretibial, Pathergy, and systemic associations, incorporate parameters recognizable on the first encounter with a patient with PG without reliance on histopathology and laboratory findings or treatment response. We postulate that this simple mnemonic will have the most utility with non-dermatology clinicians encountering a lesion suspicious for PG. By assisting in differential diagnosis formation, this mnemonic may lead to timelier biopsies and treatment initiation. The limitations of this approach mirror those of other studies and include lower sensitivities in patients with an atypical PG presentation. In conclusion, the 5 P's of PG offer a useful mnemonic for the diagnosis of PG, particularly in the initial clinical diagnosis prior to skin biopsy and treatment. J Drugs Dermatol. 2019;18(12):1282-1283.

Case Study on Management of Postsurgical Pyoderma Gangrenosum After Spinal Surgery.

BACKGROUND: Postsurgical pyoderma gangrenosum (PSPG) is a rare autoimmune, neutrophilic dermatosis that results with the occurrence of pyoderma gangrenosum (PG) within surgical incisions. Presenting symptoms include erythema and pain at the surgical incision with wound dehiscence. The clinical appearance of the PSPG wound (similar to PG) shows raised with dusky red or violaceous (violet-colored) wound edges and undermining with little or no evidence of granulation tissue. "Pathergy" is the term used to describe worsening of the wound in response to trauma such as debridement. Postsurgical pyoderma gangrenosum should be suspected in postoperative wounds, which continue to become progressively worse despite broad-spectrum antibiotics, good wound care, and surgical debridement. CASE: A clinical case study of a patient with PSPG from spine surgery is described. CONCLUSION: Postsurgical pyoderma gangrenosum should be suspected in postoperative wounds, which continue to become progressively worse despite broad-spectrum antibiotics, good wound care, and surgical debridement.

A novel topical therapy for resistant and early peristomal pyoderma gangrenosum.

Peristomal pyoderma gangrenosum (PPG) is an under-recognised and difficult condition to treat. We describe a case series using a novel topical combination therapy that promotes wound healing and allows for adhesion of the stoma appliance. A crushed oral prednisolone tablet mixed with Stomahesive Protective Powder (ConvaTec) was applied topically to seven patients with PPG and resulted in pain relief and wound healing in six of seven patients. Only one patient experienced recurrence. The novel topical therapy we describe is cost-effective, readily available, and easily applied in any inpatient or outpatient setting.

Pyoderma gangrenosum successfully treated with golimumab: Case report and review of the literature.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis frequently related to chronic inflammatory bowel disease (IBD) often associated with exacerbation of intestinal disease and/or loss of treatment efficacy. However, in patients with comorbidities, such as diabetes, the diagnosis may be a challenge. Here, we report the case of a 68-year-old man with a history of ulcerative rectocolitis (URC), type II diabetes and arterial hypertension, who had been treated with infliximab and adalimumab in the past. In September 2017, patient developed an erythematous, infiltrated and painful lesion of the third distal part of his left leg, with ulcerative evolution, rapidly worsened despite a broad-spectrum antibiotic treatment had been introducted. A worsening of rectocolitis occurred simultaneously. In agreement with the gastroenterologists, patient started a new biological therapy with golimumab, and oral prednisone with slow tapering of steroid dosage following the improvement of both cutaneous and intestinal symptoms. Dermatologists should be aware about the risk of PG in patient suffering from IBDs, and consider this diagnosis in all patients affected by URC developing rapidly extending ulcerative skin lesion. Moreover, therapeutic choice should take into consideration the effectiveness of golimumab on the inflammatory background, which sustains both intestinal and skin disease in this type of patients.

Is PASH Syndrome a Biofilm Disease?: A Case Series and Review of the Literature.

INTRODUCTION: When occurring together, pyoderma gangrenosum, severe acne, and hidradenitis suppurativa have been described as PASH syndrome. Due to the chronic autoinflammatory state existing in affected patients, PASH syndrome has been attributed to the dysregulation of wound healing. CASE REPORTS: Two cases are presented that demonstrate the paradigmatic clinical features of PASH syndrome and its potential link as an expanding spectrum of bacterial biofilm disorder. CONCLUSIONS: As reported herein, based on biofilm's clinical presentation and resistance to proper wound healing, it could serve as the common denominator and may redirect clinicians' treatment pathways in the near future.

Pyoderma Gangrenosum of the Scalp: A Rare Clinical Variant.

Pyoderma gangrenosum (PG) is a rare, neutrophil-predominant dermatosis that usually presents as a papule or pustule and progresses into a painful ulcer. Clinical and histopathological features are nonspecific, making PG a challenging condition to diagnose. Lesions may occur anywhere on the body; however, the lower extremity is the most common location. Solitary lesions in atypical locations such as the scalp are uncommon, making this clinical variant especially difficult to recognize and diagnose. Although the clinical features and subsequent management of scalp PG might be different from other anatomic sites, the typical presentation and treatment of scalp PG is still unclear. The authors present a recent case of a 34-year-old woman with scalp PG and summarize 16 other cases documented in the literature. This case report and literature review illustrate several similarities and differences between scalp PG and classic PG: (1) scalp PG occurs in a wider age demographic of patients; (2) as with classic PG, inflammatory bowel disease and pregnancy are associated conditions, but head injury and preexisting inflammatory skin conditions of the scalp may be additional predisposing factors for scalp PG; and (3) as with classic PG, scalp PG generally responds well to corticosteroids and immunosuppressive therapy. Scarring occurs in all conditions, though disfigurement and psychosomatic effects may be disproportionately higher in scalp PG.