Pancreatitis, panniculitis and polyarthritis syndrome: A case report

BACKGROUND Pancreatitis,panniculitis,and polyarthritis(PPP)syndrome is a rare form of pancreatic disease.It is characterized by bullous erythematous skin lesions and arthritis,and both are triggered by pancreatic malfunction.Few cases have been described in the literature thus far.Due to the inconsistency in its clinical presentation,its diagnosis can be a challenge.Early therapy initiation is essential to reduce mortality;however,there is currently no gold standard for treatment.CASE SUMMARY A 66-year-old polymorbid male patient presented with several superficial abscesses on both lower legs and painful swelling in the knee.Treatment for septic arthritis and septic skin infection over several weeks failed.His general condition deteriorated gradually and worsened with sudden onset of abdominal pain.A diagnosis of necrotizing pancreatitis was made.He subsequently underwent a laparotomy and drainage of the pancreas.Eventually,our patient improved,and his abdominal complaints,knee pain,and dermal lesions resolved.CONCLUSION PPP syndrome is rare and easily misdiagnosed,as abdominal symptoms may be delayed or absent.Clinicians should consider PPP syndrome if they encounter refractory panniculitis in combination with joint infection.

Mesenteric panniculitis: Various presentations and treatment regimens

Mesenteric panniculitis is a rare, benign and chronic f ibrosing inflammatory disease that affects the adipose tissue of the mesentery of the small intestine and colon. The specific etiology of the disease is unknown. The diagnosis is suggested by computed tomography and is usually confirmed by surgical biopsies. Treatment is empirical and based on a few selected drugs. Surgical resection is sometimes attempted for def initive therapy, although the surgical approach is often limited. We report two cases of mesenteric panniculitis with two different presentations and subsequently varying treatment regimens. Adequate response was obtained in both patients. We present details of these cases as well as a literature review to compare various presentations, etiologies and potential treatment modalities.

Clinical significance of mesenteric panniculitis-like abnormalities on abdominal computerized tomography in patients with malignant neoplasms

AIM To clarify the association of malignancy with mesenteric panniculitis-like changes on computed tomography(CT).METHODS All abdominal CT scans performed at North Shore University HealthS ystem showing mesenteric panniculitis from January 2005 to August 2010 were identified in the Radnet(Rad Net Corporation, Los Angeles, CA) database. Patients with a new or known diagnosis of a malignancy were included for this analysis. Longitudinal clinical histories were obtained from electronic medical records.RESULTS In total, 147794 abdominal CT scans were performed during the study period. Three hundred and fiftynine patients had mesenteric panniculitis(MP)-like abnormalities on their abdominal CT. Of these patients, 81 patients(22.6%) had a known history of cancer at the time of their CT scan. Nineteen(5.3%) had a new diagnosis of cancer in concurrence with their CT, but the majority of these(14/19, 74%) were undergoing CT as part of a malignancy evaluation. Lymphomas were the most common cancers associated with MPlike findings on CT(36 cases, 36%), with follicular lymphoma being the most frequent subtype(17/36). A variety of solid tumors, most commonly prostate(7) and renal cell cancers(6) also were seen. CT follow up was obtained in 56 patients. Findings in the mesentery were unchanged in 45(80%), worsened in 6(11%), and improved in 5 patients(9%). Positron emission tomography(PET) scans performed in 44 patients only showed a positive uptake in the mesenteric mass in 2 patients(5%). CONCLUSION A new diagnosis of cancer is uncommon in patients with CT findings suggestive of MP. MP-like mesenteric abnormalities on CT generally remain stable in patients with associated malignancies. PET scanning is not recommended in the evaluation of patients with mesenteric panniculitis-like findings on CT.

Pancreatic panniculitis and solid pseudopapillary tumor of the pancreas: A case report

Solid pseudopapillary tumor of the pancreas(SPTP), also known as solid and papillary epithelial neoplasm of the pancreas, is a rare pancreatic exocrine tumor that is difficult to diagnose before surgery. Pancreatic panniculitis is a rare type that occurs in less than 3% of all patients with pancreatic diseases. We here report a 19-year-old woman who presented with persistent left upper quadrant pain without obvious cause for 1 d. The patient also developed subcutaneous nodules involving lower abdomen bilaterally and lower limbs, and subcutaneous nodules were pathologically diagnosed as pancreatic panniculitis. Plain abdominal computed tomography revealed a soft-tissue mass in the body and tail of the pancreas, which was closely associated with the gastric wall. Contrast-enhanced ultrasound showed inhomogeneous echogenicity in the anterior pancreatic body, which had blurred parenchymal demarcation of the body and tail of the pancreas. Contrast-enhanced abdominal computed tomography revealed a mixed density mass with solid and cystic components in the body and tail of the pancreas, and the solid component was markedly enhanced. The lesion was pathologically diagnosed as SPTP after laparoscopic resection. Clinicians should be aware of the clinical manifestation, diagnosis, and treatment of pancreatic panniculitis and SPTP.

Chronic abdominal pain secondary to mesenteric panniculitis treated successfully with endoscopic ultrasonography-guided celiac plexus block: A case report

Mesenteric panniculitis is a chronic illness that is characterized by fibrosing inflammation of the mesenteries that can lead to intractable abdominal pain. Pain control is a crucial component of the management plan. Most patients will improve with oral corticosteroids treatment, however, some patients will require a trial of other immunosuppressive agents, and a minority of patients will continue to have refractory disease. Endoscopic ultrasound guided celiac plexus block is used frequently to control abdominal pain in patients with pancreatic pathology. To our knowledge there are no case reports describing its use in mesenteric panniculitis patients with refractory abdominal pain.

Relationship between abdominal trauma or surgery and mesenteric panniculitis

Mesenteric panniculitis is a rare disease characterized by chronic non-specific inflammation of mesenteric fat tissue.Several etiologic and/or associated factors have been reported in the literature so far.Although trauma or surgery is one of the potential etiologic factors for mesenteric panniculitis,to the best of our knowledge,no strong correlation has been shown in the literature until now.

Pancreatitis, panniculitis, and polyarthritis syndrome caused by pancreatic pseudocyst: A case report

BACKGROUND Panniculitis, polyarthritis, and pancreatitis (PPP) syndrome is a triad comprising an extremely rare extra-pancreatic complication of pancreatic disease. Herein, we describe a patient with PPP syndrome caused by fistula formation between the inferior vena cava (IVC) and pancreatic pseudocyst. CASE SUMMARY A 64-year-old man visited the hospital with bilateral leg pain that began one week prior. He had no specific diseases, except hypertension. His vital signs were normal. Blood test revealed the following findings: White blood cell count, 28690/μL;amylase level, 9055 U/L;lipase level, 2089 U/L;and C-reactive protein level, 12.94 mg/dL. Computed tomography of the pancreas revealed recent acute pancreatitis. Nonsteroidal anti-inflammatory drugs were administered with no improvement. After steroid administration, pain slightly improved. Skin lesions were diagnosed as panniculitis. Bone scan and knee magnetic resonance imaging revealed osteoarthritis and bone marrow infarctions. Surgical treatment was considered;total pancreatectomy with splenectomy was performed. A pseudocyst was present posterior to the head of the pancreas, forming a fistula with the suprarenal IVC. After surgery, amylase and lipase levels decreased. However, the patient died of an uncontrolled infection on the 13th postoperative day. CONCLUSION PPP syndrome should be suspected when accompanied by skin and joint lesions. Delays in diagnosis could have catastrophic consequences.

Pancreatic panniculitis and elevated serum lipase in metastasized acinar cell carcinoma of the pancreas: A case report and review of literature

BACKGROUND Pancreatic panniculitis is an extremely rare condition associated with different underlying pancreatic disorders and characterized by subcutaneous fat necrosis induced by elevated serum lipase levels.These lesions usually affect the lower extremities and may precede abdominal symptoms of pancreatic disease.Acinar cell carcinoma(ACC)of the pancreas is a rare pancreatic neoplasm,accounting for only 1%-2%of pancreatic tumors in adults.We present the case of a 72-year-old man with ACC of the pancreatic head and synchronous liver metastases.Both the primary tumor and liver metastases were resected.Serum lipase was elevated before surgery and decreased to normal postoperatively.Rising serum lipase levels at follow-up led to the diagnosis of hepatic recurrence.This disease progression was then accompanied by pancreatic panniculitis,with subcutaneous fat necrosis and acute arthritis.To the best of our knowledge,only 4 cases have been reported in the literature and each showed a similar association of serum lipase levels with pancreatic panniculitis and progression of ACC.CONCLUSION Clinical symptoms and progression of ACC may correlate with serum lipase levels,suggesting potential usefulness as a follow-up biomarker.

Cytophagic Histiocytic Panniculitis with Encephaloclastic Changes:A Case Report and Literature Review

Cytophagic histiocytic panniculitis （CHP） was first described by Winkelmann and Bowie in 1980.vj It is a rare group of diverse illnesses involving benign and malignant proliferation of macrophages in various organs and tissues. It presents with subcutaneous panniculitis with or without a hemophagocytic syndrome （HPS）. It occurs predominantly in women （male： female ratio 1：1.3） between the years of 5-61 （average, 33.5）. The major clinical features are recurrent fever, multiple panniculitic lesions, anemia, leukopenia and coagulation abnormalities. In the later phase, liver dysfuction, serosal effusion, mucosal ulceration and hemorrhage may occur. Histological findings show activated histiocyte infiltration of the fat tissue. Cytologically the benign-looking histiocytes containing cell fragments （bean-bag cells） are very typical. CHP has a broad spectrum from mild to severe. Benign CHP is selflimiting and sensitive to treatment, but up to now there is no effective therapy for malignant CHP. We report here a case of progressive and fatal cytophagic histiocytic panniculitis in a young woman who had encephaloclastic changes immediately prior to her death.

Managing panniculitis in alpha-1 antitrypsin deficiency: Systematic review of evidence behind treatment

AIM To systematically review literature for management of alpha-1 antitrypsin deficiency(AATD) panniculitis. METHODS Multiple databases were searched using combinations of pertinent terms. Articles were selected describing panniculitis treatment in patients with AAT < 11 μmol and/or PiZZ genotype, with no language limitation. All relevant articles were accessed in full text. Independent review of abstracts and full manuscripts was conducted by 2 reviewers, and quality assessment by one reviewer(checked by a second). Data extraction was conducted byone reviewer(checked by a second). Narrative synthesis only was conducted, as data were unsuitable for metaanalysis.RESULTS Thirty-two case reports and 4 case series were found. Augmentation therapy(infusions of plasma-derived AAT) was the most successful, with complete resolution of symptoms in all patients. Dapsone is a less expensive option, and it achieved clinical resolution in 62% of patients, but it is very poorly tolerated. Among other single-agent antibiotics, doxycycline was the most successful with complete clinical resolution seen in 33% of patients. Immunosuppressants were largely unsuccessful; 80% of patients exhibited no response. Liver transplantation and therapeutic plasma exchange displayed complete resolution in 66% of patients. Other strategies, such as non-steroidal anti-inflammatory drugs or antibiotics other than dapsone did not show sufficient response rates to recommend their use. Authors note the risk of bias imposed by the type of evidence(case reports, case series) available in this field.CONCLUSION Dapsone is the recommended first line therapy for AATD panniculitis, followed by augmentation therapy. Plasma exchange may be an alternative in the setting of rapidly progressive disease.

Treatment of Edematofibrosclerotic Panniculitis (Cellulite) and of Localised Adiposity with Extracts of Birch, Melilotus, Orthosiphon, Bromelin and Red Vine Leukocyanidine: Results from a Single-Centre Observational Prospective Study

The Edematofibrosclerotic Panniculitis (EFP) and the Localised Adiposity (LA) have controversial pathogenesis and therapy. A single-centre observational study has been performed to assess the potential efficacy, the limitations and the side effects of the medical treatment of EFP and AL by means of a multicomponent nutraceutical product. Fifty female subjects have been enrolled, mean age and BMI of the patients were 46 years and 24.18 respectively. The subjects have been submitted to a two-month treatment with two tablets of a nutraceutical based on extracts of Birch (100 mg), Orthosiphon (100 mg), Red Vine Leukocyanidines (100 mg), Melilotus (5 mg) and Bromelin (150 mg). The patients have been investigated before the treatment and during the treatment (at 30 and 60 days) by means of: a) volumetry of the lower limbs with tape measurement;b) visual analogue scale assessment (VAS) of symptoms such as pain paresthesia/dysesthesia, heaviness, and of the daily diuresis;c) duplex ultrasound evaluation of the thickness of the adipose tissue at the peri-trochanteric level;d) multifrequency segmental bioimpedance (MSB) analysis for the tissue liquid component. Forty-five of the 50 enrolled subjects have completed the study and five patients have discontinued the treatment, four of which due to unexpected personal problems or health problems not related to the treatment itself and one for reporting gastrointestinal disorders (such as constipation/diarrhea) with the nutraceutical intake. At the end of the therapeutical cycle, volumetry of the limbs decreased of 7% (right) and of 6% (left). Pre/post treatment VAS assessment of symptoms showed the following mean figures: heaviness from 5.10 to 0.88;paresthesia/dysesthesia from 0.90 to 0.00;pain from 2.03 to 0.12. Diuresis increased of 31%. Duplex-ultrasound assessment of the subcutaneous adipose layers at the trochanteric level showed a reduction of slightly more than 20% at the end of the treatment in both limbs. L-Dex bioimpedance values have been as follows: 3.97 at the enrolment;1.17 at the end of the treatment (reduction of the subcutaneous tissue fluid content). The present single-centre observational study proved that the tested nutraceutical multicomponent is effective and free of relevant side effects in the treatment of EFP and AL of the lower limbs. Larger cohort studies may possibly confirm the results of this preliminary experience.

Non-Mass Forming Isolated Omental Panniculitis: A Case Report

A 61-year-old man presenting with abdominal pain and fever refractory to antibiotics underwent diagnostic laparoscopy and non-mass-forming isolated omental panniculitis was identified. He presented with left-upper-quadrant abdominal pain. Laboratory data and the CT findings suggested intraabdominal bacterial disease in the splenic flexure, which we treated with antibiotics and fasting. He clinically improved once, but later relapsed with abdominal pain migration to the left-lower-quadrant. CT re-examination revealed no inflammation in the splenic flexure, but attenuation of adipose tissue in the greater omentum. We partially extracted the greater omentum during diagnostic laparoscopy and diagnosed omental panniculitis and administered steroids. He improved and was discharged three days after starting oral prednisone and is recurrence-free with a close follow-up. The characteristic CT feature of omentum panniculitis is a high-density fatty mass, but we noted only an attenuation of adipose tissue in the greater omentum. Diagnositic laparoscopy is useful for diagnosing this condition.

Abdominal panniculitis as a presentation of Munchausen Syndrome

Factitious disorders including Munchausen’s syn drome are encountered by all clinicians. A considerable number of cases of Munchausen’s syndrome are under-diagnosed in clinical practice. We present a 34-year-old man who was admitted with symptoms of epigastric pain, vomiting and peri-umbilical ecchymosis. Physical examination showed signs of inflammation in the abdominal wall with tenderness and guarding in the upper abdomen. However, various blood tests were unremarkable. Computed Tomography and demonstrated anterior abdominal wall panniculitis. After many investigations looking into various aetiological factors that could lead to fat necrosis, a diagnosis of Munchausen’s syndrome was made and the patient was discharged to the care of the local general doctor practice following psychiatric input.

Kerosene-Induced Panniculitis in Iraqi Patients

Background: Kerosene is a common household stuff that has been used as accidental oral poisoning material in children and as suicidal attempt in adults. In the last decade intradermal kerosene injection has been commonly used to induce dermatitis artefecta as a part of emotional upset. Objective: To evaluate the clinical cases of intradermal kerosene injection in Iraqi patients. Patients and Methods: This is a descriptive case study that had been conducted in Department of Dermatology Baghdad Teaching Hospital, Baghdad, Iraq during the period from Jan. 2003 to Dec. 2012. History and full clinical examination were performed including all sociodemographic aspects associated with this condition. Psychiatric evaluation was done for each patient. Results: All eleven patients had single lesion except that two had two lesions. They were distributed on accessible areas on the limbs. The exact diagnosis was not reached for at least few weeks after kerosene injection. The patients denied any kerosene injection, but after a while they all admitted that the cause of their rash, severe emotional tension was observed at the time of kerosene injection as they had sociopsychological disturbances. The initial rash was erythematous indurated tender plaque that was gradually increasing in size simulating the picture of panniculitis and then followed by rupture of lesion and associated pyoderma, forming chronic discharging ulcer. Patients were managed by topical and systemic antibiotics until complete resolution leaving a big ugly scar that was treated by topical steroids to improve its cosmetic appearance. Conclusions: Kerosene intradermal injection is an increasing problem among Iraqi adult females and it should be suspected in any patient with chronic discharging ulcer on accessible areas like limbs.

A Case Report of Lupus Panniculitis in a Hemodialysis Patient with Lupus Nephritis

Background: Systemic lupus erythematosus and lupus nephritis are relatively common autoimmune diseases that can cause damage to multiple systems. Aim: To investigate the lupus activity of patients with lupus nephritis on hemodialysis and the combined occurrence of lupus panniculitis. Case introduction: A patient with lupus nephritis on regular hemodialysis had a symmetrical hard mass under the skin of his abdomen. After surgical resection and pathological examination, she was diagnosed with lupus panniculitis and was treated with glucocorticoids and hydroxychloroquine. After that, no new subcutaneous masses appeared, and the unresectable part of the masses did not increase. Conclusion: 1) Lupus activities in patients with lupus nephritis entering end-stage renal disease still need to be paid attention to. 2) Lupus panniculitis can occur on the skin of the abdomen, and it needs to be differentiated from connective tissue panniculitis, sclerosing panniculitis and other diseases.

A Case Report of Mesenteric Panniculitis and Primary Sjogren’s Syndrome

Mesenteric Panniculitis is a benign fibro-inflammatory process involving adipose tissue of the mesentery. It is characterised by fat necrosis, chronic inflammation and fibrosis, causing thickening and shortening of the mesentery. Patients may present with localised abdominal pain, abdominal mass, intestinal obstruction and ischaemic colitis. We report a case of mesenteric panniculitis causing abdominal pain in a patient with active Primary Sjogren’s Syndrome. The rarity of this case makes it of interest. We review the current literature on mesenteric panniculitis and its association with connective tissue disease and inflammatory conditions. A 64-year-old Caucasian male presented in 1994 with dry mouth. A diagnosis of Primary Sjogren’s Syndrome (PSS) was made on salivary gland biopsy. In 2010 he presented with an exacerbation of his eye symptoms, muscle pain and fatigue. He complained of abdominal pain and night sweating, but denied any weight loss or change in bowel habit. There was no significant past medical history other than PSS. On examination he had a small right submandibular node and mild synovitis at the right proximal interphalangeal joint and carpometacarpal joint. Examination of the abdomen showed marked umbilical tenderness but no organomegally. Blood tests at this time showed an active inflammation: CRP of 61 (NR 5 mg/L), ESR 39 (NR 20 mm/s), strongly positive ENA Ro and La. IgG was elevated at 18.6 (NR 5.8-15.4), Complement was low at 0.17 (NR 0.18-0.6). An abdominal ultrasound scan demonstrated a 6 × 3 ×3 cmarea of diffuse homogenous fat encasing some mesenteric vessels in the area of focal tenderness. CT abdomen and pelvis showed oedematous mesenteric fat and lymph nodes in the jejunal small bowel mesentery, consistent with mesenteric panniculitis. Laparoscopic biopsy was discussed with the surgical team, but was felt not indicated as risk outweighed potential benefit. The patient was treated with a 9-week reducing course of oral steroids. His abdominal symptoms resolved although CT abdomen showed little improvement in mesenteric panniculitis. A review of the literature suggests that currently there is no standard treatment and management should be guided by patient symptoms. Mesenteric Panniculitis is rare;as a result evidence for treatment is limited to individual case reports. There is no clear link between symptom improvement and radiological resolution of mesenteric panniculitis. It has, therefore been suggested that follow-up imaging should be limited to those with persistent symptoms. Overall the prognosis for mesenteric panniculitis is good, up to half of patients do not require treatment, and recurrence of symptoms is uncommon.

Nodular Panniculitis with Hemophagocytic Lymphohistiocytosis

To the Editor:A 27-year-old Chinese man presented to the hospital complaining of red nodules on the chest and back that gradually enlarged with spiking fever up to 39℃.He had nodular panniculitis when he was 21 years old.The family history was silent.Physical examination on admission revealed skin pigmentation,skin nodules (four,2cm × 2cm,without movement and tenderness), and red rash without pain and pruritus over the body.Laboratory tests showed pancytopenia,especially for white blood cell (WBC) and platelet (PLT),raised triglycerides (TGs)and ferritin levels, liver enzyme abnormalities,decreased albumin value,and deranged coagulation profile.

Neutrophilic Panniculitis Associated With Myelodysplastic Syndrome/Myeloproliferative Neoplasm:A Case Report and Literature Review

Introduction:Neutrophilic panniculitis(NP)is a rare subtype of neutrophilic dermatosis,a group of neutrophilrich inflammatory skin disorders that can present in association with myeloid neoplasms.NP is defined by the presence of a neutrophilic infiltrate in the fat lobules of the subcutis in the absence of either infection or vasculitis.We herein describe a 65-year-old woman with a recent diagnosis of myelodysplastic syndrome/myeloproliferative neoplasm overlap syndrome(MDS/MPN)who abruptly developed painful,pruritic nodules consistent with NP.Case presentation:A 65-year-old woman with MDS/MPN presented for evaluation of painful and pruritic nodules on her upper and lower extremities.A biopsy revealed a lobular neutrophilic infiltrate in the subcutis without evidence of microorganisms or vasculitis.The patient was diagnosed with NP and treated with oral prednisone.Within 1 month of treatment,she reported complete resolution of the nodules.Discussion:Similar to other neutrophilic dermatoses,NP may arise in association with hematologic malignancies of myeloid origin,such as MDS/MPN.A literature review revealed that most cases of NP associated with MDS occur after the onset of MDS and respond to systemic corticosteroids,not antibiotics.Infection should be ruled out before initiating treatment with systemic steroids.Conclusion:Although the mechanism is still unknown,it is important for clinicians to be aware that NP is associated with MDS;thus,hematological malignancies should be investigated on diagnosis of NP.Once diagnosed,NP is easily treated and has an excellent response to systemic corticosteroids.

Panniculitis,an Uncommon Skin Manifestation in Adult Dermatomyositis

Objective:The skin manifestations of dermatomyositis(DM)are complex and diverse,with some rashes having diagnostic significance,while others have a certain suggestive effect on the patient’s condition,visceral damage,and prognosis.This case-cohort study was performed to investigate the skin manifestations of 205 adult inpatients with DM.We then analyzed the association between the clinical characteristics and panniculitis in adult patients with DM.Methods:This study was conducted at the First Medical Center of PLA General Hospital,Beijing,China from March 1,2016 to December 30,2020 with follow-up until December 31,2021.This case-cohort study involved 205 eligible participants with DM who met the criteria for idiopathic inflammatory myopathy.Clinical data,cutaneous manifestations,and lesion locations were screened and analyzed.Serum samples were collected,and immunoblotting was used to detect 16 types of myositis-specific antibodies and myositis-associated antibodies.Results:The results showed that 16(7.8%)of the 205 adult patients with DM had panniculitis.The panniculitis was mainly distributed in the subcutaneous fat of the abdomen,buttocks,and thighs.This condition was often accompanied by skin ulcers,atrophic scars,and calcinosis.Moreover,9(72.7%)of the 16 patients with panniculitis were positive for antibodies to nuclear matrix protein 2(anti-NXP2 antibodies).Conclusion:The data showed that panniculitis is an uncommon skin manifestation in adult patients with DM,with a positivity rate of 7.8%.The appearance of panniculitis is likely associated with anti-NXP2 antibodies.

Occasional finding of mesenteric lipodystrophy during laparoscopy:A difficult diagnosis

Mesenteric lipodystrophy is a rare pathological condition affecting the mesentery. Its initial presentation is typically asymptomatic. Pathological characteristics are unspecific, and generally attributed to inflammation, unless the diagnosis is suspected. Laparoscopy done for other reasons has been, as in this case, unsuccessful in providing evidence for the correct diagnosis, thus requiring laparotomy due to lack of diagnostic tissue. After 6 mo no further medical therapy is required, as the patient remains asymptomatic. Discussion of this case and a brief review of the literature are presented in the following paragraphs.