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.

Blastomycosis-like Pyoderma Arising in Lichen Planus.

Blastomycosis-like pyoderma (BLP) is a rare reactive skin disease that is most commonly caused by bacterial infection. Herein we present a case of BLP arising in lichen planus, a chronic inflammatory disease. We propose Wolf's isotopic response, or the appearance of a new skin disease at the site of an existing and unrelated disease, as the underlying molecular mechanism responsible for this unusual physical presentation. It is important that clinicians recognize atypical morphologies such as BLP, which mimics squamous cell carcinoma both clinically and pathologically. These similarities highlight the need for a tissue diagnosis to identify infectious etiologies and rule out malignancy when BLP is suspected. J Drugs Dermatol. 2018;17(2):233-235.

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.

Rhinophyma-like hypertrophy of the nose caused by chronic facial pyoderma in a patient with Crohn's disease.

We present our experience with a 22-year-old man who had Crohn's disease with rhinophyma-like hypertrophy of the nose arising from pyogenic skin disease of the face. The clinical appearance did not precisely match any previously reported skin diseases.

A case of pyodermatitis-pyostomatitis vegetans.

Pyodermatitis-pyostomatitis vegetans (PD-PSV) is a rare disorder characterized by mucocutaneous involvement and associated with inflammatory bowel disease. A 42-year-old woman with ulcerative colitis who manifested verrucous and pyogenic lesions on her scalp, neck, axillae, inguinal areas, umbilicus, trunk and oral cavity for about 11 months is described. She also experienced general fatigue and swelling in her lower extremities. Histology revealed eosinophilic inflammation with microabscesses and pseudoepitheliomatous hyperplasia, but she was negative on direct immunofluorescence for IgA, IgG and C3. She was diagnosed with PD-PSV and treated with infusions of 20% human albumin (100 mL) for 5 days, followed by methylprednisolone (40 mg/d), with remission of lesions observed after 1 month. The differential diagnosis of PD-PSV and pemphigus vegetans is discussed.

Common skin conditions in children with HIV/AIDS.

The most recent Joint United Nations Programme on HIV/AIDS (UNAIDS) data inform us that approximately 2.3 million children were infected with HIV at the end of 2009. The greatest burden of this infection is thrust squarely on the most impoverished healthcare systems in the world. Sub-Saharan Africa is home to at least 68% of the global total of HIV infection of 22.5 million. Although a scale up of antiretrovirals has been one of the UNAIDS priorities, and access to services to prevent mother-to-child transmission has increased, an estimated 370,000 children were newly infected in 2009. Hence, infected mothers continue giving birth to HIV-infected children who require appropriate healthcare to diagnose and treat their underlying immunodeficiency and related disorders. Skin lesions are common in these children as they present with infections common in the general population, albeit more severe. Those lesions that are markers of HIV or AIDS are important signs heralding an improving or declining immune system and the success of antiretrovirals. Cutaneous manifestations of HIV/AIDS can be classified broadly as infections and infestations, inflammatory conditions, tumors, and antiretroviral related. This manuscript discusses the more common skin conditions seen in children infected with HIV so as to improve the diagnosis and therapy administered by healthcare professionals especially in sub-Saharan Africa.

Surgery for Pyodermia fistulans sinifica to circumvent heart transplantation (Case Report).

INTRODUCTION: Pyoderma fistulans sinifica (PFS) is a rare chronic cutaneous and subcutaneous infectious disease and is associated with major physical and psychological morbidity. In this article, we present the case of a young patient affected by severe PFS, who concomitantly suffered severe left-sided cardiac insufficiency and was considered for cardiac transplantation. By radical debridement and skin grafting of all areas affected by PFS, the patient's overall condition rapidly improved and was correlated with a significant and measurable improvement of cardiac function. CASE REPORT: Plastic surgery was consulted on a 32-year-old male by the cardiac intensive care unit with a 2-year history of recurrent pyogenic skin infections of the inguinal and axillary regions. He presented with a dilated cardiomyopathy with an ejection fraction (EF) of 15%, first-degree pulmonary insufficiency and second-degree mitral insufficiency and an overlying myocarditis leading to the picture of severe cardiac insufficiency, making him eligible for heart transplantation. The intention was to eradicate the chronic infective wounds as a preparative prior to cardiac transplantation. Radical debridement of the involved areas and coverage with split-thickness skin grafts took place and led to a significant improvement of the patient's condition. Echocardiography revealed a 266.67% improvement of the EF to 40%. Cardiac transplantation became unwarranted. At 1.5 years postoperatively, the patient is free of disease and has a normal cardiac function. DISCUSSION: PFS is characterised in its early stages by local inflammation with beginning ulceration and can be managed conservatively. In advanced stages, as illustrated in this report, PFS must be considered as the potential source of systemic inflammatory process with significant alterations in multiple-organ systems and must be treated aggressively.

A case of blastomycosis-like pyoderma caused by mixed infection of Staphylococcus epidermidis and Trichophyton rubrum.

Blastomycosis-like pyoderma (BLP) is a type of chronic pyoderma characterized histologically by specific epidermal changes namely: pseudoepitheliomatous hyperplasia and intraepithelial abscesses. These epidermal changes are also seen in blastomycosis (referred to as deep dermatophytosis in North America). Here, we describe the case of a 53-year-old male with prurigo nodularis, diabetes, and chronic lymphocytic leukemia who presented with multiple yellowish-red colored papules that coalesced to form a vegetating plaque. In addition to the typical features of BLP, spores with budding were seen histopathologically in a biopsy specimen. Cultures of a skin specimen grew Staphylococcus epidermidis and Trichophyton rubrum. Antibiotic therapy was effective but failed to eliminate the lesion until antifungal therapy using terbinafine was administered concurrently. Past reports suggest that BLP is mainly caused by bacterial infection, but our case suggests that fungal infection can also be involved as the causative organism in BLP.

All that glitters is not pemphigus: Pyodermatitis-pyostomatitis vegetans misdiagnosed as IgA pemphigus for 8 years.

Pyodermatitis-pyostomatitis vegetans is a rare mucocutaneous dermatosis often associated with gastrointestinal disorders, especially with inflammatory bowel disease. It is clinically characterized by erythematous lesions with multiple pustules and erosions affecting the mucosal surfaces. Cutaneous lesions are characterized by exudative and vegetating plaques affecting frequently the axillae and groins. The clinical diagnosis is supported by histologic findings, whereas immunofluorescence studies are useful to rule out other entities such as pemphigus3. Herein we report the case of a young man who was misdiagnosed as having IgA pemphigus for 8 years due to positive immunofluorescence findings. The clue for the final diagnosis was the diagnosis of a concomitant ulcerative colitis, which prompted us to reconsider his cutaneous disease.

Two cases of pyodermatitis-pyostomatitis vegetans.

Pyodermatitis-pyostomatitis vegetans (PPV) is a rare chronic pustular and vegetating mucocutaneous dermatosis. The oral lesions present as multiple, friable and yellowish pustules, which form "snail tracts" and rupture easily. The cutaneous lesions begin as crusted erythematous papulopustules that coalesce to form large vegetating plaques, usually in the axillae, genital area and scalp. Cutaneous lesions usually develop at the same time as the oral lesions or thereafter. PPV is usually considered as a specific marker for inflammatory bowel disease since the concurrence of PPV and inflammatory bowel disease has been reported in approximately 70% of cases. We report two patients who showed typical clinical and histopathological features of PPV, but were not accompanied by inflammatory bowel disease.

[Multicentric study on a topical compound with lymph-draining action in the treatment of the phlebostatic ulcer of the inferior limbs]. / Studio multicentrico su un preparato topico ad azione linfodrenante nel trattamento dell'ulcera flebostatica degli arti inferiori.

Phlebostatic sore of the lower limbs is a typical chronic venous insufficiency complication and is still a widely controversial issue in its treatment. The common therapies, in fact, are not yet standardized and they not show complete efficacy. Since 2005 to 2007 a multicentric clinical trial was conducted at the Plastic and Reconstructive Surgery of "Sapienza" University of Rome and at the Plastic and Reconstructive Surgery Department, University of Perugia, in order to evaluate the efficacy of the Idrastin lymph-draining cream in patients with phlebostatic sores of the lower limbs. This study enrolled on 80 patients, split into 2 homogeneous groups of 40 patients: group A was treated by only elastocompressive therapy, group B by elastocompressive therapy and Idrastin. Multicentric analysis has considered the following parameters: local pain, perilesional flogosis , granulation tissue, perilesional tissue tropism healing time. In the group B results highlighted: reduction of the local pain, stopped in 72 hours; flogosis decrease disappeared in one week; tissue granulation growth in one week; lesion healing in 4 weeks. These results pointed out statistically significance of the variables considered. In our opinion Idrastin compounds such as phytoessence of hops and Hedera helix, had contributed to analgesia; Aesculus hippocastanum, and Vitis vinifera and Ruscus aculeatus phytoessence showed anti-flogistic action; allantoin and Centella asiatica and jaluronic acid aided to sore healing. Idrastin gives an effective support to the treatment of the phlebostatic sores warrants a faster and more effective healing process, than to the wounds treated by only the elastocompressive therapy.

The association of pyoderma faciale and erythema nodosum.

Pyoderma facial (PF) is a sudden severe eruption of pustules and cystic swellings which may be interconnected by sinuses. It affects mainly adult women. We report the case of a 21-year-old woman presenting with sudden onset of coalescing nodules and abscesses on the face, with mild systemic disturbance. She also had erythema nodosum (EN)-like lesions on the legs. To the best of our knowledge, this is the first reported case of PF associated with EN.

Comparative dermatology--canine endocrine dermatoses.

Endocrine diseases in the dog commonly manifest with dermatological lesions. Hypothyroidism is the most common endocrinopathy and usually presents with alopecia in areas of wear, seborrhea, and recurrent infections. Common clinical signs associated with hyperadrenocorticism include polyuria, polydipsia, and polyphagia. The most common dermatological manifestation of hyperadrenocorticism is bilaterally symmetrical alopecia sparing the head and distal extremities. Pyoderma is a common finding associated with immunosuppression. Less commonly, calcinosus cutis may occur. Sex hormone excess, primarily hyperestrogenism and hyperandrogenism, may also be associated with dermatological signs. Usually, dogs are intact, and the excess production is due to testicular or ovarian neoplasia.