Patricia Della Giovanna

Necrotizing gangrene of the genitalia and perineum.

Background  Necrotizing gangrene of the genitalia and perineum is a fulminant, life‐threatening condition. This infection is usually polymicrobial and may be idiopathic or secondary to local trauma or surgery. Histologically, it is characterized by obliterative endarteritis and thrombosis of the subcutaneous vessels, fascial necrosis, and leukocytic infiltration. Mortality rates of 25–75% have been reported. Most cases of necrotizing gangrene begin insidiously, with scrotal discomfort and malaise. Later, erythema, increasing pain, and swelling, associated with fever and chills, develop. A biopsy is useful to confirm the clinical diagnosis and to obtain culture samples. Ultrasound imaging may reveal gas or testicular involvement and may help to distinguish this infection from other causes of scrotal pathology.

Actinomyces meyeri cutaneous actinomycosis

Actinomyces meyeri cutaneous actinomycosis is a very rare disease. It often results from contiguous dissemination of an underlying focus. We report a case of pulmonary actinomicosis with secondary cutaneous involvement which led to the diagnosis.

Prolidase deficiency: case reports of two Argentinian brothers.

A 28‐year‐old man presented with his first leg ulcer at the age of 10 years. With regard to the family history, there was no consanguinity between the parents, and the patient had a brother with similar findings. He had frequent infections in childhood, such as otitis media, tuberculosis, and septic arthritis. At the age of 11 years, he showed photosensitivity, malar erythema, and positive antinuclear antibody test (1 : 40), and systemic lupus erythematosus was diagnosed ( Fig. 1 ). This disease was later ruled out because diagnostic criteria were not present.

Syndromic nevoid hypermelanosis: description of seven cases with a 10-year follow up.

Skin lesions can often be the only sign of an underlying systemic abnormality which will require further investigation. Several syndromic conditions are diagnosed after their cutaneous marker, which is in most cases a nevus. We report a neurocutaneous condition which we named “syndromic nevoid hypermelanosis” (SNH). We studied seven patients who presented with hyperpigmented disseminated macules (melanotic or pigmented nevi) as a cardinal sign. Neurological abnormalities were detected in all cases and skeletal dysmorphism in four. In spite of the genetic alteration that may be the cause of this disease, dermatologists should be able to diagnose it based on its semiological features and distinguish it from other neurocuataneous conditions. We consider SNH to be a distinct clinical entity that has not been clearly defined until now.

Síndromes cutáneos con poliposis intestinal

Existe una serie de síndromes caracterizados por poliposis intestinal, que tienen marcadores cutáneos característicos. Con sentido didáctico y pronóstico, podemos dividirlos según indica la tabla I. Esta división es importante, pues diferencia los pólipos verdaderos de los seudopólipos (como en las neurofibromatosis, que si bien en el estudio de imágenes parecen pólipos, se trata de neurofibromas que asientan en la mucosa); y los adenomatosos (con alto potencial de malignización) de los hamartomatosos (con extremadamente bajo potencial degenerativo). En la actualidad se les agrupa según el gen causal y su expresión fenotípica (tabla II).