Local neutrophil and eosinophil extracellular traps formation in pyodermatitis pyostomatitis vegetans.

Pyodermatitis pyostomatitis vegetans is a rare inflammatory condition, affecting the skin and/or mucous membrane. Some cases include both skin and mucous involvement, whereas others develop either skin or mucous lesions only. The typically affected areas are the scalp, face, trunk and extremities, including the flexural areas and umbilicus. Clinical features show erosive granulomatous plaques, keratotic plaques with overlying crusts and pustular lesions. Among mucous lesions, oral mucosa is most frequently involved, and gingival erythema, shallow erosions, cobblestone-like papules on the buccal mucosa or upper hard palate of the oral cavity are also observed. Some of the lesions assume a 'snail track' appearance. Although there are several similarities between pyodermatitis pyostomatitis vegetans and other diseases, that is pyoderma gangrenosum, pemphigus vegetans and pemphigoid vegetans, the histopathological features of pyodermatitis pyostomatitis vegetans are unique in that epidermal hyperplasia, focal acantholysis and dense inflammatory infiltrates with intraepidermal and subepidermal eosinophilic microabscesses are observed. Direct immunofluorescence findings are principally negative. Activated neutrophils are supposed to play an important role in the pathogenesis of pyodermatitis pyostomatitis vegetans. The expression of IL-36 and neutrophil extracellular traps (NETs) was observed in the lesional skin, and additionally, eosinophil extracellular traps (EETs) was detected in pyodermatitis pyostomatitis vegetans. A possible pathogenic role of NETs and EETs in the innate immunity and autoinflammatory aspects of pyodermatitis pyostomatitis vegetans was discussed.

Chronic recurrent multifocal osteomyelitis in association with pyoderma gangraenosum.

BACKGROUND: Chronic recurrent multifocal osteomyelitis (CRMO) is a rare acquired inflammatory skeletal disorder of unknown origin. CRMO was first described by Gideon in 1972 and mainly affects children and young adults of female gender. The CRMO is part of the clinical picture of non-bacterial Osteomyelitis (NBO) and typically presents a relapsing recurring course with both remission and spontaneous exacerbation. CRMO is typically encountered in the limbs and the metaphysis of long bones in particular. Usually the clinical symptoms include painful swellings of the affected regions. This case report describes the rare case of a CRMO of the mandible in association with pyoderma gangraenosum. CASE PRESENTATION: A 14-year old female caucasian patient, residing in the south of Germany, presented in the oncological outpatient clinic of our Department of Paediatrics and Adolescent Medicine in June 2014 complaining of increasing neck pain and progressive swelling at her left cheek ongoing for about 6 weeks. These symptoms had been occurring quarterly for 4 years, but had never been as pronounced. Blood biochemistry showed a moderately elevated CRP (35 mg/l) and a significantly increased blood sedimentation rate (BSR 48/120 mm). The panoramic radiograph, however, revealed a bone alteration in the left mandibular region. Further investigations confirmed the diagnosis of CRMO. CONCLUSION: The present case underlines the fact that rare diseases might occasionally present with even more rare symptoms. These occasions can obviously be considered to present a considerable diagnostic challenge.

GAPO syndrome associated with pyoderma vegetans: an unreported co-existence.

GAPO syndrome is a rare autosomal recessive disease and an acronym composed of growth retardation, alopecia, pseudoanodontia, optic atrophy. Approximately 38 cases have been reported in literature until now. Pyoderma vegetans is a chronic inflammatory disease characterized with vesicopustular, exudative and vegetative lesions usually localized on face, scalp, axilla and genitalia. Pyoderma vegetans is attributed to a bacterial infection frequently occurring in individuals with an underlying immunosuppressive condition. A 30-year-old female patient was admitted to our clinic with complaint of a hemorrhagic, crusted, exudative vegetative two plaques on the scalp. On her physical examination, she had a prematurely aged face, predominant lower lips, total tooth loss, frontal bossing, enlarged anterior fontanelle, marked scalp veins, micrognatia, depressed nasal bridge, short stature, growth retardation. She was diagnosed as GAPO syndrome as the result of her physical examination. Result of the biopsy taken from scalp was evaluated as pyoderma vegetans. And the diagnosis of pyoderma vegetans was established based on the correlate of both clinical and histopathologic findings. Pyoderma vegetans coexisting with GAPO syndrome has not been reported previously. Thus we wished to report it.

Newly isolated lytic bacteriophages for Staphylococcus intermedius, structurally and functionally stabilized in a hydroxyethylcellulose gel containing choline geranate: Potential for transdermal permeation in veterinary phage therapy.

In the present research work, we propose a new antimicrobial treatment for pyoderma via cutaneous permeation of bacteriophage particles conveyed in a hydroxyethylcellulose (HEC) gel integrating ionic liquid as a permeation enhancer. Ionic liquids are highly viscous fluids constituted exclusively by ions, that are usually hydrolytically stable and promote solubilization of amphipathic molecules such as proteins, hence serving as green solvents and promoting the transdermal permeation of biomolecules. In the research effort entertained herein, the synthesis and use of choline geranate for integrating a HEC gel aiming at the structural and functional stabilization of a cocktail of isolated lytic bacteriophage particles was sought, aiming at transdermal permeation in the antimicrobial treatment of animal pyoderma. The results obtained showed a high ability of the ionic liquid in enhancing transdermal permeation of the bacteriophage particles, with concomitant high potential of the HEC gel formulation in the antimicrobial treatment of animal skin infections.

Investigation of the clinical efficacy of 0.2% topical stannous fluoride for the treatment of canine superficial pyoderma: a prospective, randomized, double-blinded, placebo-controlled trial.

Stannous fluoride (SF) is an antibacterial compound that has been successfully used to treat gingivitis in people and dogs, and cutaneous bacterial infections in horses. The purpose of this prospective, double-blinded, placebo-controlled clinical trial was to investigate the efficacy of 0.2% SF spray (BacDerm; Emerald 3 Enterprises Inc., Camdenton, MO, USA) for the treatment of canine superficial pyoderma. Twenty-six privately owned dogs with bacterial skin infections diagnosed on clinical signs, cytology and aerobic culture were enrolled. Dogs were randomly assigned to vehicle only or active ingredient treatment groups. The product was applied topically to affected areas once daily for 28 days, with assessments at days 0, 14, 28 and 42. Clinical and cytological evaluations were performed by the same investigators at each visit. Owners scored the improvement of hair coat, odour, pruritus and overall improvement at each recheck. Linear mixed models showed significant effects of treatment (P < 0.0001) and time (P = 0.0037) for investigator's scores, and a significant time effect for owners' haircoat (P = 0.0077) and odour (P = 0.0170) improvement scores. Dogs in both placebo and SF groups showed some improvement over time, and the investigator's scores on days 0 and 28 were not significantly different between groups for both (t-test P > 0.05). Spearman's rho correlation coefficients revealed a significant negative correlation between investigator's scores and all categories of owners' assessment scores in dogs of both groups. Although some dogs improved on SF, this study does not support the use of 0.2% SF as sole therapy for canine superficial pyoderma.

Mucocutaneous manifestations in 150 HIV-infected Indian patients and their relationship with CD4 lymphocyte counts.

Mucocutaneous findings in 150 HIV+ve cases (F, 79; M, 71) were evaluated over a one-year period. Mucocutaneous manifestations were seen in 96% with 2.9 mean number of dermatoses and mean cluster of differentiation (CD4) count of 196.33 cells/mm(3). The highest number of mean dermatoses, 3.29, was seen in individuals with severe immunosuppression. The most common mucocutaneous manifestation seen was candidiasis (35.33%), followed by seborrhoeic dermatitis (31.33%), oral pigmentation (29.33%), xerosis/ichthyosis (22.67%), pyodermas (22%), periodontitis (17.33%) and nail pigmentation (16.67%). Patient stratification according to the WHO immunological staging, according to CD4 counts, showed a statistically significant association (P < 0.05) for candidiasis, scabies, paronychia, oral pigmentation and diffuse hair loss. Nail and oral pigmentary changes, trichomegaly and subcutaneous fungal infections caused by dermatophytes were highlights of the study. Incidences of xerosis/ichthyosis, pyodermas, scabies and molluscum contagiosum reported in our study were higher and pruritic popular eruptions was lower than those in previous Indian studies. Cutaneous neoplasms were not seen in the present study.

[Pyodermatitis-pyostomatitis vegetans with nasal involvement]. / Pyostomatite-pyodermatite végétante avec atteinte nasale.

BACKGROUND: Pyodermatitis-pyostomatitis vegetans (PPV) is a rare chronic disorder often associated with inflammatory bowel disease. We report an isolated case involving the oral, labial and nasal mucosa. PATIENTS AND METHODS: A 28-year-old man, in good general condition, presented with a 2-year history of painless stomatitis. The physical examination revealed pustular and exophytic lesions of the jugal, gingival and palatine mucosa on an erythematous background, as well as some pustular and crusted lesions of the lower lip and nostrils. Histopathological analysis revealed epithelial hyperplasia and a suprabasal cleft with some signs of acantholysis and numerous neutrophils and eosinophils. Direct and indirect immunofluorescence assay was negative. There was no associated bowel disease. We concluded on a diagnosis of PPV of younger subjects. The lesions disappeared with oral corticosteroids but with steroid dependency. DISCUSSION: PPV is a rare dermatosis associated in more than 75% of cases with inflammatory bowel disease, usually ulcerative colitis. Lesions of the oral mucosa are a constant finding and are characterised by aseptic pustules on an erythematous background. Skin lesions are pustular and more or less exophytic. To our knowledge, there have been no reports to date of intranasal lesions of PPV.

Granulocyte and monocyte adsorption apheresis (GCAP) for refractory skin diseases caused by activated neutrophils and psoriatic arthritis: evidence that GCAP removes Mac-1-expressing neutrophils.

In the present study, we have shown that granulocyte and monocyte adsorption apheresis (GCAP), an extracorporeal apheresis instrument whose column contains cellulose acetate (CA) beads, is useful for skin diseases attributable to activated granulocytes and psoriatic arthritis (PsA). We assessed the clinical effectiveness of GCAP and investigated the mechanisms underlying the adsorption of pathogenic granulocytes. The effect of GCAP was assessed in 14 patients with neutrophilic dermatoses and 16 with PsA. The mechanisms by which the instrument adsorbs activated granulocytes were investigated using an in vitro mini-column system that mimics the GCAP. Skin lesions and arthropathy improved in 22 of 29 patients (75.9%) and 14 of 18 (77.8%), respectively. Mac-1 (CD11b/CD18) expression on the peripheral neutrophils, increased compared with normal subjects, was reduced by GCAP. In the mini-column system, CA beads adsorbed 50% neutrophils; and adsorption was inhibited significantly by treating plasma with EDTA and blood cells with antihuman CD11b monoclonal antibody. GCAP was useful for treating neutrophilic dermatoses and PsA. GCAP adsorbs Mac-1-expressing neutrophils to the CA beads by the binding of complement component (iC3b) on CA beads and CD11b expressed on activated neutrophils.

Herpes simplex: the primary infection.

A primary infection with herpes simplex virus (HSV) occurs at some time in the life of almost every member of the population, especially among those living in crowded, unsanitary conditions. In many cases the lesions are subclinical, and in most cases they are self-limited even when clinical. In a few patients, such as the newborn infant, the patient with concomitant eczema, or the patient with reduced immunity, the primary infection may become severe, generalized, and life-threatening. Type 1 HSV has a predilection for a number of sites, including the oral cavity, the eye, and the skin; and type 2 HSV has a predilection for the genital area and for the newborn.

Pyoderma gangrenosum involving the head and neck.

Pyoderma gangrenosum (PG) is a rare pathergy associated with systemic diseases such as ulcerative colitis and polycythemia vera. Its recognition in the differential diagnosis of other ulcerative lesions is important in managing an affected patient. A patient who developed lower extremity, oral, and cervical manifestations of this disease on separate occasions is presented. It is imperative that every clinician be aware of this entity because of the potential untoward effects of delayed or improper treatment including unnecessary surgery. Systemic and intralesional steroids are the treatment of choice.

Pyodermatitis-pyostomatitis vegetans: report of a case and review of the literature.

Pyodermatitis-pyostomatitis vegetans is a benign, rare disorder characterized by a pustular eruption in the oral mucosa and vegetating plaques involving the groin and axillary folds. Its association with inflammatory bowel disease is well established. We report the case of a 49-year-old-white man with ulcerative colitis who manifested a vegetating, annular plaque in the left inguinal region of 2 months' duration. Oral examination disclosed an erythematous mucosa with multiple painful pustules involving the labial and gingival mucosa. Histopathologic study demonstrated epidermal hyperplasia and an inflammatory infiltrate composed mostly of neutrophils and eosinophils, grouped into microabscesses within the epidermis and with a bandlike configuration in the upper dermis. Results of direct and indirect immunofluorescence studies were negative. We discuss the differential diagnosis between pyodermatitis-pyostomatitis vegetans and pemphigus vegetans.

Canine granulocytopathy syndrome: neutrophil dysfunction in a dog with recurrent infections.

A male Irish Setter dog had a clinical history of recurrent life-threatening bacterial infections, with associated periods of pyrexia and severe neutrophilia. Examination of a mandibular lymph node biopsy made when the patient was 10 weeks old revealed subacute diffuse suppurative lymphadenitis with reticuloendothelial hyperplasia. Circulating leukocytes isolated from the dog when it was 5 months old had a marked bactericidal defect when compared with cells from clinically normal dogs of the same age. The clinical syndrome in the affected patient resembled that observed in the granulocytopathies described in man and other animals.

[Hoarseness after endotracheal intubation caused by submucosal hemorrage of the vocal cord and recurrent nerve palsy].

Hoarseness often follows endotracheal intubation. A 58 year old man suffered from hoarseness due to recurrent nerve palsy and submucosal hemorrhage of the right vocal cord after general anesthesia. He was scheduled for resection of chronic pyoderma of the buttocks and atheroma of the right retroauricular region. Anesthesia was induced with thiopental 300 mg and endotracheal intubation was facilitated with vecuronium 6 mg. A spiral tube with 8 mm of inner diameter (Mallinckrodt Medical) was inserted with no problems. The tube was fixed at left side of the mouth. The cuff was inflated with air but the cuff pressure was not monitored. Anesthesia was maintained with sevoflurane (1-3%) and 67% N2O in 33% O2. Respiration was controlled mechanically. The patient was turned to the prone position and his head was turned to the right. The duration of surgery was one hour and forty minutes. Extubation of the tube involved no problems. He complained of hoarseness after surgery. Right recurrent nerve palsy and submucosal hematoma were observed. Without any treatment, hematoma disappeared in 70 days and hoarseness in 183 days. These hematoma and recurrent nerve palsy might be due to the compression of the vocal cord and recurrent nerve by tracheal tube and traction of the recurrent nerve by rotation of the neck.

Pyoderma gangrenosum in Papua New Guinea.

A severe case of pyoderma gangrenosum in a Papua New Guinean girl aged 11 years in reported. Multiple lesions of the arms, legs, buttocks and mouth were present. There was no associated disease. She was treated with very high doses of prednisolone and slow resolution over 3 months in hospital was observed.

[The efficacy of treating skin ulcers and burns with film dressings with antibiotics]. / Effektivnost' lecheniia iazv i ozhogov kozhi plenochnymi poviazkami s antibiotikami.

The article summarizes the results of studies conducted during the treatment of burns (108 patients), and ecthymas (85 patients) with the help of film dressing and antibiotic powder (R. Breitman's method). During application of this method the healing process was more rapid, the administration of antibiotics was minimum, the dressing process became more easy, good cosmetic results were obtained. The period of inpatient care was shortened, cost of treatment was reduced.

Palmoplantar keratoderma with progressive gingivitis and recurrent pyodermas.

Papillon-Lefèvre syndrome (PLS) is a rare inherited palmoplantar keratoderma (PPK) that is associated with progressive gingivitis and recurrent pyodermas. We present a case exhibiting classic features of this autosomal-recessive condition and review the current understanding of its pathophysiology, diagnosis, and treatment. Additionally, a review of pertinent transgredient PPKs is undertaken, with key and distinguishing features of each syndrome highlighted.

Pyodermatitis-pyostomatitis vegetans after subtotal colectomy for ulcerative colitis.

A 51-year-old Japanese woman underwent subtotal colectomy for ulcerative colitis 5 years before her first visit to our hospital, and her symptoms stabilized. She presented with a 2-month history of proliferative, pustular lesions on her face, scalp, and gingival and anal mucosae. Histological examination showed features similar to those of pemphigus vegetans. However, the results of immunofluorescence studies, immunoblot analysis and enzyme-linked immunosorbent assay for pemphigus were negative, and colonoscopy revealed a relapse of ulcerative colitis in the remaining rectal mucosa, so a diagnosis of pyodermatitis-pyostomatitis vegetans was made. This is the first report of mucocutaneous lesions that developed with a relapse of ulcerative colitis in the remaining rectum after subtotal colectomy. Moreover, a close correlation was found between the condition of bowel inflammation and that of mucocutaneous lesions.