Weber-Christian Panniculitis Research Articles

Chronicles of A.I. Pospelov Moscow Society of Dermatovenerologists and Cosmetologists (MSDC was founded on October 4, 1891). Bulletin of the MSDC № 1150

On December 20, 2022, the 1150th meeting of the Moscow Society of Dermatovenerologists and Cosmetologists (MSDC) named after A.I. Pospelov took place.
 The meeting was held in a face-to-face format. There were 110 participants in total. Accepted as a member of the MSDC 3 people.
 Two clinical cases are presented in the clinical part of the meeting: widespread cutaneous leishmaniasis in an HIV-infected patient. The peculiarity of this clinical case was the extreme prevalence of rashes. No systemic pathology was detected; panniculitis in a patient after a COVID-19 infection. The process proceeded according to the type of non-stagnant Weber-Christian panniculitis: after surgical autopsy, a mucus-like substance was released.
 Three reports were presented in the scientific part of the meeting. Corresponding member Prof. O.Y. Olisova (Sechenov University) made a report on the benefits of treating severe and moderate psoriasis with netakimab; researcher of State Scientific Center of Dermatovenereology and Cosmetology Central Dermatology and Venereology Institute A.A. Vorontsova reported on the results of an original study of the generality of clinical and pathomorphological manifestations of psoriasis and T-cell lymphoma; with pathogenetic justification of the development of cutaneous toxicity as a result of targeted therapy of oncological diseases was performed by E.V. Orlova (Sechenov University): it is based on the suppression and dysfunction of EGFR-controlled processes in the skin.

Disseminated seronegative echinococcosis: difficulties of diagnosis and treatment

Disseminated seronegative echinococcosis, occurring with damage to soft tissues, bones and joints, is a rare variant of the course of helminthiasis, often associated with diagnostic difficulties, a high probability of complications and difficult treatment.
 This article presents a case of disseminated seronegative echinococcosis, clinically and laboratory simulating a severe form of WeberChristian panniculitis complicated by sepsis. The advantages and disadvantages of serological and morphological studies in the verification of disseminated echinococcosis are discussed, as well as the feasibility of using repeated histological studies in cases of echinococcosis under the masks of rheumatic diseases.

ОПЫТ УСПЕШНОГО ПРИМЕНЕНИЯ ТОЦИЛИЗУМАБА В КОМПЛЕКСЕ С ТОФАЦИТИНИБОМ У ПАЦИЕНТКИ С ИДИОПАТИЧЕСКИМ ПАННИКУЛИТОМ ВЕБЕРА–КРИСЧЕНА, ПРОЯВЛЕНИЯМИ СИНДРОМА МАКРОФАГАЛЬНОЙ АКТИВАЦИИ И ПОРАЖЕНИЕМ НЕРВНОЙ СИСТЕМЫ

Weber–Christian panniculitis (WCP) (syn.: Idiopathic lobular panniculitis) is a rare and poorly understood rheumatic disease characterized by the appearance of subcutaneous nodules and recurrent attacks of fever. The lack of clear diagnostic criteria and the complexity of differential diagnosis slow down the diagnosis process. The possible involvement of other organs in the process significantly complicates the diagnosis and can become a potentially life-threatening condition. The choice of tactics for the treatment of panniculitis in children is a serious problem. The article describes a clinical observation of a 15-year-old patient with a severe drug-resistant form of WCP, complicated by macrophage activation syndrome and polyneuropathy, with the effective use of genetically engineered biological therapy with the tofasitinib – Janus kinase inhibitor and the tocilizumab – interleukin-6 blocker with ineffective corticosteroids, cyclosporceporitin, and ethanercept.

Analysis of the clinical characteristics of thirteen patients with Weber-Christian panniculitis

Weber-Christian disease (WCD) is an uncommon, idiopathic disease that is challenging to diagnose and has an unclear treatment protocol. We reviewed thirteen patients with WCD and analyzed their clinical characteristics. The purpose of this article was to improve the understanding of this rare disorder. Thirteen cases of WCD were analyzed retrospectively regarding their clinical manifestation, laboratory results, misdiagnoses, therapy, and outcome. Of the thirteen patients diagnosed with WCD, the majority were female (male to female ratio, 2:11), with a mean patient age of 50.1years. Subcutaneous nodules were the most commonly reported symptom, followed by fever, arthralgias/arthritis, and myalgia. The laboratory results were typically nonspecific. In total, 61.5% patients were misdiagnosed before pathology confirmed the diagnosis of WCD. Most patients were treated with corticosteroids and/or immunosuppressants. Two patients were treated surgically. While long-term remission was successfully achieved in some patients, others had recurrent symptoms. WCD was predominantly observed in female patients in our cohort. Subcutaneous nodules and fever were the most common clinical characteristics. In addition, the patients' laboratory test results were nonspecific, which led to a high misdiagnosis rate. In this study population, corticosteroid and/or immunosuppressant treatments were efficacious therapeutic interventions for WCD.Key Points•Subcutaneous nodules and fever were the most common clinical characteristics in Weber-Christian panniculitis.•Misdiagnosis rate was higher in Weber-Christian panniculitis patient; tumors, bacterial infections and rheumatic diseases were the most common misdiagnoses.•Corticosteroid and/or immunosuppressant therapy was effective in most Weber-Christian panniculitis patients.

IDIOPATHIC LOBULAR PANNICULITIS IN RHEUMATOLOGY PRACTICE: THE AUTHORS’ OWN DATA

Idiopathic lobular panniculitis (ILP) (synonym: Weber-Christian panniculitis) is the least studied disease in the group of systemic connective tissue lesions and characterized by systemic damage to subcutaneous adipose tissue (SAT). There is no unified concept of the etiology and pathogenesis of ILP now. The literature contains almost no data on the diagnostic value of laboratory studies and therapeutic approaches, which served as the basis for this investigation. Objective: to investigate the relationship between the clinical presentation of ILP and immune inflammatory parameters in patients with this disease.Subjects and methods. Examinations were made in 67 patients (9 men and 58 women) aged 20 to 76 years with a verified diagnosis of ILP (median duration, 78.91 [48; 540] months), who were followed up at the V.A. Nasonova Research Institute of Rheumatology for the period 2007 to 2017. The determination of α1-antitrypsin titer, liver fractions, amylase, lipase, trypsin, ferritin, creatine phosphokinase, leptin, and tumor necrosis factor-α (TNFα), chest computed tomography, and induration morphological examination were done in addition to physical examination.Results and discussion. The disease was found in all age groups, but it accounted for more than half (57%) of cases at the most able-bodied age (45–60 years). Analysis of the clinical manifestations of ILP could identify its four types: nodular (n=30), plaque (n=10), infiltrative (n= 5), and mesenteric (n=12), which were characterized by typical clinical features. The observed group showed a significant increase in erythrocyte sedimentation rate (ESR) (p=0.01) and C-reactive protein (CRP) level (p < 0.0001). ESR correlated with tenderness on the visual analogue scale (VAS) (p<0.05; r=0.29), induration area (p<0.05; r=0.50), and rises in body temperature (p<0.05; r=0.68) and CRP level (p<0.05; r=0.68). The concentration of CRP correlated with tenderness on visual analog scale (p<0.05; r=0.46), induration area (p<0.05; r=0.61), node stage (p<0.05; r=0.41), and TNF-α concentrations (p<0.05; r=0.32). The latter showed a direct correlation with node stage (p<0.05; r=0.41) and leptin levels (p<0.05; r=0.28) and an inverse correlation with the number of nodes (p<0.05; r=-0.24). Leptin levels were increased in 35 (52.23%) patients and displayed a direct correlation with body mass index (p<0.05; r=0.46), induration area (p<0.05; r=0.31), CRP level (p<0.05; r=0.36) and an inverse correlation with the number of nodes (p≤0.05; r=-0.33). Morphological examination of skin and SAT biopsy specimens was performed in 65 (97.01%) patients. Pre- and retroperitoneal adipose tissues were biopsied in three of five patients without skin and SAT lesions; this was not done in the remaining patients because of access difficulties. ILP was verified in all cases. Therapy was performed using the essential drugs adopted in rheumatology practice. Their therapeutic effects were noted in 62.68% of cases; inefficiency and health deterioration were detected in 12 (17.91%) patients, which necessitated an increase in the dose of disease-modifying antirheumatic drugs. Seven patients were given the following biological agents: abatacept (n=2), adalimumab (n = 3), etanercept (n=1), and rituximab (n=1).Conclusion. There is an obvious need to expand knowledge about this pathology amongst physicians and to conduct further investigation in order to timely diagnose and search for the most effective treatment options for ILP.

Long-term follow-up of a female patient with Weber–Christian panniculitis

Weber–Christian disease is a rare form of lobular panniculitis characterized by painful subcutaneous nodules on the trunk and limbs commonly concurrent with systemic manifestations and a tendency to relapse. Known are only sporadic cases of a long-term (more than 5-year) followup of patients with Weber–Christian disease. For this reason, there are neither prognostic criteria for the disease no proven conventional treatment option now. The paper describes a clinical case of a female patient with a verified diagnosis of Weber–Christian disease who has a sustained subclinical remission due to low-dose prednisone use and basic antirecurrent therapy.

GENETIC ASPECTS OF PANNICULITIS IN A RUSSIAN POPULATION: A PILOT STUDY

Numerous clinical observations show that the human genetic background plays an important role in predisposition to many diseases. Objective: to investigate a possible relationship of the polymorphisms in 19 A/G leptin (LEP) gene, in the interleukin (IL)-1 receptor antagonist (IL-1RA) gene VNTR (variable number of tandem repeats), and in the -174G/C IL-6 gene to the risk of developing the clinical phenotypes of panniculitis (PN), clinical and laboratory parameters. Subjects and methods. The study enrolled 54 patients (48 women and 6 men) aged 15 to 76 years with a documented diagnosis of PN who had been treated at the V.A. Nasonova Research Institute of Rheumatology. Group 1 of 46 patients with erythema nodosum (EN) and Group 2 of 8 with Weber-Christian panniculitis (WCP) were formed for genetic study. The genotyping data on 197 healthy unrelated individuals were used as a control. Clinical and laboratory information was available from 39 patients for genotyping 19A/G LEP gene polymorphism, that from 43 patients for -174G/C IL-6 gene polymorphism, and that from 46 patients for IL-1RA polymorphism. Genotyping was performed by a polymerase chain reaction-restriction fragment length polymorphism (PCRRFLP) analysis. Results and discussion. The frequencies of the LEP 19 GG genotype and the LEP G allele in patients with EN and WCP were significantly higher than those in the controls (48.7 and 18.2%, p = 0.0004; 50.0 and 18.2%, p = 0.053; and 70.5 and 45.4%, p = 0.0002, respectively). The frequencies of the A1A1 genotype and A1 allele of IL-1RA gene VNTR polymorphism in EN group were significantly higher than those in the controls (67.4 and 44.2%, p=0.011; 80.4 and 61.6%, p = 0.002, respectively). The frequency of IL-6 -174GC polymorphism was also higher in the EN group than that in the controls (58.1 and 34.7%, p = 0.008). The -174G/C polymorphism showed a significant association with the site of erythema (p = 0.028). In patients with a single lesion of erythema on the body, the frequency of the -174 GC genotype was significantly higher than that in those with multiple foci of erythema (72.0 and 31.2%, respectively; p = 0.025). In the WCP group, analysis of variance showed an association of IL-1RA gene VNTR polymorphism with the intensity of pain, assessed by the visual analogue scale. The carriers of the A1A1 genotype had more severe pain than those of the A1A2 genotype (83.3±11.5 and 20.0±18.2 mm, respectively; p = 0.008). Conclusion. The findings suggest that genetic testing can be used to predict the clinical course of PN.

Weber Christian Disease Presenting With Pacytopenia and Anticardiolipin Antibodies

Weber-Christian disease is a controversial entity that represents reccurent fever, inflamation of adipose tissue and tender subcutaneus nodules. The etiolgy of WCD remains unknown. However, ıt has been related to an immunologically mediated reaction to diverse antigenic stimuli. We present a 50 year-old man with Weber-Christian disease presented with fever, reccurent pancytopenia attacks, IgG and IgM anticardiolipin antibody positivitiy.. The patient showed fever and characteristic redish subcutaneous nodules. Biopsy taken from the lesion showed areas of fat necrosis, accumulation of leucocytes and macrophages with foamy cytoplasm. and no evidence of vasculitis. Histopathologic findings were consistent with Weber-Christian panniculitis. Bone marrow aspirate and biopsy disclosed mild hypercellularitiy with concomitant mild increasing lympocyte and plasma cells. Patients with pancytopenia, cutaneous lesions and ACA positivity must be searched for WCD.

Weber-Christian panniculitis: A review of 30 cases with this diagnosis

Background: More than 60 years ago, Christian described a panniculitis that was later termed Weber-Christian disease . Objective: The purpose of this study was to investigate whether this is a specific disease or a nonspecific disease that embraces several specific conditions. Methods: We studied 30 cases diagnosed as Weber-Christian panniculitis and found it possible to make a more specific diagnosis. Results: In 12 patients, findings were compatible with erythema nodosum. Six patients had phlebitis or postphlebitic syndrome. Factitial panniculitis was diagnosed in five patients, and trauma had a role in the conditions of another three patients. Cytophagic panniculitis, lymphoma, and leukemia were recognized in one patient each. The lesion was lobular in almost all cases, and the presence of lipophagia was noted in 19 biopsy specimens. Granulomatous, neutrophilic, and lymphocytic pathologic changes were present in nine, eight, and eight tissue specimens, respectively. Conclusion: The recognition of distinct disease patterns of fat lesions as fat necrosis with pancreatic disease, α 1 -antitrypsin panniculitis, lupus and connective tissue disease panniculitis, involution lipoatrophy, lipomembranous panniculitis, factitial panniculitis syndromes, calcification panniculitis, lipophagic lipoatrophy, and cytophagic panniculitis has lessened the need for a less specific panniculitis category. All these diseases have been reported in the literature as “Weber-Christian disease.” Because separate and distinct forms of fat lesions have been described, we believe that the eponym should be abandoned and that more specific diagnoses should be made on the basis of pathogenesis or cause. (J Am Acad Dermatol 1998;39:56-62.)

Protease-inhibitor deficiencies in a patient with Weber-Christian panniculitis

Protease-inhibitor deficiencies in a patient with Weber-Christian panniculitis

Weber-Christian panniculitis with membranous glomerulonephritis

A case of Weber-Christian panniculitis is described in which an acute phase of the disease was accompanied by the presence of circulating immune complexes, elevated serum levels of IgA and IgM, and membranous glomerulonephritis, with concomitant spontaneous remission of both diseases. This case reinforces the view that an immunologic dysfunction may be involved in the pathogenesis of Weber-Christian panniculitis.

Weber-Christian disease with nephrotic syndrome.

Percutaneous renal biopsy in a child with nephrotic syndrome was followed by copious oozing of a clear, oily liquid from the skin puncture site. Subsequently, inflamed subcutaneous nodules appeared over the trunk and extremities suggesting Weber-Christian panniculitis. Extensive liquefication of fat occurred, especially over the abdomen, hips, and scrotum. Attacks of panniculitis subsided in nine months. The child received an eight-week therapy with cyclophosphamide and prednisone, and has remained free of proteinuria for one year.

A review of the concept of Weber-Christian panniculitis with a report of five cases.

SUMMARY. —Five patients with Weber-Christian panniculitis (W.C.P.) are described. These illustrate the great variability of the clinical course of cases to which this diagnosis is applied. In two (one fatal) no aetiological factor was found, one case was considered to be an artefact, one was related to tuberculosis and another was possibly a manifestation of systemic lupus erythematosus. It is concluded from a survey of the literature and from these 5 cases that W.C.P. is not nosologically distinct and that present terminology suggesting a single disease entity is undesirable. A purely descriptive term like nodular panniculitis should be used while a search is made for aetiological factors. In some cases these may be found to rest on a vascular basis, possibly the result of an immunological process.

SYSTEMIC WEBER-CHRISTIAN DISEASE.

A patient suffering from Weber-Christian panniculitis was found at necropsy to have similar lesions in the visceral adipose tissue. When the lesions occur internally, diagnosis during life is complicated by the difficulty of examining visceral adipose tissue and the disease may often go unrecognized, but patients suffering from Weber-Christian panniculitis should be investigated bearing in mind the possibility of internal lesions. Only 11 cases confirmed by necropsy appear to have been reported hitherto. The aetiology remains obscure. It is suggested that the name ;systemic Weber-Christian disease' be applied when the lesions occur in adipose tissue other than the panniculus adiposus and that the term ;Weber-Christian panniculitis' be used when the lesions are confined to the subcutaneous adipose tissue.