1/20. Treatment of alpha1-antitrypsin-deficiency panniculitis with minocycline.A small proportion of patients with alpha1-antitrypsin (alpha1AT) deficiency experience recurrent ulcerating panniculitis. Studies suggest that alpha1AT-deficiency panniculitis reflects an uncontrolled inflammatory process because of the serum's incapacity to neutralize proteolytic enzymes released by leukocytes in the skin. dapsone is considered the treatment of choice for this entity, but it is limited by its side effects, especially hematologic ones. tetracyclines have anticollagenase and anti-inflammatory activity and have been used successfully to treat this type of panniculitis. We report the case of a 42-year-old woman with recurrent alpha1AT-deficiency panniculitis who did not tolerate the side effects of dapsone or systemic corticosteroid. minocycline treatment led to disappearance of lesions, and long-term administration prevented recurrences without evident negative side effects. We consider minocycline a safe treatment that allows control of alpha1AT-deficiency panniculitis.- - - - - - - - - - ranking = 1keywords = panniculitis (Clic here for more details about this article) |
2/20. Severe panniculitis caused by homozygous ZZ alpha1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin).Severe panniculitis caused by alpha1-antitrypsin deficiency is very rare even though the ZZ phenotype occurs in 1 : 3500 of the population of northern europe. We describe a 33-year-old woman with rapidly progressing panniculitis and extensive skin necrosis with multiple life-threatening complications. Initial treatment followed by maintenance therapy with human purified enzyme (Prolastin, Bayer, Bridgend, U.K.) has been life-saving.- - - - - - - - - - ranking = 0.66666666666667keywords = panniculitis (Clic here for more details about this article) |
3/20. Alpha1-antitrypsin deficiency panniculitis.Alpha1-antitrypsin deficiency panniculitis (A1ADP) is a rare form of panniculitis that affects children and adults. Clinical and histologic features, precipitating factors, and treatments are discussed.- - - - - - - - - - ranking = 0.66666666666667keywords = panniculitis (Clic here for more details about this article) |
4/20. panniculitis secondary to extravasation of clarithromycin in a patient with alpha 1-antitrypsin deficiency (phenotype PiZ).alpha 1-antitrypsin deficiency (AATD) is known to be associated with panniculitis. Although reports of this association are rare, the true incidence may be unappreciated because of underdiagnosis of AATD. We report a case of panniculitis occurring in a 34-year-old woman with severe AATD following the extravasation of clarithromycin infused intravenously for treatment of community-acquired pneumonia. Resolution occurred with conservative management. The histopathology and management of this unusual condition are discussed, with a review of the literature.- - - - - - - - - - ranking = 0.22222222222222keywords = panniculitis (Clic here for more details about this article) |
5/20. Alpha-1-antitrypsin deficiency associated with panniculitis treated with plasma exchange therapy.BACKGROUND: Alpha-1-antitrypsin is the principal serum protease inhibitor. In addition to the well-recognized association with early-onset emphysema and cirrhosis, alpha-1-antitrypsin deficiency may be associated with panniculitis. The treatment of this type of panniculitis presents a significant challenge. Previous attempts using immunosuppressive, anti-inflammatory, and cytotoxic drugs have shown variable results. AIM: To report a case of alpha-1-antitrypsin deficiency-associated panniculitis treated with plasma exchange therapy. methods: A 23-year-old patient developed painful red nodules on her thighs and buttocks with spontaneous ulceration and discharge of oily fluid. A skin biopsy specimen showed septal and lobular panniculitis. The serum alpha-1-antitrypsin level was 22 mg/dL. She was treated with plasma exchange therapy. RESULTS: Treatment of this patient with plasma exchange therapy led to the control of the cutaneous lesions. CONCLUSIONS: plasma exchange therapy represents an alternative treatment which restores serum and tissue alpha-1-antitrypsin levels. This method is proposed because of its clinical benefits and greater availability.- - - - - - - - - - ranking = 0.88888888888889keywords = panniculitis (Clic here for more details about this article) |
6/20. Alpha-1-antitrypsin associated panniculitis: the MS variant.Over 90 mutant alleles of the alpha-1-antitrypsin (AAT) gene are recognized and classified by mobility on an acid starch gel. The four major categories include: F=fast, M=medium, S=slow, Z=very slow. Among 41 reported cases of AAT panniculitis, most have the ZZ phenotype with AAT levels below normal. We report two cases of AAT panniculitis with MS phenotype and normal AAT levels. In addition, we review the pathophysiology, epidemiology, and extracutaneous manifestations of AAT disease and propose a diagnostic algorithm for ulcerative panniculitis. A 42-year-old man presented with a solitary plaque on the left thigh exacerbated by trauma or excessive activity. The lesion frequently suppurated with a yellowish oily material. Twenty years before, he had fractured his left femur which was repaired with a metal plate. x-rays, histology with special stains for organisms, and cultures were negative. AAT phenotype was MS and AAT value was normal. A 43-year-old woman presented with multiple plaques on the proximal extremities which suppurated with exercise or trauma. AAT phenotype was MS and AAT level was normal. Histologic exam for both patients showed a dense neutrophilic infiltrate with septal and lobular panniculitis and areas of necrobiosis in the lower reticular dermis.- - - - - - - - - - ranking = 0.88888888888889keywords = panniculitis (Clic here for more details about this article) |
7/20. Alpha1-antitrypsin deficiency-associated panniculitis: case report and review of treatment options.Alpha(1)-antitrypsin deficiency, a relatively frequent mutation in the population, is associated with the development of panlobular emphysema and liver cirrhosis. The deficiency is in rare cases associated with the development of panniculitis, and very differentiated clinical courses have been reported in the literature. We report a case of panniculitis in a patient with alpha(1)-antitrypsin deficiency and describe briefly the pathophysiology of the disease and current treatment possibilities.- - - - - - - - - - ranking = 0.66666666666667keywords = panniculitis (Clic here for more details about this article) |
8/20. Alpha1-antitrypsin deficiency: incidence and implications.Alpha1-antitrypsin (AAT) deficiency is an underrecognized inherited disorder with pulmonary and hepatic implications for both adults and neonates. Clinical expressions of AAT deficiency are seen in the lung, liver, and the skin, with considerable variability in the severity of clinical disease. AAT deficiency accounts for nearly 3% of all cases of chronic obstructive pulmonary disease and is responsible for early-onset emphysema in nonsmokers. Ten to twenty percent of affected neonates develop significant liver disease. panniculitis, a rare skin complication of AAT deficiency, is characterized by acute inflammatory infiltrate and fat necrosis. While we concentrate on the pulmonary aspect of AAT deficiency, we have included discussion of liver disease and panniculitis. critical care and advanced practice nurses will benefit from gaining a better understanding of the causes, pathophysiology, diagnosis, and treatment of this disorder.- - - - - - - - - - ranking = 0.11111111111111keywords = panniculitis (Clic here for more details about this article) |
9/20. Use of anti-collagenase properties of doxycycline in treatment of alpha 1-antitrypsin deficiency panniculitis.Three patients (a 71-year-old man and 2 women, 73 and 50 years, respectively) with recurrent panniculitis associated with alpha 1-antitrypsin deficiency are presented. Because the concept of chronic and exaggerated inflammatory response in the patients with alpha 1-antitrypsin deficiency is based on the theory of protease-antiprotease imbalance, we suggest that tetracyclines will alleviate this condition. Indeed, tetracyclines were found to inhibit collagenase activity. The total remission of the condition in these 3 patients underlines for the first time the effects of doxycycline on a condition characterized by deficiency of the antiprotease system. A review of the 23 reported cases of panniculitis associated with alpha 1-antitrypsin deficiency is presented in table form.- - - - - - - - - - ranking = 0.66666666666667keywords = panniculitis (Clic here for more details about this article) |
10/20. alpha 1-antitrypsin deficiency-associated panniculitis: case report and review of the literature.Alpha 1-antitrypsin is the primary serum proteinase inhibitor. alpha 1-antitrypsin deficiency, especially the ZZ genotype, has been linked mainly to emphysema and cirrhosis; it is also associated with paniculitis. A case of alpha 1-AT-associated panniculitis was documented in a 13-year-old girl in whom a deficiency of the enzyme was known to be present from infancy. This is unusual, since alpha 1-AT panniculitis previously was described in older patients without prior knowledge of the duration of the deficiency. Our patient developed erythematous, subcutaneous nodules subsequent to trauma, which later developed into deep, painless ulcers. We report this case so that the condition may be suspected in patients with panniculitis. The diagnosis may be confirmed by measuring quantitative alpha 1-AT serum levels and by enzyme genotyping. The treatment of choice is dapsone.- - - - - - - - - - ranking = 0.77777777777778keywords = panniculitis (Clic here for more details about this article) |
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