Prevalence of autosomal recessive congenital ichthyosis: a population-based study using the capture-recapture method in Spain.

Background: Previous reports on the prevalence of autosomal recessive congenital ichthyosis (ARCI) were based on single source data, such as lists of members in a patient association. These sources are likely to be incomplete.

Objectives: We sought to describe the prevalence of ARCI.

Primary cutaneous localized amyloid elastosis.

Amyloid elastosis is a rare form of cutaneous amyloidosis characterized histologically by the deposition of amyloid-coating elastic fibers of the skin. To date, only 4 cases have been reported, all of them in the setting of systemic amyloidosis. We present the first case of primary cutaneous localized amyloid elastosis.

A new scalp formulation of calcipotriene plus betamethasone compared with its active ingredients and the vehicle in the treatment of scalp psoriasis: a randomized, double-blind, controlled trial.

Background: New topical treatments in scalp psoriasis are needed because many current topical treatments are disliked by patients and associated with poor compliance.

Objective: To compare the efficacy and safety of once-daily, two-compound scalp formulation containing calcipotriene plus betamethasone dipropionate with the individual components in the same vehicle and the vehicle alone.

Methods: In this 8-week, multicenter, randomized, double-blind study, patients with scalp psoriasis were randomized to treatment with the two-compound scalp formulation (calcipotriene 50 microg/g plus betamethasone 0.

Cutaneous apocrine cystic adenolipoma.

Adenolipoma is a rare microscopic variant of cutaneous lipoma composed of large lobules of mature adipocytic tissue admixed with eccrine ducts and glands. We report a new case of cutaneous adenolipoma, and the first showing an apocrine cystic glandular component. This lesion may not be a specific entity, and could represent entrapment of epithelial structures within a lipoma.

A genetic study of cathepsin C gene in two families with Papillon-Lefèvre syndrome.

Papillon-Lefèvre syndrome (PLS) is an inherited human disorder characterised by premature destruction of the periodontium of the deciduous and permanent teeth, palmoplantar hyperkeratosis, and increased susceptibility to bacterial infections during the first years of life. In this paper two PLS families have been studied. Family 1 presents a novel homozygous mutation (880T>C) in exon 6 causing Y294H amino acid substitution.

Membranous fat necrosis: a non-specific histological finding.

Two patients presented with nodular lesions on their lower limbs. Histologically, the dermis, in one case, and the panniculus, in the other, displayed pseudocystic lesions delimited by a serpiginous membranous structure showing the staining characteristics of ceroid. One patient had sclerosing panniculitis while the other had a traumatic panniculitis.