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Internal Medicine (Tokyo, Japan) Jul 1999
Topics: Cyclosporine; Drug Resistance; Humans; Immunosuppressive Agents; Panniculitis, Nodular Nonsuppurative; Steroids
PubMed: 10435355
DOI: 10.2169/internalmedicine.38.522 -
Orphanet Journal of Rare Diseases Apr 2011Up to now alpha 1-antitrypsin (AAT) augmentation therapy has been approved only for commercial use in selected adults with severe AAT deficiency-related pulmonary... (Review)
Review
Up to now alpha 1-antitrypsin (AAT) augmentation therapy has been approved only for commercial use in selected adults with severe AAT deficiency-related pulmonary emphysema (i.e. PI*ZZ genotypes as well as combinations of Z, rare and null alleles expressing AAT serum concentrations <11 μmol/L). However, the compassionate use of augmentation therapy in recent years has proven outstanding efficacy in small cohorts of patients suffering from uncommon AAT deficiency-related diseases other than pulmonary emphysema, such as fibromyalgia, systemic vasculitis, relapsing panniculitis and bronchial asthma. Moreover, a series of preclinical studies provide evidence of the efficacy of AAT augmentation therapy in several infectious diseases, diabetes mellitus and organ transplant rejection. These facts have generated an expanding number of medical applications and patents with claims for other indications of AAT besides pulmonary emphysema. The aim of the present study is to compile and analyze both clinical and histological features of the aforementioned published case studies and reports where AAT augmentation therapy was used for conditions other than pulmonary emphysema. Particularly, our research refers to ten case reports and two clinical trials on AAT augmentation therapy in patients with both AAT deficiency and, at least, one of the following diseases: fibromyalgia, vasculitis, panniculitis and bronchial asthma. In all the cases, AAT was successfully applied whereas previous maximal conventional therapies had failed. In conclusion, laboratory studies in animals and humans as well as larger clinical trials should be, thus, performed in order to determine both the strong clinical efficacy and security of AAT in the treatment of conditions other than pulmonary emphysema.
Topics: Alleles; Asthma; Clinical Trials as Topic; Fibromyalgia; Humans; Panniculitis; Pulmonary Emphysema; Vasculitis, Leukocytoclastic, Cutaneous; alpha 1-Antitrypsin; alpha 1-Antitrypsin Deficiency
PubMed: 21486454
DOI: 10.1186/1750-1172-6-14 -
Skin Research and Technology : Official... Mar 2022
Topics: Erythema Nodosum; Humans; Panniculitis
PubMed: 34813122
DOI: 10.1111/srt.13112 -
The Turkish Journal of Pediatrics 2019Açarı C, Ünsal E, Hakgüder G, Soylu A, Özer E. Pediatric mesenteric panniculitis: three cases and a review of the literature. Turk J Pediatr 2019; 61: 798-803.... (Review)
Review
Açarı C, Ünsal E, Hakgüder G, Soylu A, Özer E. Pediatric mesenteric panniculitis: three cases and a review of the literature. Turk J Pediatr 2019; 61: 798-803. Mesenteric panniculitis is an inflammatory and fibrotic process in the mesenteric adipose tissue with unknown etiology. It is rarely seen in general, particularly in children. Etiology is unknown, and pathophysiology is not clear. Factors that trigger the disease are malignancy, tuberculosis, trauma, medications and past surgical interventions. There is no pediatric case series in the literature except single case reports. This paper consists of 3 cases: The first case is a 5-month-old girl, the youngest patient in the literature, who was referred to a pediatric surgeon with vomiting and abdominal distention. She had diffused intraabdominal fluid and mesenteric panniculitis documented by perioperative biopsy. The second case had acute abdominal pain with perforated appendicitis, who eventually had mesenteric panniculitis in the evaluation of the pathological specimen. The last case had a diagnosis of polyarticular juvenile idiopathic arthritis (JIA), successfully treated with etanercept, and has been in remission for 2 years. Interestingly, in one of her routine visits, she had pallor, anemia and renal failure. Bilateral hydronephrosis was detected. Magnetic resonance imaging (MRI) of the abdomen revealed retroperitoneal fibrosis, and mesenteric panniculitis was the histopathological diagnosis.
Topics: Abdominal Pain; Biopsy; Child; Female; Humans; Infant; Magnetic Resonance Imaging; Male; Panniculitis, Peritoneal; Vomiting
PubMed: 32105017
DOI: 10.24953/turkjped.2019.05.024 -
Medicine Jan 1996The authors propose to encompass under the designation of "fasciitis-panniculitis syndromes" (FPS) a group of disorders characterized by induration of the skin due to... (Review)
Review
The authors propose to encompass under the designation of "fasciitis-panniculitis syndromes" (FPS) a group of disorders characterized by induration of the skin due to chronic inflammation and fibrosis of the subcutaneous septa and muscular fascia. The prototype of the FPS is eosinophilic fasciitis. Thirty-two consecutive patients with FPS were cared for at the author's hospital during a 10-year period. The association of the FPS with other diseases, clinical presentations, histologic features, and response to treatment were analyzed. Idiopathic FPS, that is, eosinophilic fasciitis, was diagnosed in 14 patients. In the remaining 18 cases, the FPS were ascribed to vascular disorders (n = 6), infections (n = 6), and neoplastic disorders (n = 3), while trauma, insect bites, and Sweet syndrome antedated the FPS in 1 patient each. The lesions had a sleeve-like distribution in 20 patients, plaque-like distribution in 7, and a combined pattern in 5. Skin biopsies revealed lesions in the deep subcutaneous layers with the pathologic triad of septal and fascial fibrosis, chronic inflammatory infiltration, and small-vessel vasculopathy. Spontaneous improvement occurred in 4 cases. Following cimetidine monotherapy, complete remission was achieved in an additional 3 of 5 patients. The concept of the FPS serves to advance our understanding on several fronts: emphasizing the clinical and etiologic diversity; recognizing a stereotypic tissue reaction pattern; highlighting the panniculitis in addition to the fasciitic component; and describing a similar response to drug therapy in different clinical settings. Based on the results of the present series, cimetidine may be recommended as first-line treatment.
Topics: Adipose Tissue; Adrenal Cortex Hormones; Cimetidine; Diagnosis, Differential; Fasciitis; Histamine H2 Antagonists; Humans; Lymphocytes; Panniculitis; Skin; Syndrome
PubMed: 8569470
DOI: 10.1097/00005792-199601000-00002 -
Postgraduate Medical Journal Nov 1990A 33 year old man developed fever, malaise, jaundice, pancytopenia, coagulation abnormalities, hepatomegaly, pleural effusions and a subcutaneous lump. Biopsies revealed... (Review)
Review
A 33 year old man developed fever, malaise, jaundice, pancytopenia, coagulation abnormalities, hepatomegaly, pleural effusions and a subcutaneous lump. Biopsies revealed a lobular panniculitis with the presence of cytophagic histiocytes; erythrophagocytosis was also demonstrated in the liver and bone marrow. Despite the use of chemotherapy (CHOP) his clinical condition deteriorated and he died 5 months after presentation. This illness is consistent with the recently described syndrome of histiocytic cytophagic panniculitis.
Topics: Adipose Tissue; Adult; Bone Marrow; Histiocytes; Humans; Male; Panniculitis
PubMed: 2267213
DOI: 10.1136/pgmj.66.781.958 -
Indian Journal of Dermatology,... 2015
Topics: Arthritis; Humans; Male; Pancreatitis; Panniculitis
PubMed: 25900941
DOI: 10.4103/0378-6323.155578 -
Oxidative Medicine and Cellular... 2020This study was conducted to assess the protective effect of extract of match (EM) on high-fat diet- (HFD-) induced cognitive deficits in male C57BL/6 mice. It was found...
This study was conducted to assess the protective effect of extract of match (EM) on high-fat diet- (HFD-) induced cognitive deficits in male C57BL/6 mice. It was found that EM improved glucose tolerance status by measuring OGTT and IPGTT with HFD-induced mice. EM protected behavioral and memory dysfunction in Y-maze, passive avoidance, and Morris water maze tests. Consumption of EM reduced fat mass, dyslipidemia, and inflammation in adipose tissue. Also, EM ameliorated hepatic and cerebral antioxidant systems. EM improved the cerebral cholinergic system by regulating ACh contents and expression of AChE and ChAT. Also, EM restored mitochondrial function in liver and brain tissue. EM attenuated hepatic inflammatory effect, lipid synthesis, and cholesterol metabolism by regulating the protein expression of TNF-, TNFR1, -IRS-1, -JNK, IL-1, iNOS, COX-2, HMGCR, PPAR, and FAS. Finally, EM regulated cognitive function and neuroinflammation in the whole brain, hippocampus, and cerebral cortex by regulating the protein expression of -JNK, -Akt, -tau, A, BDNF, IDE, COX-2, and IL-1. These findings suggest that EM might be a potential source of functional food to improve metabolic disorder-associated cognitive dysfunction.
Topics: Adipose Tissue; Animals; Cognitive Dysfunction; Diet, High-Fat; Dyslipidemias; Gene Expression Regulation; Inflammation; Male; Memory Disorders; Mice; Panniculitis; Tea
PubMed: 33312340
DOI: 10.1155/2020/8882763 -
Dermatology Online Journal Apr 2014Panniculitis, inflammation of the subcutaneous fat, is a relatively uncommon condition that usually presents with inflammatory nodules or plaques. Erythema nodosum (EN)... (Review)
Review
Panniculitis, inflammation of the subcutaneous fat, is a relatively uncommon condition that usually presents with inflammatory nodules or plaques. Erythema nodosum (EN) is clinically the most frequent form of panniculitis and is considered a reactive process that may be triggered by a wide variety of stimuli. Whilst up to 55% of EN is considered idiopathic, the most common causes include infections, drugs, systemic illnesses such as sarcoidosis and inflammatory bowel disease, pregnancy, and malignancy. EN typically presents in the teens and 20s, and is seen more commonly in females. It is often preceded by a non-specific prodrome of one to three weeks, which may include fever, malaise, and symptoms of an upper respiratory tract infection. Cutaneous lesions then follow, typically localized on the extensor aspect of the limbs. The lesions are painful rounded or oval, slightly raised, non-ulcerative red nodules. The exact pathogenesis of EN is not understood, although is thought to result from deposition of immune complexes in the venules of the septae in subcutaneous fat, causing a neutrophilic panniculitis. The classical histopathological picture is of a septal panniculitis without vasculitis. However, the pathological features vary with the chronology of the lesions. Even without specific therapy for a causative condition, EN typically resolves without treatment. Therefore, symptomatic support is adequate for the majority of patients.
Topics: Dermatologic Agents; Diagnosis, Differential; Erythema Nodosum; Humans; Panniculitis; Sex Factors; Skin
PubMed: 24746312
DOI: No ID Found -
Journal of Gastrointestinal and Liver... Jun 2023A 67-year-old man with previous cardiovascular disease was referred to our consultation due to a 5-month history of recurrent epigastric pain. Esophagogastroduodenoscopy...
A 67-year-old man with previous cardiovascular disease was referred to our consultation due to a 5-month history of recurrent epigastric pain. Esophagogastroduodenoscopy and full blood workup presented no alterations. CT scan showed an irregularly shaped mass at the root of the mesentery, measuring 40x25x47mm, with spiculated contours and retractile behaviour (a). Simultaneous densification of the adjacent fat and infracentimetric ganglionic formations scattered throughout the mesentery were shown. Surgical biopsy revealed extensive storiform fibrosclerosis, with the presence of interstitial lymphoplasmocytic infiltrate and obliterative phlebitis (b); the plasma cells had mostly IgG expression, with IgG4:IgG ratio >40% (c), accounting for more than 30- 40 IgG4 plasma cells per field. The serum IgG4 level was 137mg/dL. A diagnosis of IgG4-related sclerosing mesenteritis was made, without other organ involvement. Prednisolone (0.6mg/kg/d) improved partially the abdominal pain, so steroid sparing strategy with off-label rituximab was associated. Due to its low prevalence, the understanding of this entity is scarce, and its diagnosis is challenging. Unlike other manifestations of IgG4-related disease, the intra-abdominal disease is identified in later stages, due to unspecific symptoms. This case aims to raise awareness about this condition as a differential diagnosis of abdominal pain.
Topics: Male; Humans; Aged; Panniculitis, Peritoneal; Immunoglobulin G; Prednisolone; Abdominal Pain; Mesentery
PubMed: 37345601
DOI: 10.15403/jgld-4921