-
Dermatology Online Journal Mar 2021Pyoderma gangrenosum is characteristically associated with inflammatory bowel disease. However, the association between this neutrophilic dermatosis and diverticular... (Review)
Review
Pyoderma gangrenosum is characteristically associated with inflammatory bowel disease. However, the association between this neutrophilic dermatosis and diverticular disease is scarcely mentioned in the literature. Diverticulitis should be included in the differential diagnosis in patients with pyoderma gangrenosum and gastrointestinal complaints, or even in asymptomatic patients, particularly in the elderly. Misdiagnosis can lead to inadequate treatments and serious complications.
Topics: Aged, 80 and over; Anti-Bacterial Agents; Clindamycin; Diagnosis, Differential; Diverticulitis, Colonic; Drug Therapy, Combination; Female; Glucocorticoids; Humans; Metronidazole; Prednisone; Pyoderma Gangrenosum
PubMed: 33865277
DOI: No ID Found -
Arthritis & Rheumatology (Hoboken, N.J.) Feb 2022Dominantly inherited PSTPIP1 mutations cause a spectrum of autoinflammatory manifestations epitomized by PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum,...
OBJECTIVE
Dominantly inherited PSTPIP1 mutations cause a spectrum of autoinflammatory manifestations epitomized by PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome.). The connections between PSTPIP1 and PAPA syndrome are poorly understood, although evidence suggests involvement of pyrin inflammasome activation. Interleukin-18 (IL-18) is an inflammasome-activated cytokine associated with susceptibility to macrophage activation syndrome (MAS). This study was undertaken to investigate an association of IL-18 with PAPA syndrome.
METHODS
Clinical and genetic data and serum samples were obtained from patients referred to institutions due to symptoms indicative of PAPA syndrome. Serum IL-18, IL-18 binding protein (IL-18BP), and CXCL9 levels were assessed by bead-based assay, and free IL-18 levels were assessed by enzyme-linked immunosorbent assay.
RESULTS
The symptoms of PSTPIP1-positive patients with PAPA syndrome overlapped with those of mutation-negative patients with PAPA-like conditions, but mutation-positive patients had earlier onset and a greater proportion had a history of arthritis. We found uniform elevation of total serum IL-18 in treated PAPA syndrome patients at levels nearly as high as those seen in NLRC4-associated autoinflammation with infantile enterocolitis patients, and well above levels found in most familial Mediterranean fever patients. Serum IL-18 elevation in PAPA syndrome patients persisted despite fluctuations in disease activity. Levels of the soluble IL-18 antagonist IL-18BP were modestly elevated, and PAPA syndrome patients had detectable free IL-18. PAPA syndrome was rarely associated with elevation of CXCL9, an indicator of interferon-γ activity, but no PAPA syndrome patients had a history of MAS.
CONCLUSION
PAPA syndrome is a refractory and often disabling monogenic autoinflammatory disease associated with chronic and unopposed elevation of serum IL-18 levels but not with risk of MAS. These findings affect our understanding of the diseases in which IL-18 is overproduced and suggest a link between pyrin inflammasome activation, IL-18, and autoinflammation, without susceptibility to MAS.
Topics: Acne Vulgaris; Adaptor Proteins, Signal Transducing; Adolescent; Adult; Arthritis, Infectious; Child; Child, Preschool; Cytoskeletal Proteins; Female; Humans; Infant; Interleukin-18; Male; Middle Aged; Mutation; Pyoderma Gangrenosum; Retrospective Studies; Young Adult
PubMed: 34492165
DOI: 10.1002/art.41976 -
Postgraduate Medical Journal Feb 1997Pyoderma gangrenosum and Sweet's syndrome are classified as neutrophilic dermatoses as they exhibit intense dermal inflammatory infiltrates composed of neutrophils with... (Review)
Review
Pyoderma gangrenosum and Sweet's syndrome are classified as neutrophilic dermatoses as they exhibit intense dermal inflammatory infiltrates composed of neutrophils with little evidence of a primary vasculitis. They share several characteristics and respond to immunosuppressives. Aetiology is felt to represent a manifestation of altered immunologic reactivity. Patients with both conditions concurrently have been described. Diagnosis is based on clinical and histopathological findings. However, clinically the typical forms of the two conditions are quite distinct: pyoderma showing cutaneous ulceration with a purple undermined border and Sweet's syndrome having tender, erythematous, nonulcerated plaques and nodules. Approximately 50% of cases of pyoderma are associated with a specific systemic disorder. These include inflammatory bowel disease, rheumatoid arthritis, non-Hodgkin's lymphoma and myeloproliferative disorders. Many associations with Sweet's syndrome have been described, including acute myeloid leukaemia, myeloma and adenocarcinomas, and haematological malignancy. There is overlap between the two conditions with lesions categorised as Sweet's syndrome being clinically more characteristic of atypical pyoderma and vice versa. We believe that pyoderma and Sweet's syndrome represent a continuum of spectrum of disease. The reason for the clinical differences between the conditions is unclear and merits further investigation but may be explained by varying levels of intensity and extent of the inflammatory process. This review will describe the pathogenesis, clinical features, diagnosis, associations and treatment of the two conditions.
Topics: Aged; Diagnosis, Differential; Female; Humans; Male; Pyoderma Gangrenosum; Sweet Syndrome
PubMed: 9122099
DOI: 10.1136/pgmj.73.856.65 -
International Journal of Oral Science Jun 2010Pyoderma gangrenosum (PG) is a rare, noninfectious neutrophilic dermatosis. Clinically, it begins with sterile pustules that rapidly progress into painful ulcers of... (Review)
Review
Pyoderma gangrenosum (PG) is a rare, noninfectious neutrophilic dermatosis. Clinically, it begins with sterile pustules that rapidly progress into painful ulcers of variable depth and size with undermined violaceous borders. The diagnosis of PG is based on the history of an underlying disease, a typical clinical presentation, histopathology, and exclusion of other diseases. The peak incidence occurs between the ages of 20 to 50 years with women being more often affected than men. There have been very few reports of pyoderma gangrenosum with oral mucosal involvement. Oral lesions in previously reported cases have included ulcers of varying sizes from a few mm to several cm and have been reported to have been found on the tongue, soft and hard palate, buccal mucosa, and gingiva. Some of these oral lesions have been associated with ulcerative colitis, inflammatory bowel disease, and polycythemia rubra vera. A few cases were reported with biopsy findings, the histological picture being nonspecific, showing ulceration, and necrosis with inflammatory cell infiltrate. A peculiar case of pyoderma gangrenosum with an oral lesion is presented here, and the differential diagnosis is discussed.
Topics: Adult; Anti-Infective Agents; Chlorhexidine; Dapsone; Drug Therapy, Combination; Female; Glucocorticoids; Humans; Metronidazole; Oral Ulcer; Prednisolone; Pyoderma Gangrenosum; Tooth Mobility
PubMed: 20737938
DOI: 10.4248/IJOS10032 -
Indian Journal of Dermatology,... 2017
Review
Topics: Administration, Topical; Dermatologic Agents; Diagnosis, Differential; Humans; Pyoderma Gangrenosum; Skin Diseases
PubMed: 27917844
DOI: 10.4103/0378-6323.195077 -
Medicina 2022
Topics: Humans; Pyoderma Gangrenosum
PubMed: 35037878
DOI: No ID Found -
Journal of the European Academy of... Aug 2022
Topics: COVID-19; COVID-19 Vaccines; Hemorrhage; Humans; Pyoderma Gangrenosum; Skin Diseases, Vesiculobullous; Vaccination
PubMed: 35398933
DOI: 10.1111/jdv.18132 -
Dermatology Online Journal Dec 2017PASH syndrome (pyoderma gangrenosum, acne, and suppurative hidradenitis) forms part of the spectrum of autoinflammatory diseases. We report an unusual case of PASH... (Review)
Review
PASH syndrome (pyoderma gangrenosum, acne, and suppurative hidradenitis) forms part of the spectrum of autoinflammatory diseases. We report an unusual case of PASH syndrome in a patient with end-stagerenal disease (ESRD) who was successfully treated with the tumor necrosis factor inhibitor, adalimumab. The case underscores the challenges associatedwith the treatment of PASH syndrome as well as the ongoing search to establish a genetic basis for the syndrome. Renal impairment has been reported in association with pyoderma gangrenosum but has notbeen described in PASH syndrome. We believe this to be the first reported case of a patient who developed PASH syndrome in the setting of ESRD.
Topics: Acne Vulgaris; Adalimumab; Adult; Anti-Inflammatory Agents; Hidradenitis Suppurativa; Humans; Kidney Failure, Chronic; Male; Pyoderma Gangrenosum; Syndrome; Tumor Necrosis Factor-alpha
PubMed: 29447652
DOI: No ID Found -
Orthopaedics & Traumatology, Surgery &... Jun 2017A case of post-traumatic lower-limb pyoderma gangrenosum (PG) in a 77-year-old female is reported. The diagnosis of PG is frequently one of exclusion, and it is...
A case of post-traumatic lower-limb pyoderma gangrenosum (PG) in a 77-year-old female is reported. The diagnosis of PG is frequently one of exclusion, and it is therefore unsurprising that the condition was initially mistaken for necrotising fasciitis then for necrotising bacterial dermo-hypodermitis. Medical and surgical treatment for those conditions proved ineffective. This fact, together with the atypical presentation, promoted a re-evaluation of the diagnosis. The clinical findings and investigation results converged to suggest PG, and a therapeutic trial was initiated. The good treatment response and negative findings from tests for other conditions established the diagnosis of post-traumatic PG.
Topics: Accidents, Traffic; Aged; Amoxicillin; Anti-Bacterial Agents; Clavulanic Acid; Diagnosis, Differential; Fasciitis, Necrotizing; Female; Humans; Leg Injuries; Lower Extremity; Pyoderma Gangrenosum; Plastic Surgery Procedures
PubMed: 28286096
DOI: 10.1016/j.otsr.2017.01.009 -
Revista Espanola de Enfermedades... Dec 2022A 41-years-old female, with ulcerative colitis, presented to the emergency department with 7-days history of abdominal pain, bloody stools (> 10/day). The patient...
A 41-years-old female, with ulcerative colitis, presented to the emergency department with 7-days history of abdominal pain, bloody stools (> 10/day). The patient referred the appearance of a cutaneous lesion, on her left thigh, with subsequent appearance of similar lesions on the lower limbs. No improvement after amoxicillin/clavulanic acid. On admission, she was febrile (38.2 ºC) and tachycardic. She had three cutaneous lesions, the largest one with 8cm in the left thigh - a deep and painful lesion, with extensive ulceration, necrosis, exudative edges and with marked pathergia, compatible with pyoderma gangrenosum.
Topics: Adult; Female; Humans; Abdominal Pain; Amoxicillin-Potassium Clavulanate Combination; Colitis, Ulcerative; Pyoderma Gangrenosum
PubMed: 36043550
DOI: 10.17235/reed.2022.9120/2022