Hepatoerythropoietic porphyria: neuroimaging findings.

CT and MR findings in two patients with hepatoerythropoietic porphyria are presented. CT scans showed atrophy and cortical mineralization at the same level. MR examination performed in one of the two patients showed mainly frontal cortical atrophy and punctate bright signal on T1- and T2-weighted sequences.

Milia en plaque.

A 59-year-old woman presented with milia grouped in plaques, in the preauricular areas, bilaterally. Follicle-damaging dermatoses, tumours and external agents, can lead to this peculiar clinical pattern. We outline the clinical and histological features which allow accurate diagnosis of this condition.

[Basal cell nevus syndrome. Apropos of 2 new cases]. / Síndrome del nevus basocelular. A propósito de dos nuevos casos.

Two cases affected by the nevoid basal cell carcinoma syndrome are reported herein with multiple basal cell carcinomas on exposed areas, with the previous appearance of pigmented macules on which a morphological transformation was shown with the presence of erythematous papules and plaques in a ring-like, formation made up of epitheliomatous pearls, giving the appearance of lesional polymorphism. The clinical pathological diagnosis of epithelioma basocellular is confirmed. In both patients, numerous pits on the hands and feet are shown without noticeable subjective symptoms; one of the biopsies reveals a carcinoma of basal cells in the epidermis underlying the pit. A detailed study is carried out on both families with the existence of different osseous anomalies, without finding cutaneous stigmas. The treatments carried out are checked bringing in our experience with the locally applied 5-FU. It is clearly obvious that UV light is a key factor in these two cases.

Jadassohn's intraepidermal epithelioma.

We present a case which may be labeled Jadassohn's intraepidermal epithelioma, but which has led us to revise the problem of intraepidermal neoplasias, and establish whether or not this entity possesses distinct features which would permit us to differentiate it.

[Early familial colloid milium]. / Milio coloide precoz familiar.

The authors present here a case report of colloid milium beginning early in the childhood , with affectation of four relatives in the same generation, suggesting a pattern of inheritance in a recessive way.

[Gougerot's lupus erythematosus tumidus]. / Lupus eritematoso tumidus de Gougerot.

A case of LEDC is reported, with clinic, histopathological and immune-fluorescence studies. The injuries start suddenly on the face and neck with a capricious evolution, reaching a complete disappearance without leaving neither scar nor atrophy, returning in few time in zones normally not exposed to the sun, as subchin region, low neck zone and lateral aspects of the neck continuing on the level of the skin of the head. The clinic study of the injuries call to mind the form described by Gougerot in 1930 of his túmidus and by the evolution of the same, the forma described by Gougerot and Carteaud, denominating it "à eclipse".

Two cases in infantile and familial porphyria cutanea tarda.

In 2 children, both 7 years old, and with an obvious presence of clinical symptoms, the diagnosis of porphyria cutanea tarda was corroborated by the characteristic patterns of urinary and fecal prophyrin excretion. Familial studies allowed us to consider both cases as hereditary.