Treatment of Pyodermatitis-Pyostomatitis Vegetans: A Systematic Review and Meta-analysis

Background: Pyodermatitis-pyostomatitis vegetans (PDV-PSV) is a rare muco-cutaneous disorder characterized by vegetating and pustular plaques and is often associated with inflammatory bowel disease (IBD). The purpose of this study was to systematically identify and analyze reports of PDV-PSV to determine the most effective treatment. Methods: Reports of PDV-PSV were identified using the OVID-Medline database from inception through November 2019. Publications were excluded if no new patient case was included, if there was not clinical and histological evidence of PDV, PSV, or PDV-PSV, or if no treatment was discussed. Results: The final sample was comprised of 74 publications plus an additional patient from the authors’ institution, corresponding to 95 total patients. The basis of the review and analysis is limited to case reports and case series, which likely only report the cases with positive outcomes. Statistical analysis revealed that oral corticosteroids (OCS), 6-mercaptopurine/azathioprine, oral calcineurin inhibitors (OCNI), 5-aminosalicylic-acid (5-ASA), and biologics (BIO) were the most effective treatments for PDV-PSV. Topical medications, colchicine, oral dapsone, and other antibiotics were ineffective treatments, with topical medications being the least effective option. When OCS are used, they work best when used as initial treatment to induce remission. 5-ASA and BIO are most effective when used as maintenance therapies after initial remission. Conclusions: Thus, first line therapy for PDV-PSV should begin with OCS with transition to steroid-sparing agents including OCNI, BIO, and 5-ASA if indicated.

Dermatological Manifestations in Inflammatory Bowel Diseases

Inflammatory bowel diseases (IBDs) may be associated with extra-intestinal manifestations. Among these, mucocutaneous manifestations are relatively frequent, often difficult to diagnose and treat, and may complicate the course of the underlying disease. In the present review, a summary of the most relevant literature on the dermatologic manifestations occurring in patients with inflammatory bowel diseases has been reviewed. The following dermatological manifestations associated with IBDs have been identified: (i) specific manifestations with the same histological features of the underlying IBD (occurring only in Crohn’s disease); (ii) cutaneous disorders associated with IBDs (such as aphthous stomatitis, erythema nodosum, psoriasis, epidermolysis bullosa acquisita); (iii) reactive mucocutaneous manifestations of IBDs (such as pyoderma gangrenosum, Sweet’s syndrome, bowel-associated dermatosis-arthritis syndrome, aseptic abscess ulcers, pyodermatitis–pyostomatitis vegetans, etc.); (iv) mucocutaneous conditions secondary to treatment (including injection site reactions, infusion reactions, paradoxical reactions, eczematous and psoriasis-like reactions, cutaneous infections, and cutaneous malignancies); (v) manifestations due to nutritional malabsorption (such as stomatitis, glossitis, angular cheilitis, pellagra, scurvy, purpura, acrodermatitis enteropathica, phrynoderma, seborrheic-type dermatitis, hair and nail abnormalities). An accurate dermatological examination is essential in all IBD patients, especially in candidates to biologic therapies, in whom drug-induced cutaneous reactions may assume marked clinical relevance.

An unusual pediatric clinical case of atypical pyoderma gangrenosum

Pyoderma gangrenosum (PG) belongs to the group of neutrophilic dermatoses with unknown etiology and poorly understood pathogenesis. In children, PG is primarily associated with inflammatory bowel diseases (Crohn's disease and ulcerative colitis). By now, about 355 PG cases have been described worldwide, including 15 children with the involvement of oral mucosa. Clinical manifestations of the disease are diverse and depend on the form, stage and associated conditions. Such a rare PG as pyodermatitis-pyostomatitis vegetans manifests by combined lesions of the oral mucosa and skin. The authors present historical data on the investigation of the disease, its etiology, pathogenesis, risk factors, and clinical picture. A description of a rare clinical case of severe form of atypical PG, namely, pyodermatitis-pyostomatitis vegetans in a 10-year-old child, is presented. The unique character of the case is related to the variety of clinical manifestations and the clinical course complicated by the development of keloid and partial scar-related flexion contracture of the upper and lower extremities, the formation of microstoma and cachexia. The association of pyodermatitis-pyostomatitis vegetans with Crohn's disease was found. The lack of awareness of the clinical symptoms of this rare disease has led to diagnostic errors and late diagnosis.

Dermatological Manifestations in Pediatric Inflammatory Bowel Disease

Background and Objectives: Over the last years, inflammatory bowel disease (IBD) has been reported on a high incidence in pediatric populations and has been associated with numerous extraintestinal manifestations, making its management a real challenge for the pediatric gastroenterologist. Dermatological manifestations in IBD are either specific, related to the disease activity or treatment-associated, or non-specific. This literature review aims to identify and report the dermatological manifestations of IBD in children, the correlation between their appearance and the demographical characteristics, the relationship between these lesions and disease activity, and to highlight the impact of dermatological manifestations on an IBD treatment regime. Materials and Methods: A systemic literature review was performed, investigating articles and case reports on dermatological manifestations in children with IBD starting from 2005. A total of 159 potentially suitable articles were identified and after the exclusion process, 75 articles were selected. Results: The most common dermatological manifestations reported in pediatric IBD are erythema nodosum and pyoderma gangrenosum. More rare cases of metastatic Crohn’s disease, epidermolysis bullosa acquisita, small-vessel vasculitis, necrotizing vasculitis, leukocytoclastic vasculitis, cutaneous polyarteritis nodosa, and Sweet’s syndrome have been reported. Oral manifestations of IBD are divided into specific (tag-like lesions, mucogingivitis, lip swelling with vertical fissures, aphthous stomatitis, and pyostomatitis vegetans) and non-specific. IBD treatment may present with side effects involving the skin and mucosa. Anti-tumor necrosis factor agents have been linked to opportunistic skin infections, psoriasiform lesions, and a potentially increased risk for skin cancer. Cutaneous manifestations such as acrodermatitis enteropathica, purpuric lesions, and angular cheilitis may appear secondary to malnutrition and/or malabsorption. Conclusions: The correct diagnosis of dermatological manifestations in pediatric IBD is of paramount importance because of their impact on disease activity, treatment options, and a patient’s psychological status.