Port-wine stain as a clue for two rare coexisting entities.

Phakomatosis pigmentovascularis (PPV) is an uncommon dermatosis characterised by the presence of both pigmentary and vascular abnormalities.1 Its pathogenesis is not elucidated, and the prognosis is mainly determined by the presence of extracutaneous manifestations, such as Klippel-Trenaunay syndrome (KTS), that is defined by the triad of a port-wine stain (PWS), anomalous veins and progressive overgrowth of the affected extremity. Herein, we report a case of an adult patient, who presented with a large PWS, nevus of Ota, ocular melanosis, and limb hypertrophy and varicosities. These findings represented a rare association of PPV type IIb and KTS.

Reed's Syndrome.

Multiple cutaneous and uterine leiomyomatosis (MCUL), also known as Reed's syndrome, is a rare genodermatosis, with an autosomal dominant pattern of inheritance. It results from a germline heterozygous mutation of fumarate hydratase gene, that is classified as a tumor suppressor gene. Hereditary leiomyomatosis and renal cell cancer is characterized by the association of MCUL with renal cell carcinoma. We report a case of a 57-year-old woman, with multiple cutaneous leiomyomas as the presenting sign of Reed's syndrome.

Cutaneous nocardiosis by a new pathogenic species: Nocardia grenadensis.

Nocardiosis is a rare, predominantly opportunistic, suppurative disease caused by bacteria of the order Actinomycetales. There are currently more than 100 species of Nocardia described, less than half are pathogenic to humans. Cutaneous nocardiosis can be caused by direct inoculation from a contaminated material or by secondary dissemination. The authors present a 70-year-old man with an autoimmune haemolytic anaemia treated with prednisolone and azathioprine. The patient presented multiple erythematous tender nodules with linear distribution and proximal progression along the left upper limb with 2 months of evolution. The skin biopsy revealed non-specific inflammation with areas of abscess. Culture was positive for bacteria of the genus Nocardia, and molecular techniques revealed Nocardia grenadensis The patient was treated with minocycline with good response, but the disease recurred. N. grenadensis was first identified in 2012 in a bioprospecting process. The authors now describe the first case of cutaneous nocardiosis caused by N. grenadensis.

Pemphigoid gestationis successfully treated with intravenous immunoglobulin.

Pemphigoid gestationis (PG), also known as herpes gestationis, is a rare autoimmune blistering disease specific to pregnancy, which usually presents in the second or third trimesters and, in 15%-25% of cases, during the immediate postpartum period.1Although the ethiopathogeny of PG is not fully clarified, most patients develop antibodies against a 180 kDa transmembrane hemidesmosomal protein (BP180; BPAG2; collagen XVII).2 PG has a strong association with human leucocyte antigens DR3 and DR4.3We report a case of a 29-year-old female patient with PG successfully treated with intravenous immunoglobulin.

Hypotrichosis with Juvenile Macular Dystrophy.

Hypotrichosis with juvenile macular dystrophy is a rare autosomal recessive disease, characterized by hypotrichosis and progressive macular degeneration, leading to blindness in the first three decades of life. It is associated with mutations in the cadherin 3 gene, resulting in the abnormal expression of P-cadherin. We report a case of a 4-year-old female patient diagnosed with this genodermatosis.