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Poikiloderma with neutropenia (PN), is a rare autosomal recessive condition with many associated complications and manifestations. Here we present a patient with confirmed PN who is of one-quarter Chucktaw or Cherokee heritage with no known descent from the Navajo tribe. The patient's condition was complicated by chronic bilateral lower limb cellulitis and associated osteomyelitis which was unresponsive to extensive antibiotic regimens. Subsequent treatment with hyperbaric oxygen therapy (HBOT) was successful. To date, no author has reported on the treatment of recurrent cellulitis using HBOT in this patient population. Based on our experience, HBOT should be considered in patients with PN., (© 2021 Wiley Periodicals LLC.)

Background: Mutations in NFKB1(nuclear factor of kappa light polypeptide gene enhancer in B-cells 1) are associated with a variety of clinical symptoms, including lymphadenopathy, splenomegaly, hepatomegaly, autoimmune haemolytic anaemia, arthralgia, recurrent respiratory tract infections and post-operative necrotizing cellulitis., Case Presentation: We describe a case of a 47-year-old man, who presented with deep necrotizing cellulitis after incision of a submucous abscess by a dentist. Surgical intervention led to a massive progress. Pyoderma gangraenosum (PG) was diagnosed clinically and confirmed histopathologically. High dose corticosteroids and intravenous immunoglobulins (IVIG) improved wound healing dramatically. Until now, immune mediated inflammation events not only affected the skin, but also multiple inner organs, i.e. the heart, lungs and gut. Sequencing of all coding exons of NFKB1 revealed a heterozygous 1bp deletion in exon 23 predicting a frameshift starting at codon Ala891 and resulting in a subsequent stop codon at position 6 in the new reading frame: NM_003998.4: c.2671del; p.(Ala891Glnfs*6) Acute episodes were always successfully treated with corticosteroids, IVIG and concomitant antibiotics. To prevent further exacerbations, the patient receives IVIG once a month, low-dose corticosteroids and methotrexate., Conclusion: This is the first case of a patient with recurrent necrotizing cellulitis and immune mediated multi-organ involvement (heart, lungs, intestine) carrying the novel frameshift mutation c.2671del (p.Ala891Glnfs*6) in NFKB1 effectively treated with IVIG, low-dose corticosteroids and methotrexate., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Rationale: Follicular occlusion triad (FOT) is an autosomal recessive inherited disease and no more than 3 variants of the triad have been reported. We give a report in which scrotal elephantiasis is a variant of FOT and further perform a literature review., Patient Concerns: A 41-year-old man came to us because of a large scrotal cyst and generalized skin lesions that had occurred over the past 10 years. The generalized skin lesions consisted of hidradenitis suppurativa on the perineum and back, acne conglobata in the armpit, and dissecting cellulitis of the scalp. He took antibiotics for a long time but achieved poor effect. Furthermore, he told his father and elder brother also manifested such skin lesions., Diagnoses: Magnetic resonance showed a mass in the left scrotum with clear boundaries. A routine blood test showed a high leukocyte level of 12 × 10/L and a hemoglobin content of 78 g/L. C-reactive-protein increased. Series of autoimmune antibody tests were negative. The postoperative pathologic findings showed that the mass was an epidermoid cyst, and hematoxylin and eosin staining showed hyperkeratosis of the skin as well as inflammatory and edematous changes. A diagnosis of a variant of FOT was made., Interventions: We removed skin abscesses and lesioned the inner part with hydrogen peroxide. Then we performed an excision of the scrotal lesion., Outcome: The patient recovered well and had no evidence of recurrence at a 16-month follow-up., Lessons: We reported a case in which scrotal elephantiasis was a variant of FOT and surgical intervention played an important role in secondary urologic diseases.

Recent evidence suggests that the accumulation of macrophages as a result of obesity-induced adipose tissue hypoxia is crucial for the regulation of tissue fibrosis, but the molecular mechanisms underlying adipose tissue fibrosis are still unknown. In this study, we revealed that periostin (Postn) is produced at extraordinary levels by adipose tissue after feeding with a high-fat diet (HFD). Postn was secreted at least from macrophages in visceral adipose tissue during the development of obesity, possibly due to hypoxia. Postn -/- mice had lower levels of crown-like structure formation and fibrosis in adipose tissue and were protected from liver steatosis. These mice also showed amelioration in systemic insulin resistance compared with HFD-fed WT littermates. Mice deficient in Postn in their hematopoietic compartment also had lower levels of inflammation in adipose tissue, in parallel with a reduction in ectopic lipid accumulation compared with the controls. Our data indicated that the regulation of Postn in visceral fat could be beneficial for the maintenance of healthy adipose tissue in obesity.

Background: Infection is the most common type of complication observed in lymphedema and is promoted by lymphatic system dysfunction, which causes locoregional immune disorders. Infectious complications are primarily bacterial and most commonly cellulitis (dermato-lymphangio-adenitis, DLA) caused by patients' own skin Staphylococci epidermidis and aureus. The clinical course and outcomes in the immune response to infection have been shown to be associated with genetic polymorphisms., Aim: To investigate polymorphism of TNFα-308G>A, CCR2-190G>A, CD14-159C>T, TLR2 2029C>T, TLR4 1063A>G, TLR4 1363C>T, TGFβ 74G>C, and TGFβ 29T>C. The second part of study was the correlation of levels of TNFα and TGFβ with their genes polymorphism in one hundred patients with lower limb postdermatitis lymphedema., Results: (a) High percentage of TNFα homozygotes, no differences in genotypes of CD14-159C>T, CCR2-190G>A, TGFβ 74G>C, TGFβ 29T>C, and TLR4 1063A>G, low percentage of TLR2 2029C>T heterozygotes and homozygotes TT, and a high percentage of TLR4 1363C>T homozygotes TT, (b) low serum levels of TGFβ and TNFα in 19% and 43% of patients, respectively, however, lack of correlation between low levels of these cytokines and frequency of homozygotes CC and AA, respectively., Conclusions: The practical implications of finding high frequency of genotype TT of TLR4 1363C>T are indications for testing this gene in patients with obstructive lymphedema of lower limbs and early antibiotic prophylaxis of recurrent attacks of DLA, and during elective surgery of lymphedema. The obtained data are also important as a contribution to mapping of genetic variations in acquired lymphedema of lower limbs.

The problem actuality is caused by significant enhancement of the incidence rate for inflammatory diseases of the head and neck tissues, first of all of the oral cavity floor abscesses and phlegmons, which causes severe forms of mediastinitis while inadequate treatment. The authors have had established, that Toll-like receptors (TLR) initiate a cascade of anti-inflammatory reactions of the inborn immunity, followed by synthesis of a certain cytokines, and their genetic polymorphism changes the immune reactivity of the organism. Trustworthy correlation of the gene TLR4 (rs4986790) polymorphism 896A/G was proved with high risk of the odontogenic phlegmon of the oral cavity floor occurrence, what would permit to prognosticate the disease course in early terms, to optimize the schemes of its prophylaxis and treatment.

Background: Bacterial non-necrotizing erysipelas and cellulitis are often recurring, diffusely spreading infections of the skin and subcutaneous tissues caused most commonly by streptococci. Host genetic factors influence infection susceptibility but no extensive studies on the genetic determinants of human erysipelas exist., Methods: We performed genome-wide linkage with the 10,000 variant Human Mapping Array (HMA10K) array on 52 Finnish families with multiple erysipelas cases followed by microsatellite fine mapping of suggestive linkage peaks. A scan with the HMA250K array was subsequently performed with a subset of cases and controls., Results: Significant linkage was found at 9q34 (nonparametric multipoint linkage score (NPL(all)) 3.84, p=0.026), which is syntenic to a quantitative trait locus for susceptibility to group A streptococci infections on chromosome 2 in mouse. Sequencing of candidate genes in the 9q34 region did not conclusively associate any to erysipelas/cellulitis susceptibility. Suggestive linkage (NPL(all)>3.0) was found at three loci: 3q22-24, 21q22, and 22q13. A subsequent denser genome scan with the HMA250K array supported the 3q22 locus, in which several SNPs in the promoter of AGTR1 (Angiotensin II receptor type I) suggestively associated with erysipelas/cellulitis susceptibility., Conclusions: Specific host genetic factors may cause erysipelas/cellulitis susceptibility in humans.

The chicken major histocompatibility complex (MHC) has been implicated in conferring resistance or susceptibility to several bacterial, parasitic, and viral diseases, the most notable of which is Marek's disease. In Marek's disease certain MHC haplotypes have been shown to confer relative resistance (B21), whereas other haplotypes are susceptible (B13). Relatively little work has been performed looking at the association of the MHC with bacterial diseases. One such disease is cellulitis, which is caused by several different bacteria but most notably by Escherichia coli. In this report, a commercial broiler chicken line known to contain standard B13 and B21, as well as the unique MHC types BA9 and BA12, was examined in a challenge model for cellulitis. The MHC-defined birds were challenged with a cellulitis-causing E. coli isolate and the frequency of lesion development and severity was quantified. In conclusion, B21 had the highest incidence of cellulitis development, B13 had the lowest incidence, and BA9 and BA12 had intermediate results. Results concerning the lesion severity showed that it was independent of the birds' MHC type.

In this report, we describe a case of bilateral non-syndromic hereditary lymphedema praecox of lower legs. The patient was diagnosed at age 16. Ten years later, he was unable to ambulate due to increased bilateral lower leg volume, continuous pain, and recurrent episodes of cellulitis. He was treated at our tertiary-care center with compression therapy and circumferential liposuction of lower legs, ankles, and dorsum of feet in order to remove hypertrophic fat deposits, facilitate conservative therapy, and decrease further risk of cellulitis. No complications were seen and compression therapy was continued. Fourteen month follow-up reveals no increase in leg volume over time, absence of pain, and no further episodes of cellulitis with complete ability to ambulate and return to normal activities. Even when it does not eliminate the underlying cause of primary lymphedema, combined therapy consisting of compression and liposuction is safe and is able to achieve control, at least on a short term, of clinically disabling conditions associated with advanced stages.

Comparative analysis of cytogenetic indices and soft tissues regenerative activity of postoperative wounds was carried out in 69 patients with abscesses and phlegmons of maxillofacial region. The reparative regeneration potential was reduced that was directly connected with infectious mutagenesis in patients with prolonged inflammatory process running.

The present study examined several basic attributes of first-line defence mechanisms in the skin as potential factors that may explain the susceptibility of broiler chickens to cellulitis. The variables including structural characteristics of the skin, physicochemical properties and cellular responses to the challenge with pathogens were compared between two categories of chickens, a strain of fast-growing commercial broiler chickens (susceptible to cellulitis) and leghorn chickens (resistant to cellulitis). There were substantial differences between leghorns and broilers with regard to physiological characteristics of the skin. Broiler skin was more amenable to injury and the wound-healing process was slow. Compared with leghorns, the lesions resulting from sub-dermal challenge in broilers were more severe and disseminated over a larger area. Mobilization of phagocytic cells (heterophils and macrophages) in leghorns was brisk even in the areas distant from the site of infection, whereas only few heterophils were recruited in the skin of broilers. The functional competence of heterophils in broilers was inferior when compared with leghorns. Based on the present finding, the predisposition of broilers to cellulitis appears to be primarily associated with the inferior first line of defence of their skin. Broilers in commercial situations may be at higher risk to succumb to even minor infection and eventually develop cellulitis because: (1) structural weaknesses of the skin may predispose broilers to skin injury and thus the risk of skin infection by pathogens is increased; (2) broiler skin surface is more likely to provide a conducive environment for colonization of Escherichia coli; (3) in the event of infection, poor recruitment of phagocytic cells to the site of infection may readily lead to widespread colonization of the tissue by pathogens causing cellulitis and (4) poor functional quality of the phagocytic cells that are mobilized compromise the ability of the host to contain the spread of infection.

Background: Eosinophilic cellulitis is a polymorphous, chronic disease characterized by eosinophil infiltration and granulomatous inflammation., Objective: Our purpose was to describe the clinical, histologic, and immunohistologic findings in three family members who have had eosinophilic cellulitis since childhood associated with mental retardation and abnormal body habitus., Methods: Family members were evaluated. Multiple skin biopsy specimens were obtained and examined after hematoxylin-and-eosin staining, by immunofluorescence and by electron microscopy. Blood specimens were analyzed by immunoassays for eosinophil granule proteins and eosinophil active cytokines., Results: Three short-statured, mentally retarded family members with abnormal body habitus in at least two generations had recurrent eosinophilic cellulitis. Peripheral blood and bone marrow eosinophilia was present. Plasma eosinophil granule major basic protein and eosinophil-derived neurotoxin levels were elevated with normal plasma eosinophil cationic protein levels. Eosinophil survival in culture was increased by patients' plasma and was blocked with monoclonal interleukin-5 antibody. The level of plasma interleukin-5 was elevated. Lesional skin biopsy specimens showed massive staining for three eosinophil granule proteins. Electron microscopy showed eosinophil disruption., Conclusion: Eosinophilic cellulitis, mental retardation, and abnormal body habitus were likely inherited as a dominant syndrome in this family in which eosinophil involvement was striking.

The distribution of the genetic markers of leukocytes (HLA I and II), serum proteins (allotypes Gm, transferrin, haptoglobin, group-specific component), red cell enzymes (acid phosphatase--AP, phosphoglucomutase--PGM, esterase D, adenosine deaminase) was found to be universal in patients suffering from various pyoinflammatory diseases. The incidence of HLA A10, Cw4, DR5 antigens, IgG allotype G1m (2) and phenotype G1m (+1+2) and AP (aa) and PGM (2-2) phenotypes was found increased in patients with maxillofacial suppuration and pyoinflammatory complications of maxillofacial injuries as against normal subjects and patients with maxillofacial injuries without such complications. Traumatic osteomyelitis much more often develops in the carriers of the before genetic markers than in those in whom these markers are absent (61.6 and 20.4%, respectively).

Gingivitis occurring with a cystic lesions in young patients with facial cellulitis is highly suggestive of a pneumococcal etiology. Two cases of pneumococcal cellulitis and gingivitis with bacteremia are reported, and the pertinent literature is reviewed.

Eosinophilic cellulitis (Wells' syndrome) is characterized by recurrent episodes of nonpruritic, indurative, cutaneous swellings. Three cases of this syndrome occurred in two male siblings and their mother. In all three family members, skin lesions appeared early in infancy. Unusual clinical manifestations included eosinophilic pleural effusions and pericarditis.