The onychomatricoma: an electron microscopic study.

The electron microscopic findings in the onychomatricoma are described. In the proximal zone of the onychomatricoma, basal cells have various aspects, some being lacunar while others have only a limited cytoplasmic rim containing mitochondria and tonofilaments. In the parakeratotic cell columns the cells elongate and homogenized tonofilaments appear.

[Dermatology in current medicine].

Dermatology evolved in the second part of the last century as a branch of internal medicine and was for many years confined to morphologic descriptions. Skin diseases are common and vary enormously in severity. Although most of the conditions are not life threatening, many of them are debilitating due to functional loss, pain and itch, and the social problems they cause.

Genetic linkage between the collagen type VII gene COL7A1 and pretibial epidermolysis bullosa with lichenoid features.

Pretibial epidermolysis bullosa is a rare form of dominant dystrophic epidermolysis bullosa. The disease was diagnosed after considerable delay in a large Belgian family and was remarkable for its late age at onset and its misleading clinical presentation in the proband, which strongly resembled keratosis lichenoides chronica. Both recessively and dominantly inherited forms of dystrophic epidermolysis bullosa have been shown to be linked to the collagen type VII gene, COL7A1.

The Bazex-Dupré-Christol syndrome.

Background: The Bazex-Dupré-Christol syndrome is characterized by follicular atrophoderma, congenital hypotrichosis, and basal cell neoformations that include basal cell carcinomas and basal cell nevi.

Observations: We describe a large family in which 20 persons across four generations present with typical features of the Bazex-Dupré-Christol syndrome. However, the clinical picture in this family differs with regard to gender and age.

Pharmacokinetics of acitretin.

Acitretin, the metabolite of etretinate, is eliminated far more rapidly from the human body than is etretinate. It has therefore been suggested that only a short period of contraception would be required following the completion of long-term therapy. However, recent studies have demonstrated the presence of etretinate in the plasma of acitretin-treated patients.

Skin, adipose tissue and plasma levels of acitretin with rare occurrence of esterified acitretin during long-term treatment.

In a previous study acitretin and its 13-cis-metabolite were monitored in the plasma and epidermis of healthy volunteers. They were given 50 mg of trans-acitretin daily. No drug accumulation was observed in the skin, nor in the plasma.

[Acute hemorrhagic edema in an infant].

The clinical and histological features of acute haemorrhagic oedema of childhood are discussed with reference to an actual case. Although closely related to Schoenlein-Henoch syndrome, it is considered a distinct entity, on the basis of the typical clinical picture and the excellent prognosis.

Pharmacokinetics and drug interactions of etretinate and acitretin.

Acitretin, the metabolite of etretinate, is eliminated far more rapidly from the human body than is etretinate. It had therefore been suggested that only a short period of contraception would be required after the cessation of long-term therapy with acitretin. However, recent studies have demonstrated the presence of etretinate in the plasma of patients who were treated with acitretin.

Onychomatrixoma. Filamentous tufted tumour in the matrix of a funnel-shaped nail: a new entity (report of three cases).

Identical lesions of the nails were observed in three patients. The main signs were a yellow colouration along the entire length of the nail plate with splinter haemorrhages in its proximal portion, a tendency towards transverse overcurvature of the affected nails, and exposure of a matrix tumour after the nail had been avulsed and the proximal nail fold turned back. The nails signs were striking enough to lead to the clinical suspicion of a filamentous tufted tumour in the matrix of a funnel-shaped nail, an entity not previously described.

The distribution of cis- and trans-acitretin in human epidermis.

The concentrations of trans-acitretin and its principal metabolite, cis-acitretin, were measured by h.p.l.

Acitretin as cancer chemoprophylaxis in a renal transplant recipient.

The use of acitretin in a renal transplant recipient who had been treated for several premalignant and malignant skin lesions is the subject of this case report. During the treatment period no new dysplastic lesions developed.

Use of topical lithium succinate in the treatment of seborrhoeic dermatitis.

Twenty-one patients with seborrhoeic dermatitis were included in an open trial of lithium succinate ointment (LSO) for a total duration of 8 weeks. The same clinician made assessments of the severity of redness, scaling, greasiness and overall clinical impression of the condition every 2 weeks. Because the results appeared to be satisfactory, we decided to perform a double-blind, placebo-controlled trial of LSO.

Improved quantitation of 13-cis- and all-trans-acitretin in human plasma by normal-phase high-performance liquid chromatography.

A reliable analytical method has been developed for measurement of 13-cis- and all-trans-acitretin (Neotigason) in human plasma by normal-phase high-performance liquid chromatography, with ultraviolet detection. Human plasma was obtained after centrifugation of whole blood samples and deproteinized by ethanolic denaturation. After liquid-liquid extraction with water-n-hexane, and aliquot ws chromatographed on a silica column using isocratic elution with n-hexane-methylsalicylate-acetic acid (200:18:0.

Pseudoxanthoma elasticum: similar autosomal recessive subtype in Belgian and Afrikaner families.

A multidisciplinary survey of the clinical and genetic characteristics of 26 Belgian and 32 Afrikaner families with biopsy-proven pseudoxanthoma elasticum (PXE) was undertaken. The major PXE phenotype emerging from this study is very similar in both patient groups and is characterized by severe ophthalmologic manifestations with variable, mild cutaneous and vascular symptoms. In the families with more than one affected relative, segregation analysis is compatible with autosomal recessive inheritance in both groups.

Repeated cultured epidermal allografts in the treatment of chronic leg ulcers of various origins.

Twelve patients with 16 leg ulcers, existing for at least 3 months and not responsive to conventional inpatient therapy of at least 3 weeks, were treated with repeated applications of cultured allogenic keratinocyte sheets. A marked decrease in size was seen in all ulcers but 2. Complete closure of the ulcer was seen in 62% of the ulcers within 8 weeks.

High-performance liquid chromatographic determination of etretinate and all-trans- and 13-cis-acitretin in human plasma.

A high-performance liquid chromatographic procedure is described for the simultaneous determination of etretinate (Tigason), all-trans-acitretin (Neotigason) and 13-cis-acitretin in human plasma. The compounds are extracted from the plasma with n-hexane under acidic conditions. Quantification is performed on a normal-phase column (CP-Spher Si, 5 microns), followed by UV detection at 350 nm.

Histopathologic study of Koenen tumors. Are they different from acquired digital fibrokeratoma?

Fifteen periungual fibromas from nine patients were studied histologically. The lesions were elongated. Capillaries were found in the distal part surrounded by thin collagen bundles whereas the proximal part was made up of dense, closely packed fibers.