Malignant blue nevus of the vulva with late ovarian metastasis.

We report a case of malignant blue nevus (MBN) of the vulva which led to an ovarian metastasis 15 years after the initial diagnosis. To our knowledge, this is the first report of MBN of the vulva. The patient was a 28-year-old woman with a nodule of the labia majora. The patient noted the lesion when she was 12 and stated it did not change at that time. The lesion had the histopathologic features of MBN and was characterized by a low mitotic rate but substantial cytologic atypia. No abnormality was noticed over a 15 year period of follow-up. Fifteen years after the initial diagnosis, vaginal examination and ultrasonography imaging showed a 6.5 cm solid ovarian tumor. Hysterectomy and bilateral salpingo-oophorectomy was performed. Histologic evidence of metastasis was identified in the ovary. Malignant blue nevus can lead to late metastasis and needs long-term follow-up, as do other types of melanoma. Despite low mitotic activity in a melanocytic lesion resembling cellular blue nevus, a careful assessment of cytologic atypia is necessary before ruling out malignancy.

Cutaneous manifestations of methylmalonic and propionic acidaemia: a description based on 38 cases.

Methylmalonic and propionic acidaemias are rare metabolic disorders with an autosomal recessive mode of inheritance. A number of aminoacidopathies may have cutaneous manifestations, but these are usually absent in methylmalonic and propionic acidaemia. We have studied 38 children with propionic and methylmalonic acidaemia in the last 10 years at the Hôpital Necker-Enfants Malades. Thirteen had cutaneous manifestations: acute superficial scalded skin and superficial desquamation, bilateral and periorificial dermatitis, psoriasiform eruptions, and alopecia. The relative uniformity of these manifestations (scalded skin and desquamation after metabolic decompensation, chronic bilateral and periorificial dermatitis) suggests that methylmalonic and propionic acidaemias should be included in the category of aminoacidopathies with cutaneous manifestations. All these patients were suffering from severe forms of these diseases, with no residual enzyme activity, and they were all subjected to a very severe natural protein-restricted diet. These cutaneous manifestations may therefore either be part of a complex multideficiency syndrome, or be due to the enzyme deficiency itself.

[Cutaneous metastasis disclosing adrenal cortical carcinoma]. / Métastase cutanée révélatrice d'un corticosurrénalome.

Metastases to the skin from internal malignant neoplasms are uncommon and often preterminal event. Cutaneous metastases from an adrenal cortical carcinoma have rarely been reported even in the advanced stages of the disease. A patient was initially seen with a small cutaneous lesion on the cheek and was found to have a large adrenal cortical tumor. Histologic comparison proved that the cutaneous lesion was a metastase from an adrenal cortical carcinoma.