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A case of pyoderma gangrenosum associated with idiopathic myelofibrosis is described. It is important to recognise it, since the treatment with corticosteroids and sulphonamides is frequently effective.
Pyoderma gangrenosum (PG) is a painful chronic ulcerative skin disorder often occurring in association with systemic disease. It typically affects the lower limbs, but may also involve other sites, or sometimes develop after trauma of surgical procedures. We report the case of a woman with rheumatoid arthritis who developed disfiguring and severe PG of the right breast, a rare site, following biopsy for a benign breast lesion, and who was subsequently successfully treated with low-dose cyclosporin A.
Both pyoderma gangrenosum (PG) and cutaneous (metastatic) Crohn's disease (CCD) may occur in the setting of inflammatory bowel disease (IBD). Clinical distinction between PG and CCD may be difficult because clinical and pathologic features often are similar. Although surgical debridement is therapeutic in CCD, it may lead to increased tissue loss and disease progression (pathergy) in PG. Thus, it is important to determine a definitive diagnosis before surgical debridement, especially in tissue-sensitive sites. We present a patient with chronic ulceration of the penis who ultimately was diagnosed with CCD following an initial misdiagnosis of PG.
Six months after the occurrence of pyoderma gangrenosum, subcorneal pustular dermatosis developed in a 60-year-old woman. The patient's serum was found to contain an IgA-lambda paraprotein. Although similar cases have been reported, this case is unique in that a distinct episode of pyoderma gangrenosum was followed by subcorneal pustular dermatosis.
Two patients with refractory pyoderma gangrenosum (PG) were treated with oral Tripterygium wilfordii multiglycoside (TWG). TWG is a Chinese medicine extracted from a medicinal herb, Tripterygium wilfordii Hook F, and has potent anti-inflammatory and immunosuppressive effects. The effect of TWG on PG was demonstrated by clinical findings. Improvement of the lesions occurred within two weeks, and the ulcers healed about a month. Mild side effects such as gastrointestinal disturbances were observed in both patients. These side effects were patient-acceptable, and there was no need to stop the treatment. Transient elevation of serum ALT was observed in one patient; the serum ALT returned completely to normal after the discontinuation of TWG. These results suggest that TWG may be an effective alternative for refractory PG and that careful monitoring of liver function during TWG treatment is necessary.
A 46-year-old woman had a 17-year history of intermittently severe pyoderma gangrenosum without identifiable associated systemic disease. Her condition had become unresponsive to corticosteroid and sulfone therapy given for systemic effect, but responded completely to 150 mg/day of cyclophosphamide. Immunosuppressive therapy should be considered in patients with severe, recalcitrant pyoderma gangrenosum, even in the absence of associated systemic disease.
UNLABELLED: Inflammatory bowel disease is becoming increasingly frequent in children of all ages. In addition to the usual gastrointestinal stigmata of weight loss, anaemia, and rectal bleeding, children may exhibit prominent extra-intestinal manifestations such as joint symptoms, skin signs and some other auto-immune manifestations. Here we present a 15-year-old girl with ulcerative colitis in whom pyoderma gangrenosum and acute sensorineural hearing loss developed. CONCLUSION: Although pyoderma gangrenosum is well described with inflammatory bowel disease, sensorineural hearing loss a is very unusual finding, possibly of auto-immune aetiology. We recommend steroid or immunosuppressive therapy in such a patient.
Pyoderma gangrenosum is an unusual neutrophilic dermatosis associated to different rheumatologic, haematologic and inflammatory bowel diseases which generally responds to the therapy of the underlying disease. We report a case of pyoderma in a 44-years-old woman with paucisymptomatic, distal, ulcerative colitis which appeared simultaneously in her forehead and hands. We think it of general interest because of its localization, its importance, the failure of response to steroids and the need of cyclosporine A for regression.
Postoperative endophthalmitis may present in an atypical fashion (absent or minimal anterior chamber reaction) in the presence of underlying immunosuppressive disorder. The authors describe an apparently healthy 58-year-old man who displayed endophthalmitis with minimal anterior chamber reaction following penetrating keratoplasty for granular corneal dystrophy with underlying acute myeloid leukemia. Scleromalacia perforans in association with pyoderma gangrenosum subsequently developed, leading to ciliary staphyloma and corneal melting. Pyoderma gangrenosum is an uncommon, idiopathic skin disease that may also have ocular manifestations.
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.
A 68-year old woman presented with ulcerations on the calves that had occurred spontaneously. The very painful lesions both clinically and histologically showed the characteristics of pyoderma gangrenosum. During hospitalization she was treated with corticosteroids (oral, i.v., topically), clofazimine, cyclophosphamide, intravenous immune globulin, cyclosporine (oral, local), dapsone, thalidomide and sodium cromoglycate (topically) without any benefit. Finally, when treated with mycophenolate mofetil (CellCept) (oral) and cyclosporine (oral), her skin lesions showed continuous improvement. The topical application of thrombocytic growth factors (cytokines) probably accelerated the granulation. Eight weeks after initiating this treatment the lesions could be covered with split thickness skin grafts. Our observation suggests that mycophenolate mofetil, a novel immunosuppressive agent which has thus far been used almost exclusively in transplantation medicine, may be an effective therapeutic modality in combination with cyclosporine A for the treatment of pyoderma gangrenosum.
We present the case of a 59-year-old woman who had large ulcerations on her right leg that were diagnosed initially as pyoderma gangrenosum and treated with three immunosuppressive agents (cyclosporin, prednisone and azathioprine) for 6 months. Results of a biopsy at 6 months showed numerous cigar-shaped bodies consistent with Sporothrix schenckii; identification was confirmed by tissue culture. A retrospective review was performed for all cases diagnosed as sporotrichosis from tissue culture or biopsy specimens at the Mayo Clinic. Nineteen cases were identified. The present case was the only one in which fungal organisms were visible on histological examination. The present case emphasizes the importance of making a definitive histological diagnosis in unusual ulcer cases or in suspected cases of pyoderma gangrenosum before the initiation of immunosuppressive therapy. The large number of cigar-shaped bodies in the tissue is a rare finding in sporothrix infection and has been reported in only two cases previously.
The antiphospholipid antibody syndrome has been associated with various cutaneous manifestations, the most common of which may be leg ulceration. Cutaneous ulcers with clinical features suggestive of pyoderma gangrenosum have been reported in this setting only rarely. We report here two additional cases of the association between antiphospholipid antibody syndrome and pyoderma gangrenosum-like skin lesions. These patients suffered from the primary antiphospholipid syndrome. We suggest that clinicians assay for antiphospholipid antibodies when a clinical diagnosis of pyoderma gangrenosum is entertained.
A 58-year-old man with chronic "idiopathic' pyoderma gangrenosum, recurrent secondary staphylococcal infection, nail candidiasis, markedly impaired lymphocyte function and a serum blocking factor is described. Despite an initially favourable response to immunosuppressive therapy, the patient later relapsed whilst on high dose systemic corticosteroids and azathioprine. Immune function remains abnormal 3 years cessation of azathioprine. Attempted immunostimulation with Corynebacterium parvum proved unsuccessful. A striking but transient increase in lymphocyte function followed parenteral iron therapy for an unexplained iron-deficiency anaemia. Gradual reduction in prednisone dosage to 5 mg daily has coincided with a period of clinical resolution and modest improvement in lymphocyte reactivity. Prolonged immunosuppressive therapy may be inadvisable in chronic idiopathic pyoderma gangrenosum associated with defective cellular immunity.
In two patients, active chronic hepatitis was complicated by the development of pyoderma gangrenosum. The favorable response of the pyodermatous lesions to azathioprine therapy suggests that this drug may be of value in treating this disorder in patients for whom corticosteroid therapy produces no benefit or is contraindicated because of side effects. Azathioprine, too, is a potentially toxic drug and may need to be discontinued. Possible causal relationships between pyoderma gangrenosum and active chronic hepatitis are discussed.
We present the case of a 15-year-old boy who developed facial pyoderma gangrenosum following an allogeneic bone marrow transplantation for the treatment of a 'blast' crisis developing in the course of chronic myelogenous leukaemia. The lesion appeared 7 months before any evidence of relapse. The discussion is focused on both the presentation of pyoderma gangrenosum associated with myelo-proliferative disorders and its pathogenesis via the underlying immunosuppression.
A male patient 68 years, suffering from pyoderma gangrenosum which was resistant to conventional treatment, received clofazimine 400 mg daily for 5 months, then reducing to 300 mg daily for the next 6 months. Eleven months after starting the drug, he was admitted to hospital with severe abdominal pain, laparotomy revealing infarction of the spleen, with violaceous congestion of the small bowel. The spleen was removed and post-operative recovery was satisfactory. Histopathological examination of the spleen (removed at operation) and of tissue from a duodenal biopsy (taken postoperatively) showed large numbers of striations and outlines suggestive of crystal deposition. Mesenteric lymph node revealed a massive accumulation of crystals in cortical and medullary sinuses. The findings emphasize that clofazamine should not be used in high dosage over prolonged periods of time, except under close clinical and laboratory supervision, and for conditions not amenable to other drugs.
PURPOSE: A 37-year-old Hispanic man with a history of chronic myelogenous leukemia was first seen with fever and preseptal cellulitis of this right orbit after a tooth extraction. METHODS: The patient subsequently developed bilateral, severe peripheral ulcerative keratitis. He was treated with systemic antibiotics for a presumed underlying infectious cause. Several painful, necrotizing skin lesions developed over his face, trunk, and extremities. RESULTS: Repeated skin biopsies of the necrotizing lesions were consistent with pyoderma gangrenosum. Both the skin lesions and peripheral ulcerative keratitis responded dramatically to systemic prednisone. CONCLUSIONS: Pyoderma gangrenosum should be included in the differential diagnosis of peripheral ulcerative keratitis.