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Connective tissue disease in children.

As our understanding of connective tissue disease expands, so too does our therapeutic armamentarium. We have learned that autoimmunity triggers inflammation through unchecked, proliferative cell-mediated inflammation. By targeting this arm of the cytokine cascade, it may be possible to arrest further progression. Several biologic agents, such as etanercept, alefacept, infliximab, efaluzimab, and, recently, adalimumab, have come to market for adult psoriasis and are now undergoing trials for juvenile SLE, psoriasis, and psoriatic arthritis. Of note, etanercept has been used successfully in juvenile rheumatoid arthritis for more than 10 years. These agents target cell-mediated inflammation through various mechanisms and hold great promise for the treatment of many of the disease states discussed above. Moreover, the biologics carry an improved side-effect profile not seen with traditional agents such as corticosteroids and will be central in the evolution of targeted therapeutics for these complex immunologic diseases.

Child↗

Tularemia: a case transmitted from a sheep.

Tularemia is an arthropod-borne infectious disease caused by Francisella tularensis, a gram-negative microorganism that normally resides in a wide range of wild and domestic animals. The disease is characterized by a sudden onset with high fever, headache, malaise, chills, myalgia, and arthralgia. A short time after exposure, an inflamed and ulcerated lesion rapidly appears at the site of entry. A regional lymphadenopathy follows the cutaneous presentation. Cultures from the lesions or blood generally give negative results. Histopathologic examination reveals either a nonspecific inflammatory infiltrate or an infectious granuloma. The most useful laboratory procedure in the diagnosis of tularemia is serologic tests. Streptomycin, gentamicin, and tetracycline are the drugs of choice in the treatment. Quinolones are also effective. Tularemia is fairly rare in Turkey. We present a typical case of ulceroglandular tularemia transmitted from a sheep to a young man.

Adult↗

[Leukemic neutrophilic dermatosis].

A case of a 67 year-old female patient with acute myeloid leukemia is presented. As the first manifestation of the disease, the patient had symptoms of Sweet's syndrome, later signs of gangrenous pyoderma have developed. This transient form is termed as a "leukemic neutrophilic dermatosis". The authors focus on the important diagnostic and prognostic value of this entity.

Aged↗

[Dermato-mucosal manifestations of Behçet's disease].

Oral and genital aphthae are the main clinical dermatologic manifestations of Behçet's disease. They look like those that occur in other aphthosis. Cutaneous lesions include pseudofolliculitis, folliculitis, erythema nodosum-like lesions, Sweet's-like lesions and pyoderma gangrenosum-like lesions. Histologically, these lesions are frequently perivascular with proeminent infiltrates of neutrophils and/or lymphocytes. Hypersensibility to needle pricks is explored by the pathergy test which sensibility is highly variable depending on the countries. When there is no systemic lesions requiring oral corticosteroids or immunosuppressive therapy, colchicine, aspirine, or dapsone may be prescribed. Thalidomide is sometimes required if aphthosis is refractory to other treatments despite its neurotixic and teratogenic effects.

Behcet Syndrome↗

[Interobserver variation in the Red-Yellow-Black wound classification system].

Communicating wound descriptions between disciplines for treatment and wound care necessitates a simple and unequivocal classification system. The Red-Yellow-Black (RYB) system has been suggested to comply with these demands. The reliability of the RYB-system has, however only been investigated in small studies. The aim of this study was to determine interobserver homogeneity (group Kappa a.m. Schouten) of the RYB-system and further to examine whether interobserver homogeneity was dependent on educational level. One-hundred-and-twenty photo-slides of non-healing ulcers of various etiologies were shown to 21 observers who recorded their assessments in an entry form without discussing their assessments with the other observers. Eighty-nine percent of the possible assessments were completed. Observed agreement for all observers = 0.65; Kappa = 0.47. Kappa-coefficient in subgroup of nurses = 0.49, subgroup of physicians with less than three years of experience with wound treatment = 0.46 and for physicians with more than 10 years of experience with wound healing = 0.48. In conclusion, we demonstrated moderate interobserver agreement for using the RYB-characteristics. The RYB-system is useful for communication about wound care and treatment. However, continuous education and consensus meetings are advisable to increase agreement.

Humans↗

Cutaneous side effects induced by indinavir.

HIV-protease inhibitors demonstrated such high efficacy in short-term studies that they have been approved by the FDA, even though possible toxicity still needs further investigation. In the period between January 1997 and August 1998, 101 patients, staying at San Patrignano Medical Centre (Italy), received an HIV protease inhibitor (indinavir) plus two nucleoside reverse transcriptase inhibitors (NRTI's) selected from the following: AZT, didanosine, zalcitabine, lamivudine or stavudine. Seventy-three patients were male, 28 female and their ages ranged from 25 to 60 years, with an average of 34. At the end of the study, 84 patients were suitable for evaluation, as the other 17 dropped out for various reasons. Forty-eight patients (57.1%) developed cheilitis, 34 (40.5%) experienced diffuse cutaneous dryness and pruritus, 10 (11.9%) developed asteatotic dermatitis on the trunk, arms and thighs and another 10 (11.9%) complained of scalp defluvium. A severe alopecia was observed in only 1 patient (1.2%), while 6 reported that their body hair had become fairer, thinner and shed considerably. Multiple pyogenic granulomas were observed in the toenails of 5 patients (5. 9%). Softening of the nail plate was noted in 5 subjects as well. A peripheral lipodystrophy syndrome was noted in 12 patients (14.3%). Among these, one patient only developed a "buffalo hump" and another had diffused lipomatosis. The temporal relationship between the taking of indinavir and the onset of such cutaneous effects was striking. This was confirmed by the regression of symptoms in those patients who later discontinued indinavir. The emerging side effects of protease inhibitors require a multidisciplinary team for adequate diagnosis and treatment. Cutaneous toxicity involving the patient's own body image has a peculiar influence on compliance to the treatment and the patient's quality of life.

Adult↗

[Cutaneous and mucosal manifestations of inflammatory bowel diseases].

The rheumatological, ophthalmological- and dermatological complications are the most common ones among the extraintestinal manifestations of inflammatory bowel diseases (IBD). The incidence of skin manifestations is estimated to be 15-20% in case of Crohn's disease and 10% in case of ulcerative colitis. The so called specific lesions (perianal fissures, metastatic Crohn's disease), which are part of the skin symptoms associated with IBD, show a intimate connections with the bowel disease itself, as they histologically show granulomatous inflammation with epitheloid cells, similar to the ones seen in the intestines. The reactive lesion (erythema nodosum, pyoderma gangraenosum), that form the second main group of skin changes, can also be found is other systemic diseases, but they are more frequently associated with IBD than the average. Cutaneous manifestations may occur due to malabsorption or drug therapy. Finally, there are dermatoses (epidermolysis bullosa acquisitia, acne fulminans) which have a still questionable connection with IBD. Authors present an overview of the IBD's possible skin and mucosal symptoms and their prognostic significance and they demonstrate some rare common skin manifestations found among the IBD patients of Borsod Country (580 ulcerative colitis, 265 Crohn's disease) in the last 25 years.

Colitis, Ulcerative↗

Superficial granulomatous pyoderma.

We report the case of a 30-year-old man who, from the age of 16, presented with abscess-type lesions that turned into ulcers with a torpid course and with histopathologic characteristics of superficial granulomatous pyoderma. The patient has been followed for 6 years, during which time the only treatment necessary to prevent recurrence of lesions has been the administration of different doses of minocycline.

Adult↗

Cutaneous blastomycosis in New Brunswick: case report.

Blastomycosis is a fungal infection of immunocompetent hosts. We present a case of cutaneous blastomycosis acquired in New Brunswick, which provides evidence that this disease is endemic in Atlantic Canada. This case also demonstrates that the diagnosis of blastomycosis may be elusive. Perseverance, a high index of clinical suspicion and close cooperation with the microbiology laboratory may be required to diagnose this uncommon condition.

Blastomycosis↗