The extracutaneous involvement in the neutrophilic dermatoses.
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BACKGROUND: Erosive pustular dermatosis of the leg (EPDL) has been described in association with venous insufficiency and atrophy of the skin of the lower leg. Like erosive pustular dermatosis of the scalp, this disease has also been reported to be a non-infective condition. OBJECTIVES: To investigate the clinicopathological features and, where possible, the aetiology of clinical EPDL. METHODS: We identified a group of patients undergoing continuous compression bandaging for venous dermatitis of the legs and/or predominantly venous leg ulceration with clinical features described in patients with EPDL. They were investigated by skin biopsy, patch testing and microbiological tests for the presence of bacteria and fungi. RESULTS: Twenty-four of 400 (6%) patients were noted to have pustules on the leg(s). There was laboratory evidence of fungal infection in 13 of 24 patients (54%), with complete and sustained resolution of pustules after antifungal treatment. Pustulation in the other 11 patients (46%) was unresponsive to antibiotics for confirmed bacterial infection; some improvement was seen with potent topical corticosteroids but full clearance was achieved only after a switch from continuous four-layer compression bandaging to the use of intermittent long stretch compression. CONCLUSIONS: EPDL is a fairly common clinical picture seen in patients undergoing continuous compression bandaging. It may be produced by opportunistic, particularly fungal, infection. In almost half an infective aetiology cannot be demonstrated and a pyoderma gangrenosum-like process may be implicated.
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A 27-year-old woman presented with a right infra-auricular noduloulcerative lesion progressing to a peri-auricular pyoderma gangrenosum-like ulcer with destruction of her right earlobe over an 8-month period. Similar nodules appeared on the right malar and left infra-auricular regions. The cutaneous manifestations were associated with nasal congestion, rhinorrhoea and serosanguineous nasal crusting. Skin biopsy demonstrated suppurative granulomatous inflammation. Investigation of both renal and pulmonary function showed no abnormality. Serological testing revealed a positive cytoplasmic pattern antineutrophil cytoplasmic antibody with high proteinase-3 specificity, which in conjunction with the clinical findings is consistent with a diagnosis of protracted superficial Wegener's granulomatosis. Initial treatment with prednisone 1 mg/kg/day and azathioprine 100 mg/day resulted in complete resolution of her lesions. Reduction of the corticosteroid dose below 0.3 mg/kg/day led to recrudescence of cutaneous and upper respiratory tract symptoms, at which stage methotrexate was substituted for azathioprine with rapid induction of remission and further prednisone withdrawal. Thirty-two months after the initial diagnosis the patient remains well with no other organ involvement.
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BACKGROUND: Sinus tracts are difficult wounds where clinical evidence to direct management is lacking. Should medical therapy fail, surgical options focus on various flaps and tissue grafts. We report the successful use of a simple surgical procedure to promote healing in recalcitrant sinus tracts: the use of the dermal matrix substitute Cymetra (LifeCell Corp., The Woodlands, NJ). OBSERVATIONS: Two patients-one a woman with a chronic sacral decubitus and the other an older gentleman with pyoderma gangrenosum-each developed a sinus tract that was unresponsive to medical therapy. After use of Cymetra, both patients healed completely without adverse reaction. They remained ulcer free at 1-year follow-up. CONCLUSION: Cymetra, micronized decellularized cadaveric dermis, may have utility in repairing soft tissue defects and promoting wound healing. It offers a safe and effective alternative in the management of recalcitrant sinus tracts.
Behçet's disease is a multisystem inflammatory disorder of unknown origin, characterized by recurrent oral and genital ulcerations, ocular and cutaneous lesions, arthritis, central nervous system, and vascular disease. There is no pathognomonic laboratory test, but there are clinical criteria to assist in establishing the diagnosis. Behçet's is most common along the Silk Road. It is particularly common among persons who have the HLA-B51 major histocompatibility type. Cutaneous lesions include pustules, erythema nodosum-like lesions, Sweet's-like lesions, pyoderma gangrenosum-ike lesions, and pathergy. The major cutaneous findings may be classified as neutrophilic vascular reactions. There is considerable morbidity resulting from Behçet's disease, most notably a high risk of blindness from ocular involvement. Mortality may occur as a result of neurologic or vascular disease or gastrointestinal perforation.
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Pyoderma gangraenosum (PG) is a serious chronic, ulcerative skin disorder afflicting both adults and children. As PG is often associated with systemic diseases (>50%) such as inflammatory bowel disease, rheumatoid arthritis or haematological disorders, it requires a multidisciplinary approach. This disorder is not commonly reported in paediatrics; therefore children with PG represent a particularly difficult diagnostic challenge. Clinical diagnosis is often delayed and PG is only considered after eliminating other causes of cutaneous ulcers. We report a 4-year-old boy with secondary myelodysplastic syndrome following treatment for acute lymphoblastic leukaemia who presented with a massive inflammatory, ulcerative proliferation of the lower lip which was diagnosed as PG. We have reviewed the literature with reference to diagnostic criteria and treatment options of this disorder that is particularly rare in childhood.
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BACKGROUND: Crohn's disease (CD) is frequently associated with extra-intestinal manifestations (EIMs) and infliximab has been recently proposed for the treatment of CD with EIMs. Our aim was to evaluate the short-term efficacy of infliximab in this treatment. PATIENTS AND METHODS: Thirty CD patients were treated with infliximab. Fifteen patients (50%) showed EIMs before starting therapy. Ten patients presented an arthritis (five sacroiliitis, five spondylitis), with six also reporting peripheral arthralgias. Four patients presented cutaneous EIMs while three patients had an ocular EIM. RESULTS: At week 10, all patients reported an improvement in EIMs. Regarding arthritis, ASAS20 and ASAS40 improvement was observed in 80% and 60% of patients, respectively. In the four patients with cutaneous EIMs and in the three with ocular EIMs, complete healing was observed. Recurrence was observed in 10 out of 15 patients (66%) and a second course of treatment with infliximab was required. This proved to be effective in all cases except for one patient who stopped treatment because of a severe adverse reaction. CONCLUSIONS: Infliximab is an effective drug in the short-term treatment of EIMs complicating CD. Although relapse of EIMs occurs frequently, retreatment ensures effective control of the symptoms.
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