Pyoderma gangrenosum as an early revelator of acute leukemia

Bullous pyoderma gangrenosum is an atypical, more superficial variety of the classical pyoderma and is often associated with myeloproliferative disorders. We present the case of a patient who presented initially with subcutaneous nodules and who developed bullous lesions afterwards. Histological evaluation showed the presence of neutrophilic infiltrates in both lesions. A few months after the diagnosis of bullous pyoderma gangrenosum, an underlying leukemia was revealed. Our case illustrates the importance of regular blood and bone marrow examinations in patients with atypical bullous pyoderma gangrenosum, resulting in a rapid diagnosis of the underlying disease.     

Pyoderma gangrenosum associated with ulcerative colitis

We report the case of a 45-year old man with non-healing ulcers located on his chest, lumbal, sacral, retroauricular areas and forehead. Both clinical and histopathological examinations suggested pyoderma gangrenosum (PG). For six months the diagnosis of ulcerative colitis was established. PG in our patient was presented as a rapidly enlarging, painful ulcer with purple, undermined edges and a necrotic, haemorrhagic base. Initially, he was treated with a high dosage of peroral glucocorticosteroid, sulfasalazine, and systemic antibiotics, together with daily wound care. Ulceration partially regressed. Total colonoscopy showed pancolitis. When the dose of glucocorticosteroids was tapered down to 35 mg, new ulcerations on his right thigh and abdomen were formed. He also developed E. coli sepsis and flare up of bowel disease. Azathioprine, together with two pulse doses of glucocorticosteroids and antibiotics, were administered. He was scheduled for a total colectomy. The management of PG continues to be a therapeutic challenge.     

Pyoderma gangrenosum associated with acne conglobata

We report a 16-year-old male in whom pyoderma gangrenosum appeared in conjunction with acne conglobata. The patient also developed a seronegative spondyloarthropathy that was the main presenting complaint. There was no evidence of inflammatory bowel disease. Treatment with isotretinoin was successful. Both acne and pyoderma lesions healed and the articular symptoms improved. The present case, together with other reports in the literature show that acne conglobata must be included in the list of possible associations of pyoderma gangrenosum. We also comment on acne arthritis, a relatively frequent phenomenon, although still not generally known, in acne conglobata.     

Pyoderma gangrenosum associated with hidradenitis suppurativa

Pyoderma gangrenosum (PG) is associated with a number of systemic diseases. PG in association with hidradenitis suppurativa (HS) has been rarely reported. We describe six patients (three men, three women; aged 35--51 years), who developed PG on a background of HS. The onset of PG occurred only after HS had been present for at least two decades. No relationship in disease activity between the two conditions was observed. Three patients described previous severe adolescent acne vulgaris, one had concurrent systemic lupus erythematosus and another had chronic iron-deficiency anaemia. The course of PG was severe and refractory in four patients, who required treatment including high-dose oral corticosteroids, ciclosporin, intravenous immunoglobulin and intravenous cyclophosphamide.     

Pyoderma gangrenosum associated with Takayasu's arteritis

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by destructive, necrotizing and noninfective ulceration of the skin mostly on lower extremities. PG is well known as a complication of Takayasu's arteritis in Japan. However, this association is not commonly observed in North American and European patients. We describe a case of PG that was associated with Takayasu's arteritis who was successfully treated with systemic cyclosporin. We have reviewed 35 well-documented PG cases with Takayasu's arteritis in comparison to 106 PG cases without Takayasu's arteritis. The results demonstrate that this association occurs predominantly in young females and that these cases exhibit more widespread PG lesions.     

Pyoderma gangrenosum following tattoo placement in a patient with acute myelogenous leukemia

A 37-year-old African American female with a diagnosis of acute myelogenous leukemia (AML) being treated with chemotherapy presented with a lesion on her lower back within the confines of a newly inked tattoo. Five days after tattoo placement, she developed an oozing, indurated, necrotic plaque at the site. Four days later, she developed chills, fever, and neutropenia. A skin biopsy was performed and was consistent with pyoderma gangrenosum (PG) or neutrophilic dermatoses. PG is an inflammatory skin disease associated with both cutaneous trauma and systemic disease, including hematologic malignancy. PG after tattoo placement, in both healthy patients and those with hematologic malignancies, has, to our knowledge, not yet been described in the literature. While further studies are necessary to investigate the link between PG and tattooing, oncologists may wish to counsel patients with leukemia to refrain from obtaining new tattoos.     

Pyoderma gangrenosum associated with paroxysmal nocturnal haemoglobinuria

A case of pyoderma gangrenosum of the lip occurring in association with paroxysmal nocturnal haemoglobinuria is described. This is an extremely rare association, which has been documented in the literature on only two previous occasions.     

Pyoderma gangrenosum associated with polycythaemia rubra vera

Pyoderma gangrenosum may occur in patients with haematological disorders but has not frequently been reported in patients with polycythaemia rubra vera. To support the view that this is a significant association, we report a further two cases and discuss the implications of the development of pyoderma gangrenosum in patients with polycythaemia.     

坏疽性脓皮病合并炎症性肠病

报告1例坏疽性脓皮病合并炎症性肠病。患者男,30岁。腹痛、腹泻及稀薄脓血便1个月余;躯干及四肢出现散在的红色丘疹、脓疱疹,溃疡伴疼痛12 d入院。皮损组织病理检查:表皮角化过度,棘层增厚,真皮全层及皮下脂肪间大量中性粒细胞浸润,可见红细胞外渗及血管壁纤维蛋白样变性。电子肠镜诊断为炎症性肠病。抗核周型中性粒细胞胞质抗体(anti perinuclear antineutrophil cytoplasmic antibody,p-ANCA)阳性。予糖皮质激素、美沙拉嗪及支持治疗后,患者病情明显好转。     

Pyoderma gangrenosum coexisting with acute myelogenous leukaemia

The frequency of occurrence of malignant neoplasms in the cases of pyoderma gangrenosum is not exactly determined, but it can be assessed to be at 7%. The aim of the study was to report a 26-year-old male patient with pyoderma gangrenosum coexisting with acute myelogenous leukaemia. The first skin lesions on both tibia occurred in June 2001. Prior to the proper diagnosis of pyoderma gangrenosum, the patient was treated surgically. Because of the dramatic dermatological and general condition in November 2001, the patient was admitted to the Dermatological Department of the Silesian Medical Academy in Katowice where the diagnosis of pyoderma gangrenosum was established. On the clinical and biochemical picture, the diagnosis of pyoderma gangrenosum within acute myelogenous leukaemia was made. Initially, cyclosporin A 200 mg orally per day in the therapy of pyoderma gangrenosum was administered to achieve a slight clinical improvement. Although chemotherapy leukaemia was performed, the patient died after 4 months of the confirmation of the acute myelogenous leukaemia diagnosis.     

Pyoderma gangrenosum associated with c-ANCA (h-lamp-2)

An 18‐year‐old African–American woman presented with a 3‐week history of painful ulcerations on her legs and trunk; she linked their onset to a fall and injury to the ankle. Well‐circumscribed hemorrhagic ulcerations with ragged borders were noted on the legs, thighs, hands, and breasts. A skin biopsy demonstrated abscess formation and focal dermal necrosis compatible with a diagnosis of pyoderma gangrenosum. A laboratory work‐up disclosed the following: white blood cells (WBC) 9.5 cells/mm³, hemoglobin 10.5 g/dL; erythrocyte sedimentation rate (ESR) 33 mm/h (0–20); c‐ANCA (ANCA, antineutrophil cytoplasmic antibody) titer 1 : 320. Further testing revealed that the ANCA targeted both human lysosomal‐associated membrane protein‐2 (h‐lamp‐2) (> 200 EIA units [< 10]) and bactericidal/permeability increasing protein (BPI) (21 EIA units [< 10]), but neither proteinase‐3 (PR3) nor myeloperoxidase (MPO). The ANCA isotypes were both immunoglobulin G (IgG) and IgA. Urinalysis, antinuclear antibodies (ANA), antiphospholipid antibodies, serum protein immunofixation electrophoreses, and chest X‐ray were unremarkable. Therapy with minocycline was initiated pending biopsy results, with a favorable but incomplete response. Oral prednisone was added at 0.6 mg/kg/day, resulting in closure of all lesions except those on the lower legs. These lesions resisted subsequent trials of topical nitroglycerine and clofazimine. During questioning the patient acknowledged having three loose stools daily. She also reported an isolated episode of blood in her stool. A colonoscopy was performed, ulcerative colitis was diagnosed, and therapy with mesalamine was begun. At the family's request, the prednisone was stopped, notwithstanding the persistence of ankle ulcerations, and oral tacrolimus was administered at 0.2/mg/kg/day, leading to further improvement. Tacrolimus‐induced hypomagnesemia and paresthesias precluded the use of higher doses, and this agent was discontinued. Reinstitution of prednisone combined with dapsone ultimately led to complete healing.