CONCLUSION: The present case underlines the fact that rare diseases might occasionally present with even more rare symptoms. These occasions can obviously be considered to present a considerable diagnostic challenge. [ncbi.nlm.nih.gov]

Discussion This case showed an atypical and unusual presentation of PG. [karger.com]

Nonperistomal pyoderma can present anywhere on the body, and case 4 (Figures 4A-4C) shows a case of pyoderma presenting on the hands. [woundsresearch.com]

For example, our patient had a longstanding diagnosis of IBS and episodes of diverticulitis; following this presentation, she was diagnosed with IBD. [consultant360.com]

Pathergy sign was present. Skin biopsy showed an aspect of neutrophilic dermatosis. ANA profile revealed positive anti-SSA/Ro and positive anti-SSB/La. [oatext.com]

• Surgical Procedure

  Rutuxamab Topical therapies include gentle local wound care and dressings whereas debridement or other plastic surgical procedures should be deliberately avoided in most cases since they actually provoke and worsen the disorder. [encompasshealthcare.com]

  Given the significant clinical improvement, a new surgical procedure was carried out in order to close the wound (Figure 5). Figure 5. After a new surgical procedure. [rbcp.org.br]

  If these obstructions can’t be removed surgically, in vitro fertilization (IVF) may be needed for conception. IVF is a two-part surgical procedure. [healthline.com]

  Other reported treatment options include local and systemic immunosuppressors (i.e. cyclosporine A and anti–TNF-α), antibiotics, surgical procedures such as skin graftings, and hyperbaric oxygen therapy. [eyewiki.aao.org]

• Malaise

  The patient may also have fever, malaise, or myalgia. Pyoderma lesions are erythematous due to inflammation, but may also have a blue hue from epidermal necrosis. Treatment Immunosuppressive and anti-infective agents are commonly prescribed. [woundeducators.com]

  Patients are often systemically unwell with symptoms such as fever, malaise, arthralgia, and myalgia. Lesions are usually painful and the pain can be severe. When the lesions heal the scars are often cribriform. [bmj.com]

  People commonly have fever and a general feeling of illness (malaise). [msdmanuals.com]

• Infertility

  […] accounts for nearly one-quarter of all cases of infertility. [fertilitypedia.org]

  As indicated, in developed countries, GTB can be seen in young women who present with infertility. [ncbi.nlm.nih.gov]

  This includes tubal abscesses, ectopic pregnancy, and infertility. [healthline.com]

  Sign in Female Genital Tuberculosis. 38 Sharma JB 27365923 2016 19 Pyosalpinx causing acute appendicitis in a 32-year-old Cameroonian female: a case report. 38 Agbor VN...Aminde LN 27461228 2016 20 Magnetic resonance imaging structured reporting in infertility [malacards.org]

  Infertility - the fallopian tube may become deformed or scarred to such an extent that the egg and sperm are unable to meet. After one bout of salpingitis or other PID, a woman's risk of infertility is about 15 per cent. [betterhealth.vic.gov.au]

• Pallor

  On physical examination, splenomegaly and pallor of ocular mucosa were detected. [scielo.br]

  Pallor SA, Sahn EE, Garen PD, Dobson RL, Chadwick EG. Pyoderma gangrenosum in paediatric acquired immunodeficiency syndrome. J Paediatr. 1990; 117 :63–6. [ Google Scholar ] 19. Bhat RM, Shetty SS, Kamath G.H. Pyoderma gangrenosum in childhood. [ncbi.nlm.nih.gov]

  Pallor SA, Sahn EE, Garen PD, Dobson RL, Chadwick EG. Pyoderma gangrenosum in paediatric acquired immunodeficiency syndrome. J Paediatr 1990;117:63-6. 19. Bhat RM, Shetty SS, Kamath G.H. Pyoderma gangrenosum in childhood. [idoj.in]

• Chronic Infection

  Other systemic conditions have also more rarely been associated with PG, including those of chronic infection and inflammation.1,16 Drugs such as propylthiouracil, tyrosine kinase inhibitors, TNFα inhibitors and granulocyte-colony stimulating factor have [rcpjournals.org]

  Long-standing (chronic) infection and/or inflammation can also cause amyloidosis. This is known as secondary or AA amyloidosis. These other kinds of amyloidosis are not covered here. [cancer.org]

• Hypertension

  […] immune-suppression/treatmentApplied immune-suppressionLocalization and dimension (cm)Hospitalization (time)Surgical treatment (preconditioning time)Graft take at discharge/primary outcomeDisease-free follow-up/recurrences163MDiabetes mellitus type 2, arterial hypertension [jaad.org]

  No 80 F Peristomal Ulcerative Colon cancer Hypertension, Guillain-Barre syndrome, diabetes type 2, and osteoarthritis Prednisolone, minocycline No 73 F Peristomal Ulcerative Lung cancer Hypertension, hypercholesterolaemia, and osteoarthritis Prednisolone [hindawi.com]

  Good responses can be achieved with ciclosporin, but side effects including nephrotoxicity, hypertension and an increased risk of cancer have been reported. [woundeducators.com]

  If taken for many months prednisolone may cause side effects including high blood pressure (hypertension) and high blood sugar (diabetes). Long-term oral steroid use can cause other side effects including bone thinning (osteoporosis). [britishskinfoundation.org.uk]

  In patients with coronary artery disease or hypertension its prevalence increases to 25-35%. [elenaconde.com]

• Vascular Disease

  (D) Persisting right lower extremity discoloration due to arterial and venous vascular disease (January 18, 2018). [jaoa.org]

  The diagnosis is made by excluding other causes of similar-appearing cutaneous ulcerations, including infection, malignancy, vasculitis, collagen vascular diseases, diabetes, and trauma. [autoimmune.org]

  Specified criteria (see below) suggest the diagnosis of PG, but other conditions (particularly infection, vascular disease, and malignancy) must be excluded. [accessmedicine.mhmedical.com]

  Several vasculitic and vasculopathic inflammatory conditions can also have similar appearances, including Wegener’s granulomatosis and vasoocclusive disorders such as peripheral vascular disease and cryoglobulinemia. [mdedge.com]

  Essential thrombocytopenia Diabetes type 2, hypertension, hypercholesterolaemia, osteoporosis, and peripheral vascular disease Prednisolone, skin graft No 4. [hindawi.com]

• Eruptions

  […] the skin and subcutaneous tissue, unspecified 2016 2017 2018 2019 Billable/Specific Code suppurative L08.0 Pustular rash L08.0 Pyoderma, pyodermia L08.0 Pyodermatitis L08.0 Rash (toxic) R21 ICD-10-CM Diagnosis Code R21 Rash and other nonspecific skin eruption [icd10data.com]

  One feature is a phenomenon known as pathergy, in which trauma to the skin can cause new eruptions of ulcers in patients with the disease. Types Two variants of the disease exist--the classic/typical and the atypical variants. [woundcarecenters.org]

  The eruption initially appears are a pustule which enlarges and ulcerates with ill defined undermined borders that are bluish or violaceous in color. The surrounding skin also becomes edematous. [radiopaedia.org]

  Even a needle taking blood may trigger a new PG eruption in a patient with the disease. This is called “pathergy.” Patients who have skin ulcers that are painful, rapidly growing, and hard to heal should see a doctor. [jamanetwork.com]

  Vesiculopustular eruption of ulcerative colitis. Arch Dermatol 1983;119: 664-9. [PubMed] [Google Scholar] 7. Callen JP, Woo TY. Vesiculopustular eruption in a patient with ulcerative colitis. Pyoderma gangrenosum. Arch Dermatol 1985;121: 399, 402. [ncbi.nlm.nih.gov]

• Ecthyma

  Infections of the skin and subcutaneous tissue Approximate Synonyms Ecthyma Clinical Information Any purulent skin disease (Dorland, 27th ed). [icd10data.com]

  Pyoderma (Ecthyma) gangrenosum in 2 patients with B2A paraprotein in the blood. Nederl. tijdschr. geneesk. 1965; 109: 1427 PubMed Google Scholar Brunsting L.A. Goeckerman W.H. O'Leary A. [jpeds.com]

  Frequently Asked Questions What’s the difference between ecthyma gangrenosum and pyoderma gangrenosum? Ecthyma gangrenosum is a rare skin infection typically caused by Pseudomonas bacteraemia found in water and soil. [my.clevelandclinic.org]

  These include superficial bacterial infections such as impetigo, impetigo contagiosa, ecthyma, folliculitis, Bockhart's impetigo, furuncle, carbuncle, tropical ulcer, etc.[1][2] Autoimmune conditions include pyoderma gangrenosum. [en.wikipedia.org]

  Several other ulcerating conditions can mimic pyoderma gangrenosum such as: Venous or arterial ulcers Medium and large size vessel vasculitis Occlusive vasculopathy Ecthyma and ecthyma gangrenosum Ulcerating infections e.g. [dermnetnz.org]

• Blister

  Less often, it begins as a blister. The bump or blister then becomes an open, painful sore (ulcer) that expands rapidly. The sores have a raised border that is dusky or purple. The sores can grow together to form larger sores. [msdmanuals.com]

  Atypical or bullous pyoderma gangrenosum: Blisters that typically appear on your hands. This type often appears in those who have leukemia. Pustular pyoderma gangrenosum: Pus-filled blisters (pustules) that appear on your arms and legs. [my.clevelandclinic.org]

  This variant is characterized by relatively shallower ulcers accompanied by vesicles (fluid-filled blisters) or pustules (pus-filled blisters). [woundcarecenters.org]

• Dizziness

  Blood tests are performed weekly to begin with, and patients are asked to report any unusual symptoms (sore throat, dizziness or faintness) urgently to their doctor. Dapsone also causes some patients to have headaches. [britishskinfoundation.org.uk]

  Other common signs and symptoms of multiple myeloma include: nausea weight loss constipation loss of appetite weakness or loss of feeling in your legs swelling in your legs increased thirst frequent urination dizziness confusion pain, especially in your [healthline.com]

In both cases, a workup for systemic diseases associated with PG yielded negative results and corticosteroids were initiated by subcutaneous injection into the ulcer and/or systemic delivery. [jcm.asm.org]

A complete clinical workup is mandatory. Table II. [dermatologyadvisor.com]

(See Presentation, DDx, and Workup.) Patients with pyoderma gangrenosum may have involvement of other organ systems that manifests as sterile neutrophilic infiltrates. [emedicine.medscape.com]

Cutaneous ulcerations in patients with suspected pyoderma gangrenosum often prove, on further workup, to have a different cause. [nejm.org]

• Pulmonary Infiltrate

  […] with pulmonary infiltrates that proved refractory to antibiotics. [mayoclinicproceedings.org]

  Cavitating pulmonary infiltrate in an adolescent with pyoderma gangrenosum: a rarely recognized extracutaneous manifestation of a neutrophilic dermatosis. J Am Acad Dermatol. 2000 Jul. 43(1 Pt 1):108-12. [Medline]. Ayyala RS, Armstrong S. [emedicine.medscape.com]

  Cavitating pulmonary infiltrate in an adolescent with pyoderma gangrenosum: A rarely recognized extracutaneous manifestation of a neutrophilic dermatosis. [scielo.br]

• Multiple Ulcerations

  There is usually a single large ulcer. Occasionally there may be multiple ulcers. Ulcers may become infected, oozing fluid or pus. Pain or discomfort from the ulcer is common. Pyoderma gangrenosum is not a skin cancer and does not lead to cancer. [britishskinfoundation.org.uk]

  […] of papule, pustule or vesicle that rapidly ulcerated Clinical examination (or photographic evidence) of Peripheral erythema, undermining border, and tenderness at site of ulceration Multiple ulcerations (at least 1 occurring on the lower leg) Cribriform [clinicaltrials.gov]

  History of papule, pustule, or vesicle that rapidly ulcerated Clinical examination (or photographic evidence) of peripheral erythema, undermining border, and tenderness at site of ulceration Multiple ulcerations (at least 1 occurring on an anterior lower [en.wikipedia.org]

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]

Using a standardized questionnaire, a German survey on the treatment of PG among wound experts revealed that 11 % of PG patients exclusively received topical treatment; 22.7 %, only systemic treatment; and 66.2 %, combined topical and systemic therapy [onlinelibrary.wiley.com]

[…] ulcers and number of treatment side effects. [discover.dc.nihr.ac.uk]

Despite recent advances in treatment, the prognosis of PG remains unpredictable.1 The optimal treatment of PG includes a combination of local wound care and systemic treatment. [ard.bmj.com]

Treatment Treatment of pyoderma gangrenosum is aimed at reducing inflammation, controlling pain, promoting wound healing and controlling any underlying disease. [mayoclinic.org]

Prognosis Despite recent advances in therapy, the prognosis of PG remains unpredictable. The documents contained in this web site are presented for information purposes only. [orpha.net]

There are some factors which can affect the prognosis of PG. Reichrath et al. suggested that the type and severity of the associated systemic disease can affect the prognosis of PG [8]. [hindawi.com]

It is most commonly used to predict prognosis. Higher blood levels of LDH indicate a poorer prognosis. [cancer.net]

Prevention, and Complications Prognosis chronic, relapsing ulcers leave atrophic scars when healed Please rate topic. [medbullets.com]

Etiology The etiology has not yet been clearly determined. [orpha.net]

The etiology of pyoderma is unclear. Half of patients have a systemic inflammatory condition such as ulcerative colitis, Crohn’s disease, irritable- bowel syndrome, lupus, or rheumatoid arthritis. [woundeducators.com]

Favorite Table | 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. [accessmedicine.mhmedical.com]

These similarities highlight the need for a tissue diagnosis to identify infectious etiologies and rule out malignancy when BLP is suspected. J Drugs Dermatol. 2018;17(2):233-235. [ncbi.nlm.nih.gov]

Given its low incidence, few studies have addressed its epidemiology or treatment. [actasdermo.org]

Summary Epidemiology The exact prevalence of PG is unknown. The incidence has been estimated to range between 1 and 3.3 in 330,000. The incidence peak occurs between the ages of 20 to 50 years, with women being more often affected than men. [orpha.net]

Its pathophysiological mechanisms remain controversial and many other comorbidities have been reported in the literature, including endocrinological, cardiovascular and metabolic, neoplastic and autoimmune comorbidities. [clinicaltrials.gov]

RESULTS Pathophysiology The pathophysiology of PG is yet to be fully elucidated, but it is most likely a complex reaction pattern with convergent pathways. [nursingcenter.com]

Etiology Pathophysiology The etiology of PG is unknown, and its pathogenesis is poorly understood. [dermatologyadvisor.com]

Alavi A, French LE, Davis MD, et al: Pyoderma gangrenosum: An update on pathophysiology, diagnosis and treatment. [merckmanuals.com]

Prevention There are no current guidelines to prevent pyoderma gangrenosum. References Charles CA, Leon A, Banta MR, Kirsner RS. Etanercept for the treatment of refractory pyoderma gangrenosum: a brief series. Int J Dermatol. 2007;46(10):1095-1099. [lahey.org]

Prevention There are no current guidelines to prevent pyoderma gangrenosum. [winchesterhospital.org]

Prevention How can I prevent pyoderma gangrenosum? Medical experts aren’t sure exactly what causes pyoderma gangrenosum, so there may be no way to prevent it. Pyoderma gangrenosum can advance quickly and leave permanent scars. [my.clevelandclinic.org]

Take precaution to prevent injuries when possible. Wear proper safety gear and avoid high impact or full contact activities. See your doctor as soon as you notice a possible ulcer. Prevention There is no known way to prevent pyoderma gangrenosum. [uvahealth.com]

CONCLUSION: Recognition of this rare pathologic association is essential, to prevent delays in diagnosis and treatment that can lead to major craniofacial tissue loss. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V. [ncbi.nlm.nih.gov]