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.

Abstract: Although wound or traumatic myiasis is common in tropical countries, only recently cases associated with underlying dermatoses, such as seborrheic dermatitis and psoriasis, have been reported. We describe a patient with seborrheic dermatitis and an ulcerated lesion on the scalp, in which the dermatological examination with the aid of dermoscopy allowed the identification of larvae (maggots) compatible with infestation by Cochliomyia hominivorax. Treatment was performed with oral and topical ivermectin, followed by manual extraction of the larvae.

Abstract: Atopic dermatitis is a common inflammatory skin disease. New understanding in disease pathogenesis has led to a considerable number of promising new drugs in development. New topical agents can be especially helpful for children, providing an alternative to the need for chronic topical corticosteroid use. While many patients with mild or moderate disease can be managed with topical treatments, there are unmet needs for recalcitrant and severe cases. New and developing therapies hold promise for real advances in management of this complex disease.

Abstract: Systemic light chain amiloydosis is a rare disease. Due to its typical cutaneous lesions, dermatologists play an essential role in its diagnosis. Clinical manifestations vary according to the affected organ and are often unspecific. Definitive diagnosis is achieved through biopsy. We report a patient with palpebral amyloidosis, typical bilateral ecchymoses and cardiac involvement, without plasma cell dyscrasia or lymphomas. The patient died shortly after the diagnosis.

Abstract: Metastatic Crohn's disease is a rare skin manifestation, defined by granulomatous skin lesions that are discontinuous to the affected gastrointestinal tract and histopathologically resembling inflammatory bowel lesions. Up to 44% of patients with Crohn's disease have cutaneous manifestations, of which metastatic lesions are the least common. We present a case of an adolescent with refractory Crohn's disease and persistent papules and plaques on the skin.

A síndrome de Urbach-Wiethe, também conhecida como lipoidoproteinose ou hialinose cutânea e mucosa (OMIM 247100), é rara genodermatose de herança autossômica recessiva, de patogênese desconhecida, que se caracteriza pela deposição de material hialino na pele, mucosa oral e órgãos internos. Alterações características incluem a rouquidão, pápulas e nódulos na face, especialmente na borda das pálpebras, cicatrizes varioliformes, espessamento difuso e aspecto amarelado da pele e lesões verrucosas em áreas de atrito.

Urbach-Wiethe disease, also named lipoproteinosis or hyalinosis cutis et mucosae (OMIM 247100), is a rare autosomal recessive disorder of unknown pathogenesis with hyaline-like material deposition in the skin, oral mucosa and internal organs. Typical features include hoarseness, papules and nodules on the face, characteristically along the eyelid margins, pitted scars, diffuse thickening and yellow discoloration of the skin, and verrucous lesions in areas subjected to trauma.

A síndrome de Alagille, doença autossômica dominante de expressividade variável, é uma das causas mais freqüentes de hepatopatia crônica na infância. Sua principal característica é a escassez de ductos biliares intra-hepáticos, com repercussões variáveis sobre o órgão. O quadro cutâneo caracteriza-se por prurido intenso, muitas vezes intratável, e múltiplos xantomas.

Alagille syndrome, an inherited autosomal disorder of dominant trait, is one of the most common causes of chronic liver disease in childhood. Its hallmark is the paucity of intrahepatic bile ducts, with variable degrees of liver impairment. Cutaneous manifestations include intense pruritus, often refractory to treatment, and xanthomata.

A síndrome de Wells é dermatose inflamatória benigna rara, de curso recorrente, descrita em todas as idades e sem predileção por sexo, de etiologia e patogênese desconhecidas. Embora sua autonomia seja questionada, a síndrome de Wells é entidade distinta cujo diagnóstico se faz pela combinação de quadro clínico marcante, achados histopatológicos típicos quando associados à clínica, e curso característico.

Wells’ syndrome is a rare, benign, recurrent, inflammatory dermatosis affecting all age groups, with no sex predilection. Its etiology and pathogenesis remain unknown. Although its autonomous existence is questioned by some, Wells’ syndrome is a distinct disease entity diagnosed by the combination of a striking clinical picture, typical histological finding when associated to the clinical features, and a characteristic time course.