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Archives of Dermatological Research Jun 2024Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) syndrome is a rare condition characterized by clinical features of all three dermatologic conditions. The...
BACKGROUND
Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) syndrome is a rare condition characterized by clinical features of all three dermatologic conditions. The management of PASH syndrome is difficult, with no consensus on treatment guidelines. Since PASH syndrome can increase morbidity and adversely impact quality of life, better characterization of effective therapies is needed.
METHODS
A retrospective cohort study was conducted to identify all patients with pyoderma gangrenosum (PG) treated at The Ohio State University Wexner Medical Center between 2015 and 2021. PG diagnosis was confirmed via PARACELSUS score. Subsequent chart review identified eight patients with concomitant hidradenitis suppurativa (HS) and acne who were clinically diagnosed with PASH syndrome.
RESULTS
Eight patients were clinically diagnosed with PASH syndrome based on their clinical presentation at our institution. Seven patients had failed some type of medical therapy prior to presentation, including topical corticosteroids, oral corticosteroids, oral antibiotics, and biologics. One patient had also tried surgical drainage at an outside institution. Six patients were effectively treated with biologics, usually in combination with other therapies. One patient experienced improvement of her skin lesions after diagnosis and treatment of her underlying hematologic malignancy.
CONCLUSIONS
Medical management with biologics in combination with corticosteroids and/or antibiotics was effective in the management of most patients. Diagnosis and treatment of an underlying condition should be prioritized in refractory cases. If workup is negative, surgical management may be considered. Further investigation with a greater number of patients is required to develop management guidelines for PASH syndrome.
Topics: Humans; Pyoderma Gangrenosum; Female; Retrospective Studies; Acne Vulgaris; Hidradenitis Suppurativa; Adult; Male; Anti-Bacterial Agents; Young Adult; Middle Aged; Biological Products; Treatment Outcome; Quality of Life; Syndrome; Adolescent; Adrenal Cortex Hormones
PubMed: 38878169
DOI: 10.1007/s00403-024-03125-7 -
Wounds : a Compendium of Clinical... May 2022Atypical wounds account for approximately 5% to 20% of chronic ulcerations. Typically, clinical suspicion of an uncommon etiology is warranted for wounds that do not... (Review)
Review
Atypical wounds account for approximately 5% to 20% of chronic ulcerations. Typically, clinical suspicion of an uncommon etiology is warranted for wounds that do not show signs of healing with conventional care, that are associated with pain out of proportion to the clinical presentation, or that are atypical in appearance. This review provides a general overview of various atypical wound etiologies, clinical presentations and appearance, and current treatment protocols. The clinical presentation, pathophysiologic etiology, and current literature on each etiology are presented. The etiologies discussed are pyoderma gangrenosum, calciphylaxis, lichen planus, necrobiosis lipoidica, infectious ulcers, hidradenitis suppurativa, artefactual ulcers, hydroxyurea-induced ulcers, vasculopathies, and neoplastic ulcers. Patients with atypical wounds experience a poorer prognosis and slower healing rate compared with patients with typical wound etiologies (eg, vascular and diabetic wounds). Biopsy is often vital in wound care to identify and differentiate wound etiologies. It is important to note that multiple characteristics or histologic features can overlap in a biopsy with atypical wounds. Therefore, a biopsy will still require an understanding of the presentation of these different wounds and should only be used when appropriate. The proper diagnosis for an atypical wound can greatly hasten wound closure, decrease the cost for the patient and the health care system, and improve the patient's quality of life. Because of the limited availability of patient populations with atypical wound etiologies, literature concerning specific pathologies is limited. More research on each pathology is needed, as is a universally accepted treatment protocol for atypical wounds.
Topics: Calciphylaxis; Humans; Pyoderma Gangrenosum; Quality of Life; Ulcer; Wound Healing
PubMed: 35839157
DOI: 10.25270/wnds/2022.124134 -
Wounds : a Compendium of Clinical... Jun 2019Pyoderma gangrenosum (PG) is a rare, ulcerative inflammatory skin disease that most commonly occurs in patients with inflammatory bowel disease, rheumatologic diseases,... (Review)
Review
Pyoderma gangrenosum (PG) is a rare, ulcerative inflammatory skin disease that most commonly occurs in patients with inflammatory bowel disease, rheumatologic diseases, or hematologic diseases. Successful treatment of PG often requires immunosuppression and appropriate wound care. Systemic corticosteroids and cyclosporine are the first-line treatments for PG. However, chronic use of these systemic agents places patients at risk for developing significant side effects, including hyperglycemia, osteoporosis, hypertension, and weight gain. Furthermore, when treating small or superficial PG ulcers, the use of local agents as monotherapies or adjuvant treatments can be ideal to control inflammation and promote healing without placing the patient at risk for many severe side effects that can be seen with long-term use of systemic agents. This literature review assesses all available local therapies in order to summarize the use and reported efficaciousness of the broad range of local treatments available for PG.
Topics: Administration, Topical; Female; Humans; Immunosuppressive Agents; Injections, Intralesional; Male; Prognosis; Pyoderma Gangrenosum; Risk Assessment; Severity of Illness Index; Treatment Outcome
PubMed: 31215868
DOI: No ID Found -
Actas Dermo-sifiliograficas Dec 2017
Review
Topics: Aged; Female; Follow-Up Studies; Humans; Immunosuppressive Agents; Myeloproliferative Disorders; Prednisone; Pyoderma Gangrenosum; Ultrasonography; Ultrasonography, Doppler, Color
PubMed: 28705518
DOI: 10.1016/j.ad.2017.02.025 -
Journal of Medical Case Reports Jan 2020Pyoderma gangrenosum is a rare ulcerating skin disease of unknown etiology, making its coincidence with orthopedic trauma a rare challenge. Patients are at risk of... (Review)
Review
BACKGROUND
Pyoderma gangrenosum is a rare ulcerating skin disease of unknown etiology, making its coincidence with orthopedic trauma a rare challenge. Patients are at risk of progression of the existing lesions and development of new lesions upon skin injury when surgical procedures are performed. To our knowledge, this is the first report in the literature of disease unrelated surgery during active pyoderma gangrenosum.
CASE PRESENTATION
We present a case of femoral neck fracture in a Caucasian patient with concurrent pyoderma gangrenosum localized in the axilla. Hemiarthroplasty was safely performed after disease activity was reduced with systemic corticosteroids. Tissue-protective wound closure was used together with perioperative corticosteroids and antibiotics. No signs of pyoderma gangrenosum developed at the surgical wound site, and the axillary lesions showed constant improvement until healing with scar tissue.
CONCLUSIONS
In our patient, the preoperative steroid treatment, perioperative antibiotics, and soft tissue protective surgical technique led to successful management of this rare coincidence.
Topics: Female; Femoral Neck Fractures; Hemiarthroplasty; Humans; Middle Aged; Perioperative Care; Pyoderma Gangrenosum; Wound Healing
PubMed: 31931877
DOI: 10.1186/s13256-019-2329-8 -
Dermatology Online Journal Feb 2006We present a case of penile pyoderma gangrenosum (PG) that responded dramatically to an 8-week course of prednisone and has not recurred over a 6-month period. Although...
We present a case of penile pyoderma gangrenosum (PG) that responded dramatically to an 8-week course of prednisone and has not recurred over a 6-month period. Although quite uncommon, penile PG should be a diagnostic consideration in any patient with non-healing ulcerative lesions of the penis. A correct diagnosis in this situation precludes unnecessary or harmful therapeutic interventions and leads to proper management.
Topics: Anti-Bacterial Agents; Anti-Inflammatory Agents; Diabetes Mellitus, Type 2; Humans; Male; Middle Aged; Penile Diseases; Prednisone; Pyoderma Gangrenosum
PubMed: 16638401
DOI: No ID Found -
The Turkish Journal of Pediatrics 2024Takayasu arteritis (TA) is an uncommon chronic inflammatory and autoimmune disease primarily affecting large vessels, particularly the aorta and its branches. Skin...
BACKGROUND
Takayasu arteritis (TA) is an uncommon chronic inflammatory and autoimmune disease primarily affecting large vessels, particularly the aorta and its branches. Skin manifestations have been documented in association with TA. Pyoderma gangrenosum (PG) is a chronic neutrophilic dermatosis characterized by destructive, necrotizing, and painful ulcers, predominantly found on the lower extremities. The coexistence of PG and TA is extremely rare, with most reported cases involving adult patients. Interestingly, the association between PG and TA appears to be more common in Japan compared to North American and European populations. Childhood TA (c-TA) accompanied by PG is exceptionally rare, with only 10 cases reported in the literature thus far.
CASE REPORT
We present the case of a 7-month-old patient initially diagnosed with PG. Despite aggressive immunosuppressive therapy, the patient`s high acute phase reactants remained elevated. Although the abdominal ultrasound was normal, advanced imaging was performed due to severe abdominal pain. Contrastenhanced computerized tomography angiography of the aorta and its branches revealed extensive vascular involvement consistent with TA.
CONCLUSION
In this report, we highlight an infantile case of PG that was subsequently diagnosed as infantile TA. Recognizing the rare association between PG and TA is important. Thorough evaluation and prompt diagnosis of TA in infants with PG can guide further investigations and prevent vascular complications.
Topics: Adult; Infant; Humans; Child; Pyoderma Gangrenosum; Takayasu Arteritis; Inflammation; Ultrasonography; Aorta
PubMed: 38523387
DOI: 10.24953/turkjped.2023.714 -
Thorax Apr 2000
Topics: Antibodies, Antineutrophil Cytoplasmic; Biomarkers; Diagnosis, Differential; Granulomatosis with Polyangiitis; Humans; Middle Aged; Pyoderma Gangrenosum
PubMed: 10766647
DOI: 10.1136/thorax.55.4.345d -
The Korean Journal of Gastroenterology... May 2019Inflammatory bowel disease (IBD) is a chronic inflammatory disease of the gastrointestinal tract with an unknown etiology and pathogenesis. The incidence and prevalence... (Review)
Review
Inflammatory bowel disease (IBD) is a chronic inflammatory disease of the gastrointestinal tract with an unknown etiology and pathogenesis. The incidence and prevalence of IBD are increasing rapidly in Korea. Approximately one-third of patients with IBD appear to develop extra-intestinal manifestations with the skin being one of the most commonly affected organs. They may precede, occur simultaneously, or follow the diagnosis of IBD. In addition, they may parallel with the luminal symptoms or independent from the disease activity of IBD. This review outlines the skin manifestations associated with IBD and discusses their management. Skin manifestations should be managed in close collaboration with a dermatologist.
Topics: Erythema; Humans; Immunosuppressive Agents; Inflammatory Bowel Diseases; Periodontitis; Psoriasis; Pyoderma Gangrenosum; Skin Diseases; Steroids; Sweet Syndrome
PubMed: 31132835
DOI: 10.4166/kjg.2019.73.5.285 -
International Wound Journal Dec 2022
Topics: Humans; Pyoderma; Pyoderma Gangrenosum
PubMed: 35560865
DOI: 10.1111/iwj.13828