1/26. panniculitis revealing qualitative alpha 1 antitrypsine deficiency (MS variant).A 16-year-old girl presented painful, red, nodular lesions on the abdomen. A cutaneous biopsy showed inflammatory cell infiltrate and fibrosis in the dermis and in the septa with isolated adipocyte lobules. alpha1-antitrypsin level was found to be normal but M1S phenotype of alpha1-antitrypsin was determined by isoelectric focusing in polyacrylamide gel. alpha1-antitrypsin level was normal for her family but M2S phenotype was found in her father. Alpha 1-antitrypsin (alpha1 AT) deficiency is a common hereditary disorder of Caucasians. The locus is pleiomorphic and 75 alleles have been identified. Numerous pathological mutations can be classified by the mechanisms which cause the deficiency. The major clinical importance of this deficiency is emphysema and liver disease. panniculitis is rarely reported and seems to occur principally for the ZZ or MZ phenotype and for low levels of alpha1 AT. MS phenotype has been more rarely reported and triggering agents such as trauma and infections must be present. However, normal levels of alpha1 AT in the serum have previously been reported as in our case, and we suggest the study of alpha1 AT phenotype even if the plasma level is normal.- - - - - - - - - - ranking = 1keywords = antitrypsin, alpha, deficiency (Clic here for more details about this article) |
2/26. 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 = 0.6266541874426keywords = antitrypsin, alpha, deficiency (Clic here for more details about this article) |
3/26. 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 = 3.4327380456338keywords = antitrypsin deficiency, antitrypsin, alpha, deficiency (Clic here for more details about this article) |
4/26. 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 = 3.4300687506089keywords = antitrypsin deficiency, antitrypsin, deficiency (Clic here for more details about this article) |
5/26. 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 = 2.7468676712519keywords = antitrypsin deficiency, antitrypsin, alpha, deficiency (Clic here for more details about this article) |
6/26. Nasal-type natural killer cell lymphoma preceded by benign panniculitis arising in an asymptomatic HTLV-1 carrier.We report a case of an Epstein-Barr virus (EBV)-associated nasal-type natural killer cell lymphoma (NKCL) preceded by benign panniculitis, which arose in a 48-year-old woman with an asymptomatic human T-cell leukemia/lymphoma virus type-1 (HTLV-1) infection. A biopsy of the initial panniculitis lesion demonstrated lobular panniculitis with a germinal center composed of benign mononuclear cells with a phenotype of CD4 CD45RO CD5sCD3 cCD3 epsilon T-cell intracellular antigen-1 (TIA-1)- and granzyme B-. One year after oral prednisolone therapy, the patient developed subcutaneous nodules composed of atypical lymphoid cells with a phenotype of CD4-CD45RO CD56 sCD3-cCD3 epsilon (TIA-1) and granzyme B . In the initial panniculitis lesion, neither EBV-encoded rna (EBER-1) nor clonal proliferation of EBV-infected cells was identified. In later lesions, however, a large number of atypical cells were positive for EBER-1, and a clonal expansion of EBV-infected cells was detected. No clonal rearrangement of T-cell receptor-alpha, -beta, or -gamma genes was found in either specimen. This patient was an asymptomatic carrier of human T-cell leukemia/lymphoma virus type-1 (HTLV-1) without clonal integration of proviral HTLV-1 in neither the peripheral blood nor the skin lesions. These observations suggest that EBV-associated NKCL occurred subsequently in the clinical course of benign panniculitis under the influence of immunosuppression caused by prednisolone treatment and HTLV-1 infection.- - - - - - - - - - ranking = 0.00053385900498467keywords = alpha (Clic here for more details about this article) |
7/26. 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 = 4.4906394070379keywords = antitrypsin deficiency, antitrypsin, alpha, deficiency (Clic here for more details about this article) |
8/26. 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 = 0.62123630948381keywords = antitrypsin, alpha (Clic here for more details about this article) |
9/26. Small vessel vasculitis and relapsing panniculitis in tumour necrosis factor receptor associated periodic syndrome (TRAPS).case reports: A 66 year old female patient had relapsing fever and non-suppurative panniculitis suggestive of enigmatic "Weber-Christian disease" (WCD). Antineutrophil cytoplasmic antibodies with specificity for human leucocyte elastase (HLE-ANCA) were detected. A biopsy showed small vessel vasculitis and panniculitis. A 53 year old man had recurrent episodes of abdominal pain, erythematous rash, and myalgia. Fever attacks had stopped a few years ago. A biopsy showed panniculitis and fasciitis. In both patients mutations (R92Q, T50M) of the tumour necrosis factor receptor super family (TNFRSF) 1A gene were disclosed. Mutations of the TNFRSF 1A gene are the cause of tumour necrosis factor receptor associated periodic syndrome (TRAPS). Both patients responded favourably to treatment with the human soluble p75 TNF alpha receptor fusion protein etanercept (2 x 25 mg subcutaneously/week). DISCUSSION: Small vessel vasculitis and panniculitis have not been reported in TRAPS so far. The cases underline the importance of TNF alpha regulation in inflammatory processes including vasculitis. Genetically determined causes of fever may account for some cases of WCD.- - - - - - - - - - ranking = 0.0010677180099693keywords = alpha (Clic here for more details about this article) |
10/26. Subcutaneous panniculitic T-cell lymphoma in a cardiac allograft recipient.BACKGROUND: Post-transplant lymphoproliferative disorder (PTLD) is the third leading cause of death in heart transplant patients beyond the immediate peri-operative period (Ouseph R, Denny DM, Erbeck KM. J Am Soc Echocardiogr 1998; 11: 758; Armitage JM, Kormos RL, Stuart RS, et al. J heart lung Transplant 1991; 10: 877; Swinnen LJ, Mullen M, Carr TJ, et al. blood 1995; 86: 3333; Ying AJ, Myerowitz D, Marsh WL. Ann Thorac Surg 1997; 64: 1822). The majority of PTLD cases are of B-cell origin whereas T-cell neoplasms have been reported as rare, aggressive, and late complications of solid-organ transplantation (Fatio R, Sutsch G, Mayer K, et al. Transplant Proc 1998; 30: 1118). CASE REPORT: A 50-year-old cardiac allograft heart transplant patient presented with subcutaneous nodules involving his trunk and extremities. RESULTS: light microscopy revealed features characteristic of subcutaneous panniculitic-like T-cell lymphoma. Immunohistochemical analysis showed expression for CD45RO, TIA-1, and focal CD3 positivity by tumor cells. flow cytometry performed on a subsequent subcutaneous nodule demonstrated an abnormal T-cell population with expression of CD3, CD8, CD56, and T-cell receptor alpha-beta, and no expression of CD4. T-cell gene rearrangement studies revealed a clonal population of cells with a bi-allelic gene rearrangement. CONCLUSION: We report a case of an unusual subtype of PTLD in a cardiac allograft recipient.- - - - - - - - - - ranking = 0.00053385900498467keywords = alpha (Clic here for more details about this article) |
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