Pyoderma gangrenosum: a review with special emphasis on Latin America literature

Abstract Pyoderma gangrenosum is a neutrophilic dermatosis characterized by chronic ulcers due to an abnormal immune response. Despite the existence of diagnostic criteria, there is no gold standard for diagnosis or treatment. In Latin America, recognizing and treating pyoderma gangrenosum is even more challenging since skin and soft tissue bacterial and non-bacterial infections are common mimickers. Therefore, this review aims to characterize reported cases of pyoderma gangrenosum in this region in order to assist in the assessment and management of this condition. Brazil, Mexico, Argentina, and Chile are the countries in Latin America that have reported the largest cohort of patients with this disease. The most frequent clinical presentation is the ulcerative form and the most frequently associated conditions are inflammatory bowel diseases, inflammatory arthropaties, and hematologic malignancies. The most common treatment modalities include systemic corticosteroids and cyclosporine. Other reported treatments are methotrexate, dapsone, and cyclophosphamide. Finally, the use of biological therapy is still limited in this region.

Pioderma gangrenoso vulvar: revisión de la literatura a propósito de un caso causado por rituximab / Vulvar pyoderma gangrenosum: review of the literature on a case caused by rituximab

RESUMEN El Pioderma Gangrenoso (PG) es una enfermedad inflamatoria necrotizante crónica, que pertenece al espectro de las dermatosis neutrofílicas. Histológicamente se caracteriza por mostrar un infiltrado inflamatorio denso de neutrófilos de origen no infeccioso. El PG suele asociarse a enfermedades sistémicas como la enfermedad inflamatoria intestinal, la artritis reumatoide o diversas enfermedades hematológicas. Presenta fenómeno de patergia y suele responder satisfactoriamente a tratamientos inmunosupresores. Su etiología no está bien definida. En la literatura se han publicado 15 casos de pioderma gangrenoso vulvar asociado al uso de rituximab. Nosotros presentamos un nuevo caso, que tuvo lugar en una mujer de 37 años en tratamiento de mantenimiento con rituximab por un linfoma no Hodgkin folicular. El rituximab (MabThera®) es un anticuerpo que reconoce la molécula CD20, que es una proteína no glucosilada que se expresa en la superficie de los linfocitos B. Este fármaco se ha utilizado para el tratamiento de diferentes enfermedades reumatológicas en los últimos años.

ABSTRACT Pyoderma Gangrenosum is a chronic necrotizing inflammatory disease that belongs to the spectrum of Neutrophilic Dermatoses. Histologically, it is characterized by a dense inflammatory infiltrate of non-infectious neutrophils. Etiology is not yet well defined. It is usually associated with systemic diseases such as inflammatory bowel disease, rheumatoid arthritis or hematological diseases. It presents pathergy phenomenon and usually respond satisfactorily to immunosuppressive treatments. There have been published only 15 cases of vulvar pyoderma gangrenosum associated with the use of rituximab. We present a new case, which occurred in a 37-year-old woman on maintenance treatment with rituximab for a follicular non-Hodgkin's lymphoma. Rituximab (MabThera®) is an antibody that recognizes the CD20 molecule, which is a non-glycosylated protein that is expressed on the surface of B lymphocytes. This drug has been used for the treatment of different rheumatic diseases in recent years.

Dermatologic manifestations in inflammatory bowel disease in Tunisia

Cutaneous manifestations are the most common extra intestinal manifestations associated with inflammatory bowel disease [IBD]. To assess the epidemio-clinical profile of skin manifestations in IBD. A prospective and descriptive study was conducted. We have examined skin, mucosa, hair and nails, of all patients with an IBD during one year. One hundred-ninety-five patients were included. Crohn's disease [CD] was noted in 154 cases [79.8%], ulcerous rectocolitis [UC] in 39 cases [21.2%] and inclassable IBD in 2 cases. Cutaneous manifestations were found in 91% of Crohn's patients and in 92% of UC patients. Granulomatous perianal skin lesions were the main cutaneous manifestations of CD [53%]. The most common affected sites were ano-perineal fistulae, perianal and perineal fissures and oedematous and infiltrated perianal and genital plaques. Reactive lesions [Erythema nodosum, Pyoderma gangrenosum, Aphthous stomatitis] were noted in 14 cases. Skin manifestations due to malabsorption were also frequently observed [101 cases: 51.7%].Other dermatoses implicating various mechanisms such as psoriasis, alopecia areata, vitiligo, rosacea, lichen planus, were also noted. Adverse skin manifestations due to treatment [folliculitis, acne, macula-papular rash and DRESS syndrome] were present in 16 cases. Our series is characterized by a high frequency of cutaneous manifestations associated to IBD. A better recognition of these skin manifestations by the physician may improve their management