Presentation
Robles Medicine Clinical and experimental dermatology 2001 An 82-year-old man with a previous history of polycythaemia rubra vera and ischaemic heart disease presented with a discharging sinus of the upper back. [semanticscholar.org]
Classical PG The most common form of PG presents as a rapidly progressive painful ulcer with a violaceous undermined edge (Fig 1). [rcpjournals.org]
In our patient, no lesions were present near the abdominal surgical sites, and there was no antecedent cribriform ulceration. [mdedge.com]
Thus, in conjunction with a clinical presentation of PG, a nonspecific mixed inflammatory infiltrate may suggest a diagnosis of PG. 2. [empendium.com]
We present a case of multiple vegetative pyoderma gangrenosum lesions arising in prior surgical sites in a patient found to have IgA monoclonal gammopathy and abnormal urinary protein electrophoresis. [escholarship.org]
Entire Body System
- Fever
Bone involvement is rare in PG and initially presents as arthralgia, myalgia, and fever, which may progress to sterile osteomyelitis (most common), osteitis, and osteolysis. 5. [empendium.com]
Additional findings sometimes associated with PG include fever, localized tenderness, joint pain (arthralgia), and a general feeling of ill health (malaise). [rarediseases.org]
The appearance of these lesions is often accompanied by systemic symptoms such as fever, malaise and arthralgias. In 25–50% of cases, lesions occur in places of previous minor injury by way of the pathergy phenomenon. [mdpi.com]
In such patients, pustules are painful and occur mainly on the extensor aspects of the extremities and upper trunk.[5] Pustular PG is usually associated with exacerbations of IBD and manifests with fever and arthralgias. [journals.lww.com]
Associated symptoms may be present and include fever, malaise, myalgia, and arthralgia. [dovepress.com]
- Swelling
[…] steroid ointment Tacrolimus ointment Intralesional steroid injections into the ulcer edge Ciclosporin solution Special dressings Oral anti-inflammatory antibiotics such as doxycycline or minocycline If tolerated, careful compression bandaging to reduce swelling [dermnetnz.org]
It presents as eyelid swelling with nodules early on in the disease and can progress to scleritis, proptosis, and conjunctival injection. [empendium.com]
Special inquiry regarding drug intake (especially iodides/bromides, hydroxyurea); exposure to and symptoms of infectious diseases (Box 33-1); symptoms relating to the musculoskeletal system (joint pains, swelling, etc.), the gastrointestinal tract (abdominal [accessmedicine.mhmedical.com]
[…] therefore, immunosuppressors, as well as biological agents, can be risky.18 It is not uncommon for PG to have secondary wound infection, documented by a positive culture from smear, and clinically suspected by CRP elevation and signs such as erythema and swelling [dovepress.com]
- Severe Pain
Pyoderma gangrenosum is diagnosed by its characteristic appearance and severe pain. The pathergy test is usually positive (a skin prick test causing a papule, pustule, or ulcer). [dermnetnz.org]
History A patient with PG usually complains of severe pain that is out of proportion to the clinical appearance of the lesion. [accessmedicine.mhmedical.com]
Introduction PG is a rare inflammatory skin disease with a chronic relapsing course, commonly associated with severe pain and tenderness that often regresses with cribriform mutilating scars.4 PG has been estimated to occur in three to ten individuals [dovepress.com]
- Gangrene
"Tattoo related pyoderma/ectyma gangrenous as presenting feature of relapsed acute myeloid leukaemia: an exceptionally rare observation". Injury. 42 (5): 546–7. doi:10.1016/j.injury.2010.08.014. [en.wikipedia.org]
Bacterial/Cellulitis Fungal (e.g., mucormycosis) Breakdown Suture allergy Mechanical Consider Malignancy Genital Most Likely Infection Bacterial/Viral infection (herpes simplex virus, Epstein–Barr virus, cytomegalovirus) Tuberculosis/Tuberculide Fournier gangrene [accessmedicine.mhmedical.com]
- Asymptomatic
Pulmonary involvement is the most common extracutaneous manifestation of PG and can present asymptomatically or as severe respiratory distress. [empendium.com]
Skin
- Papule
Jerajani Medicine Indian journal of dermatology, venereology and leprology 2007 A 9-year-old female, presented with recurrent bilaterally symmetrically distributed flesh colored vegetative plaques, papules and nodules on trunk and upper and lower extremities [semanticscholar.org]
The pathergy test is usually positive (a skin prick test causing a papule, pustule, or ulcer). The wound should be swabbed and cultured for microorganisms, but these are not the cause of pyoderma gangrenosum. [dermnetnz.org]
[citation needed] Papules that begin as small "spouts" can be treated with Dakin's solution to prevent infection and wound clusters also benefit from this disinfectant. Wet to dry applications of Dakins can defeat spread of interior infection. [en.wikipedia.org]
Synonyms of Pyoderma Gangrenosum PG General Discussion Pyoderma gangrenosum (PG) is an inflammatory skin disorder that is characterized by small, red bumps or blisters (papules or nodules) that eventually erode to form swollen open sores (ulcerations) [rarediseases.org]
Pyoderma gangrenosum lesions usually begin as small, tender papules which progress to large, necrotic and exquisitely painful ulcers. [mdpi.com]
- Blister
Atypical or bullous pyoderma gangrenosum is characterized by superficial blisters (bullae). [rarediseases.org]
This presents as an extremely painful erythematous lesion which rapidly progresses to a blistered or necrotic ulcer. There is often a ragged undermined edge with a violaceous/erythematous border. [rcpjournals.org]
It may start as a small pustule, red bump, or blood-blister, often misinterpreted as an insect bite. The skin then breaks down resulting in an ulcer. The ulcer can deepen and widen rapidly. [dermnetnz.org]
Bullous PG (sometimes called atypical PG) presents as a painful, rapidly expanding superficial inflammatory blister that quickly erodes. [accessmedicine.mhmedical.com]
- Dermatitis
Die durchgeführte ausführliche Diagnostik ergab keinen Anhalt für Systemerkrankungen, und in der Histologie zeigte sich eine chronische granulomatöse Dermatitis. [link.springer.com]
[…] artefacta/Malignancy Pyoderma vegetans SITE SPECIFICa Parastomal Most Likely Dermatoses (extraintestinal Crohn's) Irritant/Allergic contact dermatitis Other (e.g., psoriasis) Infection Bacterial (Staphylococcus/Streptococcus)/Cellulitis Fungal [accessmedicine.mhmedical.com]
Therefore, dermatophyte infections often mimic other dermatologic conditions, including atopic dermatitis, rosacea, psoriasis, bacterial abscess, erythema gyratum repens, lupus, granuloma annulare, cutaneous lymphoma, Hailey-Hailey disease, scarring alopecia [mdedge.com]
Workup
Excluding differential diagnoses is essential in the diagnostic workup for PG. [empendium.com]
Due to these factors, the diagnosis remains challenging and requires a high index of suspicion after the thorough workup of the patient for other causes. 6. [mdpi.com]
Treatment
[…] directed at pyoderm gangrenoum, those in 8 were exacerbated by such treatment, and those in 15 improved with such treatment. [semanticscholar.org]
Cited by Novel use of apremilast for adjunctive treatment of recalcitrant pyoderma gangrenosum. Laird ME, Tong LX, Lo Sicco KI, Kim RH, Meehan SA, Franks AG Jr. Laird ME, et al. [pubmed.ncbi.nlm.nih.gov]
Treatment A gold standard of therapy for PG does not exist, making the treatment of this skin condition extremely challenging. [mdpi.com]
Many patients respond differently to different types of treatment, for example some benefit from a moist environment, so treatment should be carefully evaluated at each stage. [en.wikipedia.org]
Patients typically improve shortly after treatment initiation; however, complete resolution of the ulcer takes several months. Nonpharmacologic Treatment Wound care is essential in PG management. [empendium.com]
Prognosis
Pulmonary manifestations: If treated promptly with systemic glucocorticoids or other immunosuppressants, prognosis of pulmonary involvement in PG is favorable, with no reports of relapse. 2. [empendium.com]
Patients with bullous PG who have an associated hematological disorder also have a poor prognosis. [accessmedicine.mhmedical.com]
Analgesia can be tapered gradually after clinical evidence of improvement, since pain tends to decrease with ulcer healing.26,47 Prognosis and outcome Factors that are considered to be associated with a poor prognosis include disease severity, older age [dovepress.com]
The prognosis for pyoderma gangrenosum is unpredictable. About half of treated patients achieve wound healing on treatment with prednisone or ciclosporin within a year. [dermnetnz.org]
Etiology
Infectious etiologies including dimorphic fungi and blastomycosislike pyoderma generally are not restricted to the head and neck, and tissue culture helps exclude them. [mdedge.com]
INTRODUCTION Pyoderma gangrenosum (PG) is a rare inflammatory disease of unknown etiology characterized by neutrophilic infiltration of the dermis and destruction of the tissue.[1] PG was first described by Brocq in 1916 as “phagedenisme geometrique” [journals.lww.com]
| Print Pyoderma Gangrenosum at a Glance Pyoderma gangrenosum (PG) is a rare inflammatory disease of unknown etiology characterized by sterile neutrophilic infiltration of the skin. Similar neutrophilic infiltrations may occur in other organs. [accessmedicine.mhmedical.com]
However, genetic, autoimmune and autoinflammatory mechanisms have been proposed that could potentially explain the etiology of this ulcerating skin disorder. [mdpi.com]
Last Updated: January 2, 2021 Last Reviewed: June 26, 2021 Chapter Information Definition, Etiology, PathogenesisTop Pyoderma gangrenosum (PG) is a neutrophilic ulcerative dermatosis with a spectrum of clinical presentations and variable clinical course [empendium.com]
Epidemiology
[…] considered PG to be the dissemination of a distant focus of infection (i.e., the bowel in ulcerative colitis or lungs in empyema).[3] Presently PG is considered a reactive inflammatory dermatosis and part of the spectrum of neutrophilic dermatosis.[4] EPIDEMIOLOGY [journals.lww.com]
However, it is difficult to determine the exact disease prevalence, as accurate epidemiological data are missing. [dovepress.com]
Pathophysiology
Pathophysiology The pathophysiology of PG has yet to be fully understood. Several hypotheses exist about the etiology of this ulcerating skin disease. [mdpi.com]
Additionally, the characteristic features of the wound, such as malodor or exudate, may trigger or aggravate anxiety, depression, and social isolation.7 Pathophysiology The etiology of PG is still unknown, and its pathophysiology is far from being completely [dovepress.com]
[…] factor.17 PG associated syndromes have been described and these include PG with cystic acne and hidradenitis suppurativa (PASH),18 PG with pyogenic arthritis and acne (PAPA),19 and PG with pyogenic arthritis, acne and hidradenitis suppurativa (PAPASH).20 Pathophysiology [rcpjournals.org]
Prevention
Significant care should be taken with dressing changes to prevent potentially rapid wound growth. [en.wikipedia.org]
Managing underlying disorders is essential in preventing rebound flares. [empendium.com]
According to some researchers, individuals with a past history of PG should receive preventive (prophylactic) treatment with corticosteroids before undergoing surgery because surgery may cause a recurrence of the disorder. [rarediseases.org]
Prompt and appropriate treatment can stop new lesions from forming and help to slow the progression of existing lesions, thus preventing widespread cribriform scar formation. [mdpi.com]
In these cases, antibiotic treatment should be promptly initiated, and except in sepsis, immunosuppression should be maintained to prevent progression.27 A persistent ulcer does not necessarily mean treatment failure. [dovepress.com]