Pyoderma gangrenosum in a patient with essential thrombocythemia

BACKGROUND: Pyoderma gangrenosum is an uncommon ulcerative condition associated with inflammatory bowel disease, arthritis, and hematologic disease. We report a patient with essential thrombocythemia and pyoderma gangrenosum. OBJECTIVE: This article is a review of the associations between pyoderma gangrenosum and other diseases. RESULTS: There have been two previous reports of patients with pyoderma gangrenosum and essential thrombocythemia. CONCLUSION: There may be a possible association between pyoderma gangrenosum and essential thrombocythemia. The diagnosis of pyoderma gangrenosum should be considered in patients with essential thrombocythemia and cutaneous ulcers.     

Pyoderma gangrenosum over a saphenectomy scar in a cANCA-positive patient with diverticulitis

Pyoderma gangrenosum is a chronic, painful, ulcerative skin disease of unknown etiology. In 50 % of the cases, it is associated with systemic disease, primarily with inflammatory bowel disease. It can develop over areas of trauma or surgical wounds, possibly because of local immunoreactant deposits. We describe the case of a cANCA-positive patient with colonic diverticulitis who developed pyoderma gangrenosum over a saphenectomy scar.     

Pyoderma gangrenosum: a clinical manifestation of difficult diagnosis

Pyoderma gangrenosum is an uncommon ulcerative cutaneous dermatosis associated with a variety of systemic diseases including inflammatory bowel disease, arthritis, hematological malignancies, hepatitis and acquired immunodeficiency syndrome (AIDS). The pathogenesis of pyoderma gangrenosum remains unknown. Its diagnosis is usually based on clinical evidence and confirmed through a process of elimination of the other possible causes of cutaneous ulcers. This report describes a case of pyoderma gangrenosum with extensive ulceration that responded well to treatment.     

Familial ulcerative pyoderma gangrenosum: a report of 2 kindred

Pyoderma gangrenosum is a rare, chronic ulcerative skin disease. It is a diagnosis of exclusion, after ruling out other causes of cutaneous ulceration. The etiology of pyoderma gangrenosum is poorly understood but is likely multifactorial. We describe 2 families affected by ulcerative pyoderma gangrenosum. This familial clustering suggests a possible genetic role in the development of pyoderma gangrenosum in some cases.     

Pyoderma gangrenosum involving the head and neck

In six patients with pyoderma gangrenosum, the head and neck region was a major site of ulcerative skin disease. In two patients, the disease was limited to this anatomic site. Corticosteroids were effective therapy in five cases. In one case, occurring in association with ulcerative colitis, total proctocolectomy was required to control ulcerative scalp disease. Detailed histologic examination of a primary lesion in one case with 0.5-micron sections demonstrated morphologic evidence of mast cell activation, suggesting that mast cells may contribute to the pathogenesis of the inflammatory process in pyoderma gangrenosum.     

Penile pyoderma gangrenosum: successful treatment with colchicine

Pyoderma gangrenosum is an ulcerative skin disease with variable clinical outcomes. The diagnosis is based on clinical features and exclusion of other ulcerative diseases. To date, a specific treatment is not known. Since the disease can be destructive, aggressive treatment is preferable. Here, we present a patient with a penile pyoderma gangrenosum who was successfully treated with low-dose colchicine.     

Treatment of pyoderma gangrenosum with cyclosporine.

BACKGROUND AND DESIGN--Pyoderma gangrenosum is a chronic inflammatory ulcerative skin disease of unknown origin, often associated with various diseases including inflammatory bowel disease, inflammatory arthritis, monoclonal gammopathies, hepatitis, and myeloproliferative disorders. Treatment of associated systemic disorders may improve the ulcers, but lesions may be recalcitrant and persist for months to years. Therapy for pyoderma gangrenosum includes high-dose systemic corticosteroids, sulfa drugs such as sulfasalazine, clofazimine, and immunosuppressive agents such as mercaptopurine and azathioprine; these drugs are sometimes ineffective. RESULTS--We present a series of 11 patients with pyoderma gangrenosum, with a wide range of underlying diseases, whose ulcers were refractory to usual therapy and who were treated with low-dose cyclosporine. Ten of the 11 patients cleared rapidly and completely with cyclosporine therapy. CONCLUSIONS--Cyclosporine should be seriously considered as a primary form of treatment for pyoderma gangrenosum.     

Pustular pyoderma gangrenosum: an uncommon variant which is easily misdiagnosed

Pustular pyoderma gangrenosum is a relatively uncommon clinical form of pyoderma gangrenosum; it presents with vesiculo-pustular lesions that do not develop into frank ulceration. We report a case of a 44-year-old man with associated ulcerative colitis, who was misdiagnosed as having necrotizing fasciitis. He underwent multiple debridements and a subsequent skin grafting procedure, but without improvement. The diagnosis of pyoderma gangrenosum is often challenging because there is no defining diagnostic clinical, laboratory, or histopathological feature. A high index of suspicion is, therefore, essential to diagnose pyoderma gangrenosum clinically because failure to do so in the early stages of the disease can lead to disfigurement and even unnecessary and detrimental surgery.     

Genital pyoderma gangrenosum: Report of two cases and published work review of Japanese cases

Pyoderma gangrenosum is an ulcerative skin disorder showing characteristic non‐infectious ulcers and affects the lower extremities in approximately 70% of cases. Pyoderma gangrenosum is commonly associated with systemic diseases such as inflammatory bowel disease, rheumatoid arthritis and hematological malignancies. Herein, we report two cases of Japanese patients diagnosed with genital pyoderma gangrenosum. Case 1 was a 74‐year‐old woman without associated systemic complications, whose skin lesion resembled a squamous cell carcinoma and was limited to the vulva. Case 2 is an 89‐year‐old man, who suffered from myelodysplastic syndrome and acute myeloid leukemia, and presented with penile and leg ulcers mimicking pressure sores. Both cases responded well to systemic steroids. We review 13 genital pyoderma gangrenosum cases (76.9% male; aged 30–89 years) from 1996 to 2012 in Japan, including 11 previously reported cases and the present study's two cases. Four of the 13 genital pyoderma gangrenosum cases had associated systemic diseases and their skin lesions spread to the extragenital areas. Eight of the remaining nine genitalia‐localized pyoderma gangrenosum cases had no associated systemic diseases. In conclusion, genital pyoderma gangrenosum is rare and may be misdiagnosed. It should therefore be considered in cases of refractory genital ulcers. In addition, genitalia‐localized pyoderma gangrenosum tends to be without systemic complications.     

Management of pyoderma gangrenosum--an update

Pyoderma gangrenosum is a neutrophilic dermatosis with distinctive clinical manifestations. It is frequently associated with systemic diseases like inflammatory bowel disease, rheumatoid arthritis and myeloproliferative diseases. The etiopathogenesis of pyoderma gangrenosum is still not well understood. Clinically it is classified into ulcerative, pustular, bullous and vegetative types. The diagnosis mainly depends on the recognition of evolving clinical features as there are no specific investigations for the diagnosis. It is essential to exclude other infectious diseases before therapy is initiated as corticosteroids and immunosuppressant therapy are the mainstays in the treatment of this disease. Recently, drugs like tacrolimus, mycophenolate mofetil and infliximab have shown promising results in this condition. Recent concepts regarding the various types of pyoderma gangrenosum and its management are reviewed.     

Pyoderma gangrenosum with pathergy in a pregnant patient without associated systemic disease

Pyoderma gangrenosum (PG) is a rare chronic ulcerative skin condition often associated with systemic disease. PG associated with pregnancy is an extremely rare presentation; only 9 other cases have been reported in the literature. We present PG in a pregnant patient (third trimester) with pathergy. No associated systemic disease was identified. Histology was consistent with PG and the lesions responded to intralesional triamcinolone therapy.     

Refractory pyoderma gangrenosum associated with ulcerative colitis successfully treated with infliximab

Pyoderma gangrenosum (PG) is a rare, immune-mediated ulcerating skin disease. In up to 50 percent of the cases, PG is associated with underlying systemic disorders, most commonly inflammatory bowel diseases, connective tissue diseases, or hematological disorders. Herein, we present a case of refractory PG associated with ulcerative colitis (UC), successfully treated with infliximab.     

PsAPASH: a rare and recent autoinflammatory syndrome associated with hidradenitis suppurativa

Hidradenitis suppurativa is a chronic inflammatory skin disease, which affects 1% of the population, being more common in young, obese and smokers, and mainly affects armpits and groin, with formation of pustules, nodules, abscesses, scars and fistulas. Recently, its association with other autoimmune diseases such as psoriasis, psoriatic arthritis, pyoderma gangrenosum, pyogenic arthritis and ulcerative colitis have been reported. These associated forms are usually resistant to standard treatment, with immunobiologicals as promising therapy. The case of a rare form of association is reported, with only one case previously described in the literature: psoriasis arthritis, pyoderma gangrenosum, acne and hidradenitis suppurativa.     

Pyoderma Gangrenosum in Infants and Children

Abstract: Pyoderma gangrenosum is an uncommon ulcerative skin disorder that occurs in all age groups. Approximately 4% of patients are infants and children. There are several notable differences between the childhood and adult manifestations of the disease, including the distribution of lesions and associated disorders. We reviewed the childhood cases (≤18 yrs of age) of unequivocal pyoderma gangronosum in the English literature and tabulated the trends in clinical features, associated disorders, and therapy. We report our 3-week-old patient, the youngest documented case. Of the 46 patients, only 4 were less than 1 year of age. A systemic Illness was present in 74% of the older children, most commonly, ulcerative colitis. Only one Infant had an associated problem (HIV+) at the time of onset. Infants appear to have an unusual distribution of perianal and genital lesions not often described in other age groups. Our review suggests that pyoderma gangrenosum in children has a similar clinical appearance to that in adults. It Is associated with some of the same underlying disorders, but with different frequencies. The distribution of lesions in children Is similar, often involving the lower extremities, but pyoderma gangrenosum of the head and face appears to be more common in children. Infants may have ulcers in genital and perianal areas. The most frequently prescribed treatment for children is systemic corticosteroids, which generally are very effective.     

Pyoderma gangrenosum associated with Takayasu's arteritis responding to cyclosporin

A 33-year-old caucasian woman with pyoderma gangrenosum associated with Takayasu's arteritis responded to treatment with cyclosporin. This patient is unusual in that both ulcerative and vesiculopustular forms of pyoderma gangrenosum were present. This has not previously been reported with Takayasu's arteritis.     

Pyoderma gangrenosum or cutaneous lymphoma: a difficult clinical diagnosis

Pyoderma gangrenosum is frequently associated with an underlying condition such as ulcerative colitis or a myeloproliferative syndrome. However, lymphoproliferative malignancies have only rarely been seen concurrently with pyoderma gangrenosum. We present the case of a patient with small lymphocytic lymphoma who noted a slowly enlarging skin ulcer that was clinically consistent with pyoderma gangrenosum. Examination of a biopsy specimen showed infiltration of the skin with lymphoma cells as well as deeper necrotic material and thrombosis of vessels that were infiltrated by lymphoma. This case illustrates the difficulty of differentiating pyoderma gangrenosum from cutaneous lymphoma clinically.     

Pyoderma gangrenosum as initial manifestation of Graves' disease

Pyoderma gangrenosum (PG) is an uncommon ulcerative condition. It is most commonly associated with inflammatory bowel disease, haematological malignancies and autoimmune disease. We report on two patients have developed PG during the initial course of Graves' disease (GD), prior to treatment of this condition. The lesions completely cleared with treatment by colchicine in the first case and with oral ciclosporin in the second case. The report emphasizes that a diagnostics of PG should be considered in patients with GD and skin ulcers.     

Treatment of idiopathic pyoderma gangrenosum with infliximab: induction dosing regimen or on-demand therapy?

Pyoderma gangrenosum is an ulcerative skin disease of unknown origin. Recently tumor necrosis factor alpha inhibitors have been gaining attention in the treatment of recalcitrant pyoderma gangrenosum. However, there is a lack of an optimal dosing strategy in the published literature about the treatment of idiopathic pyoderma gangrenosum, and this is responsible for substantially different treatment strategies. Therefore, it is necessary to report experiences in this patient population in order to develop an optimal dosing strategy and to clarify whether regularly scheduled or on-demand therapy is suitable for idiopathic pyoderma gangrenosum. Herein we report a recalcitrant case of an idiopathic pyoderma gangrenosum treated successfully with a dosing regimen similar to the one that has been used in psoriasis. By doing so, we were capable of controlling the disease and improving the quality of life in our patient.     

Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne

Autoinflammatory syndromes associated with hidradenitis suppurativa (HS) and/or acne are rare but potentially debilitating disorders if not diagnosed and treated correctly. They share a common pathogenesis involving a dysregulated innate immune system with abnormal interleukin (IL)‐1 signaling leading to sterile neutrophilic inflammation. The clinical features are recurrent episodes of fever, painful arthritis, and skin lesions consistent with HS, acne, and pyoderma gangrenosum (PG) accompanied by elevated systemic inflammatory markers in blood. So far, several clinically different syndromes have been reported in the literature including pyoderma gangrenosum, acne, and pyogenic arthritis (PAPA), pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH), pyoderma gangrenosum, acne, and spondyloarthritis (PASS), pyoderma gangrenosum, acne, pyogenic arthritis, and hidradenitis suppurativa (PAPASH), psoriatic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PsAPASH), and pyoderma gangrenosum, acne, and ulcerative colitis (PAC). The rarity of the syndromes complicates the establishment of evidence‐based treatment guidelines. Furthermore, treatment can be challenging due to lack of response to standard treatment modalities. Therefore, it is important to increase the awareness about these diseases in order to optimize disease management and ultimately improve the quality of life of patients.     

Chronic Recurrent Multifocal Osteomyelitis Preceding Pyoderma Gangrenosum and Occult Ulcerative Colitis in a Pediatric Patient

Abstract: Chronic recurrent multifocal osteomyelitis (CRMO) is a clinicopathologic entity of unknown origin mainly affecting children and young adults. It shares many clinical features with pyoderma gangrenosum (PG), an uncommon ulcerating inflammatory disorder of the dermis and underlying subcutaneous fat. Both conditions are chronic and relapsing, and have been associated with inflammatory bowel disease (IBD). The diagnoses are made by exclusion. Histology is nonspecific and cultures are negative. There has been only one previous report of both conditions occurring in the same patient. Here we describe the second case, a 12-year-old girl who developed pyoderma gangrenosum in direct continuity to an underlying CRMO lesion of the right anterior tibia. Occult ulcerative colitis (UC) was subsequently discovered.