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PubMed1999

Atypical presentation of herpes simplex virus in a patient with chronic lymphocytic leukemia.

Perianal infections caused by herpes simplex virus are common in immunocompromised patients. The cutaneous presentation in these patients is often atypical, overlaps with the clinical features of other diseases, poses a difficulty in diagnosis, and responds poorly to treatment. An immunocompromised patient with chronic lymphocytic leukemia, treated with oral corticosteroids, presented with chronic perianal ulcerations. This patient was referred for evaluation and treatment of "recalcitrant" pyoderma gangrenosum. Prompt diagnosis was possible when the clinical features were recognized and appropriate biopsy and cultures were obtained. We describe an atypical presentation of herpes simplex virus associated with both an endogenous and exogenous induced immunodeficiency, and stress the importance of routinely performing cultures on all perianal ulcerations and anal fissures to avoid the misdiagnosis, inappropriate treatment, and prolonged discomfort of these afflicted patients.

Aged↗
PubMed1999

[Hepatobiliary and pancreatic complications in patients with ulcerative colitis].

Twenty-four cases of primary sclerosing cholangitis (PSC) and 17 cases of acute or chronic pancreatitis associated with ulcerative colitis (UC) reported in Japan were reviewed. Most of PSC cases revealed intra- and extra-hepatic bile duct involvement. Symptoms of the 22 cases disappeared by predonisolone (PSL) and/or salazosulfapyridine (SASP). Ursodeoxycholic acid was effective in 4 patients. One case received liver transplantation, but currently his liver is biliary cirrhotic 11 years after operation. Another case received total colectomy and pyoderma gangrenosum was cured. Twelve cases had acute pancreatitis, and 5; chronic. Pancreatic duct of 9 patients on ERCP was stenotic and dilated; pancreas was swelling in 3; and normal, in 2. SASP and/or PSL for UC and pancreatitis in 16 patients were effective. Gabexate mesilate and/or urinastatin was used in 10 patients. Only one patient with jaundice received pancreatoduodenectomy. When UC is well controlled, PSC and pancreatitis may be remitted.

Cholangitis, Sclerosing↗
PubMed2000

Considerations for the global assessment and treatment of patients with recalcitrant wounds.

Many factors, both intrinsic and extrinsic, may contribute to wound recalcitrance. For example, arterial circulation may be impaired by atherosclerosis, vasospastic disorders, microemboli, thromboangiitis obliterans, vasculitis, sickle cell anemia, and antiphospholipid syndrome, all of which may impair healing. Inflammatory disorders that may lead to recalcitrance include pyoderma gangrenosum and necrobiosis lipoidica. Chronic venous insufficiency, infection, diabetes mellitus, systemic malignancy, malnutrition, and exposure to pressure and shear prolong the healing process. Wounds secondary to primary skin carcinoma will not heal. Calciphylaxis, a life-threatening metabolic disorder, leads to multiple ulcerations that are especially difficult to heal. Knowledge of common factors that lead to wound recalcitrance is essential to the wound care clinician, as accurate diagnosis results in appropriate treatment. To arrive at the diagnosis, the wound care clinician must perform a thorough history and physical examination and order relevant investigative studies. Treatment is based on correction of the identified underlying condition. By utilizing a systematic approach in the management of each patient with a chronic wound, the wound care clinician increases the probability of achieving wound closure.

Bandages↗
PubMedDate not supplied

Recombinant human GM-CSF in the treatment of poorly healing wounds.

Although most wounds heal rapidly, impaired or delayed tissue repair represents a major clinical challenge. Current therapy is directed at providing a wound with the most favorable environment in which to heal, rather than aiming to increase the rate of healing pharmacologically. Recent studies have suggested that a number of drugs may act specifically to increase healing rates. In vivo studies have demonstrated that recombinant human granulocyte-macrophage colony-stimulating factor facilitates wound contraction, causes local recruitment of inflammatory cells, and induces keratinocyte proliferation. It also activates mononuclear phagocytes, promotes migration of epithelial cells, and further regulates cytokine production. In 2 recent placebo-controlled studies involving venous leg ulceration, subcutaneous perilesional injections of recombinant human granulocyte-macrophage colony-stimulating factor were found to be significantly better than placebo in the time to complete wound healing. In other studies, recombinant human granulocyte-macrophage colony-stimulating factor was administered topically to wounds. Several case reports have also demonstrated the use of recombinant human granulocyte-macrophage colony-stimulating factor for postsurgical wounds, chronic leg ulcers of sickle cell anemia patients, and refractory pyoderma gangrenosum. Despite proper attention to wound care, some wounds fail to heal in an appropriate fashion and may become chronic. Studies of wound physiology as well as experimental and clinical evidence suggest that recombinant human granulocyte-macrophage colony-stimulating factor may promote healing of these lesions.

Chronic Disease↗
PubMedDate not supplied

[What do we know today about diaminodiphenylsulfone?].

INTRODUCTION: Diaminodiphenylsulfone or dapsone is a chemical analogue of sulfapyridine, synthesized in 1908. Dapsone is a bacteriostatic agent that proved to be efficient in treating leprosy and malaria, but today it is used in treating dermatologic noninfectious inflammatory diseases. PHARMACOLOGY: Dapsone is orally used in a dose of 50-400 mg per day in treatment of dermatologic diseases, and also in a dose of 50-100 mg per day in leprosy treatment. Dapsone is mainly eliminated from the body by urine and smaller part by faeces. Pharmacological interaction was reported when it is used with rifampicin and probenecid. MECHANISM OF ACTION: The bacteriostatic effect of dapsone is well known. It involves inhibition of folic acid synthesis in susceptible organisms. The anti-inflammatory effect of dapsone, which proves to be efficient in treating noninfectious inflammatory diseases, has not been explained completely yet. There are some pieces of evidence that anti-inflammatory action is not connected with its antibacteriological action. CLINICAL USE: Based on previous studies about therapy efficiency of dapsone in treating some diseases, there are two groups of diseases: the group responding well to dapsone (leprosy, malaria, DH, linear IgA-dermatosis, erythema elevatum diutinum, bullous systemic lupus erythematosus) and a group responding with average good response to dapsone (pyoderma gangrenosum, bullous and cicatricial pemphigoid, acne conglobata, discoid cutaneous lupus erythematosus, subcorneal pustulosis dermatosis, granuloma faciale, rheumatoid arthritis, polychondritis, leucocytoclastic vasculitis). ADVERSE EFFECTS: Adverse effects depend on the dose and they rarely occur at doses less than 100 mg per day. They are mainly shown on skin, nervous system, digestive system, hepatobiliary system, and kidney and hematologic system. The most important adverse effects are hemolytic anaemia and methemoglobinemia. Hemolysis usually occurs at doses of 200 mg and more per day. In patients with glucose-6-phosphate dehydrogenase deficiency, hemolysis may be provoked by a dose less than 50 mg per day. For prevention, before using dapsone in therapy, clinical examination with history, blood parameters, liver and renal parameters and determination of glucose-6-phosphate dehydrogenase level are recommended. CONCLUSION: The use of dapsone is absolutely indicated in DH treatment and erythema elevatum diutinum. Because of anti-inflammatory effects, dapsone can also be used in treating other inflammatory noninfectious dermatoses when one should take care about "therapy efficiency/adverse effect" balance using the correct dose, monitoring relevant clinical and laboratory parameters and educating patients.

Anti-Infective Agents↗
PubMed2001

[Cutaneous aseptic abscesses, manifestations of neutrophilic diseases].

BACKGROUND: Neutrophilic skin disease includes several entities: Sweet syndrome, pyoderma gangrenosum, erythema elevatum diutium, Sneddon-Wilkinson sub-keratous pustulosis, and neutrophilic eccrine hidradenitis. We report two cases of aseptic abscesses which correspond to the deepest anatomoclinical form of neutrophilic dermatosis. CASE REPORTS: A 28-year-old man was hospitalized for fever and abdominal pain with bloody diarrhea in relation with Crohn's disease. The patient also presented two skin abscesses on the lower limbs. Bacteriology specimens were negative. The histology specimen of a skin lesion revealed neutrophil infiltration of the hypodermis without granulomatosis. Systemic corticosteroid therapy was given and rapidly led to resolution of the inflammatory bowel disease and the skin lesions. The patient developed inflammatory spondylarthropathy several months later. The second patient was a 36-year-old woman with a history of splenomegaly with asceptic abscesses. She was admitted for abdominal pain with non-bloody diarrhea, fever and multiple joint pain related to spondylarthropathy. She developed several simultaneous abscessed nodules on the legs. Biopsy revealed neutrophil infiltration of the hypodermis. The diagnosis of neutrophilic disease with aseptic cutaneous and visceral abscesses was retained. Nonsteroidal antiinflammatory drugs and dapsone were given leading to regression of the skin lesions and the abdominal and joint pain. DISCUSSION: Aseptic skin abscesses result from a deep localization of neutrophilic disease. They suggest the presence of inflammatory bowel disease, spondylarthropathy or other aseptic visceral localizations.

Abscess↗
PubMed2001

[Metastatic Crohn's disease in childhood].

Crohn's disease (CD) is an intestinal inflammatory disease of unknown origin that is sometimes associated with cutaneous manifestations. These skin lesions are usually nonspecific (erythema nodosum, erythema multiforme and pyoderma gangrenosum) and should be differentiated from the cutaneous lesions corresponding to the typical histological pattern of CD; most of these extend directly from the involved bowel. Noncaseating granulomatous infiltration in skin anatomically distant from areas of gastrointestinal CD and separated by normal skin is extremely rare. This entity, known as metastatic Crohn's disease, can be the first symptom of CD or even more unusually can appear during the course of the disease.We report the case of a 9-year-old boy, previously diagnosed with ileocolic and perianal CD, who had been receiving treatment with mesalazine, prednisone and 6-mercaptopurine for 3 years. He presented with swelling of the penis and scrotum of 2 weeks' evolution, without any other symptoms. Testicular ultrasound showed thickening of the scrotal wall and increased flux at this site. Skin biopsy showed noncaseating granulomas. Results of special staining techniques were negative for the presence of microorganisms. The prednisone dose was increased and metronidazole was added, with noticeable improvement in the lesions. Due to reappearance of cutaneous lesions when the prednisone dose was reduced, the patient currently receives maintenance treatment.

Anal Canal↗
PubMed2002

[Skin manifestations with internal diseases. Finding the origin].

Inspection of the skin in its entirety often turns up initial and orientating symptoms allowing the early detection of internal disorders. For example, the painful ulcerations of pyoderma gangrenosum can provide clues as to the presence of underlying inflammatory bowel or joint or hematologic disturbances. Extremely painful ulcerations with livedoid bleeding located on the lower limbs are indicative of calciphylaxis as a serious complication of terminal renal failure. Periorificial and migratory necrolytic erythema may signal a glucagon-producing carcinoma of the pancreatic islet cells. Pasty, whitish-yellow papules on the face associated with a tendency for developing petechiae may be a manifestation of amyloidosis in a plasmacytoma. Numerous diffuse angiomas covering the body of a younger patient should prompt the physician to consider the genetic enzyme defect of alpha-galactosidase (Fabry's disease). In the case of pacemaker- or defibrillator-associated erythema, unnecessary revision of the device can be avoided if such possibilities are considered. In consultation with the dermatologist, the exact classification of symptoms, an accurate diagnostic work-up, and differential-diagnostic considerations should be undertaken with the aim of enabling early initiation of causal or stabilizing treatments.

Biopsy↗
PubMedDate not supplied

Anti-TNF therapy for other inflammatory conditions.

The use of biological agents in inflammatory conditions is rapidly increasing. TNFalpha blocking treatments have changed the course of rheumatoid arthritis, Crohn's disease, juvenile rheumatoid arthritis and psoriatic arthritis. Open label studies with TNFalpha inhibitors in other inflammatory conditions such as adult Still's disease, uveitis, Wegener's granulomatosis, Behçet's disease, scleroderma, Sjögren's syndrome, sarcoidosis, pyoderma gangrenosum, polymyositis/dermatomyositis have shown promising results. However, whether anti-TNFalpha therapy can be safely and efficaciously applied to these other inflammatory disorders requires further controlled studies.

Antibodies, Monoclonal↗
PubMed2002

Targeting tumor necrosis factor-alpha as a potential therapy in inflammatory skin diseases.

Tumor necrosis factor-a (TNFalpha) plays an important role in the initiation and persistence of inflammation in a variety of skin disorders. The availability of selective TNFalpha blockers has provided new therapeutic opportunities. TNFa inhibitors have been employed with success in treating psoriasis in controlled trials, although further studies are warranted to better define which subgroup of patients can benefit most from this treatment and modalities of combination therapy. In addition, an increasing number of case reports indicates that anti-TNFalpha agents are of value in the treatment of other skin diseases, such as pyoderma gangrenosum and Behcet's syndrome, and in the treatment of graft-versus-host disease.

Animals↗
PubMed2002

Topical tacrolimus Protopic.

Topical tacrolimus is the first topical immunomodulator of its kind, and its mechanism of action, pharmacokinetics, metabolism and efficacy will be discussed. Multiple studies have supported the safety and efficacy of topical tacrolimus in the treatment of atopic dermatitis in both adults and children. Studies have also suggested that topical tacrolimus may be effective in the treatment of acute contact dermatitis. Although oral administration of tacrolimus proved to be efficacious in psoriasis, results with topical tacrolimus have been disappointing in some studies. Additional reports of success in the use of topical tacrolimus in the treatment of pyoderma gangrenosum show promise, although there is a paucity of randomized, placebo-controlled trials examining its use. It has also been suggested that topical tacrolimus is useful in the treatment of alopecia areata, erosive lichen planus, isolated ichthyosis linearis circumflexa, steroid-induced rosacea, and in the prevention of allograft rejection, however more clinical trials need to be performed.

Animals↗
PubMed2003

Recurrent eosinophilic mastitis.

Systemic diseases rarely present with manifestations in the breast. Disorders such as sarcoidosis and pyoderma gangrenosum have been described to produce signs and symptoms in the breast. Peripheral eosinophilia is a systemic disease associated with a finite group of conditions including asthma, allergic or atopic disease, collagen vascular disease, and parasitic infection. In addition it has been described in association with several malignancies. A 50-year-old woman with a history of asthma and significant eosinophilia presented with an enlarging breast mass. Complete excision of the mass revealed eosinophilic mastitis. Two years later the patient again presented with a large mass in the same breast. Repeat excision demonstrated the same pathology. The clinical significance of this lesion is important because it presents as an enlarging, painless, dominant mass. Excision is necessary for management and to conclusively rule out malignancy. Its recurrence despite excision to negative margins, however, may indicate that control of the eosinophilia--and possibly the underlying disorder--is important to prevent further recurrence.

Breast↗
PubMed2003

A review of thalidomide's history and current dermatological applications.

Once abandoned because of devastating teratogenic effects, thalidomide has reemerged as an alternative treatment in many dermatologic diseases. In 1998, thalidomide became FDA approved for the acute treatment and suppression of the cutaneous manifestations of erythema nodosum leprosum (ENL). ENL is a systemic disorder that typically occurs after several years of antileprosy treatments, usually for lepromatous leprosy. Off-label uses for thalidomide include: aphthous stomatitis, Behçet disease, pyoderma gangrenosum, chronic discoid lupus erythematosus, systemic lupus erythematosus, lichen planus, prurigo nodularis and sarcoidosis. This review examines the background, pharmacokinetics, mechanism of action, side-effects, and indications of thalidomide.

Dermatologic Agents↗
PubMed2003

[Therapy resistant leg ulcer caused by multiple myeloma].

The authors review an elderly woman suffering from leg ulcer with bad curability which was a consequence of a malignant haematologic disease. Multiple relapse of this ulcer was observed and did not react to usual conservative therapy. The only sign of multiple myeloma was the extremely high level of iron measured in the blood serum bound to a monoclonal paraprotein. Sternum aspiration was made and the sample showed presence of plasmoblasts supporting diagnosis of multiple myeloma. The poor therapeutical results were caused by hyperviscosity syndrome in consequence of the high level of the monoclonal component in the blood serum. The ulcer was cured within eight weeks by suppression of the monocloclonal component and thus, elimination of hyperviscosity. This case is a special one from several points of view. Leg ulcers not reacting to usual therapy may be caused by haematologic disease thus the physician should consider this and extend examinations as well and necessarily hospitalize patient. Appearance of multiple myeloma is unusual in this case. Hystology made from the skin excised from the periphery of the wound has not showed signs of pyoderma gangrenosum, which is known mostly being associated with multiple myeloma.

Aged↗
PubMed2003

[Extra-intestinal manifestation of IBD in Veszprém county (of Hungary): results of a 25-years follow-up study].

INTRODUCTION: IBD is a systemic disease associated with a number of extraintestinal manifestations (EIM). The author's aim was to determine the prevalence and features of EIM in a large IBD population in Veszprém county in a 25-year follow-up study. PATIENTS AND METHODS: 873 IBD patients were enrolled in the study (UC: 619, m/f: 317/302, age at presentation: 38.3 yrs, disease duration: 11.2 yrs; CD: 254, m/f: 125/129, age at presentation: 32.5 yrs, disease duration: 9.2 yrs). Intestinal, extraintestinal symptoms and laboratory tests were monitored regularly. Any alteration suggesting an EIM was investigated by specialist. RESULTS: A total of 21.3% of patients with IBD patients had EIM (UC: 15.0%, CD: 36.6%). Age at presentation did not affect the likelihood of EIM. Prevalence of EIM was higher in women and in CD, ocular complications and PSC were more frequent in UC. In UC there was an increased tendency of EIM in patients having a more extensive disease. In CD patients colonic location increased the frequency of axial and type-1 peripheral arthritis. In UC positive family history increased the risk of joint (OR: 3.63) complications. Joint complications were more frequent in CD (22.4%) than in UC (10.2%, p < 0.01). PSC was present in 1.6% in UC and 0.8% in CD. Dermatological complications were present in 3.8% in UC and 10.2% in CD, the rate of ocular complications was around 3% in both diseases. Erythema nodosum, pyoderma gangrenosum and uveitis were the most frequent manifestations among them. Rare complications were glomerulonephritis, autoimmune haemolytic anaemia and celiac disease. CONCLUSION: The prevalence of EIM in Hungarian IBD patients was in concordance with data from Western countries. The high number of EIM supports a role for complex follow-up in these patients.

Adolescent↗
PubMed2003

[The use of tacrolimus in dermatology].

This article aims to review the literature on the use of tacrolimus in dermatology. We tried to focus on the local application of tacrolimus. The local application of tacrolimus is known to be effective for several skin diseases, especially atopic dermatitis. Since the major action site of tacrolimus is activated T-lymphocytes, the drug showed efficiency mostly in inflammatory dermatological diseases including psoriasis, lichen planus, alopecia areata and pyoderma gangrenosum. Tacrolimus lacks most of side effects of local steroids and seems to have better results for short and long term application. We presume that tacrolimus will be widely used on skin diseases, especially when it becomes less expensive.

Dermatitis, Atopic↗
PubMedDate not supplied

Pulmonary and hematological alterations in idiopathic ulcerative colitis.

BACKGROUND: Patients with idiopathic ulcerative colitis (IUC) may suffer from one or more extraintestinal manifestations. We decided to prospectively study the prevalence of extraintestinal manifestations among patients with IUC, with special reference to pulmonary and hematological alterations. METHODS: Fifty-one consecutive patients with IUC attending the gastroenterology services of our tertiary-care referral center were evaluated prospectively. A detailed clinical evaluation of the musculoskeletal system, eye and skin, X-ray examination of the sacroiliac joints and chest, liver function tests, coagulation profile, hemogram, pulmonary function tests (PFT) and diffusion capacity for carbon monoxide (DLCO) were done in all patients. ERCP, liver biopsy and high-resolution computerized tomography (HRCT) of the chest were performed whenever indicated. RESULTS: Nearly half (24/51; 47%) the patients had one or more extraintestinal manifestations; these included pulmonary function abnormalities (14 patients; 27%), sacroiliitis (8; 16%), arthritis (5; 10%), ocular complications (4; 8%), and pyoderma gangrenosum and Budd-Chiari syndrome (one patient each). Of the 14 (27%) patients who had abnormal pulmonary function, isolated PFT abnormalities were seen in 8 (restrictive pattern in 7, obstructive pattern in 1), decreased DLCO in four, and both of the above in two. All patients with decreased DLCO (n=6) were asymptomatic and had normal chest X-ray and HRCT chest. Thirteen (25%) patients had thrombocytosis, 19 (37%) had increased fibrinogen level, and one patient had decreased antithrombin level. Disease activity had significant association with decreased DLCO (p=0.008), increased platelet count (p<0.0001), increased fibrinogen level (p=0.016), low antithrombin levels (p=0.046) and arthritis (p=0.002). CONCLUSION: Extraintestinal manifestations of IUC were seen in 47% of patients. Asymptomatic pulmonary interstitial involvement was seen in 12%, more often among patients with active disease.

Adult↗