Hypermelanocytic guttate and macular segmental hypomelanosis.

We report two sisters, 27 and 30 years of age, with a cutaneous pigmentary anomaly, which seems to be a new entity. At the age of 26 years the elder sister developed an asymptomatic and persistent rash consisting of discrete, grouped, round to oval, guttate and nummular, hypopigmented macules, 0.2-5 cm in diameter.

Molecular heterogeneity of second and fourth components of complement and their genes in systemic sclerosis and association of HLA alleles A1, B8 and DR3 with limited and DR5 with diffuse systemic sclerosis.

Deficiency of the complement component C4 at the functional, protein and gene level and deficiency of complement component C2 at the functional level were investigated and HLA analysis was performed on patients with limited and diffuse systemic sclerosis (SSc). One of the patients with limited SSc (n = 15) had subnormal C4, 1 subnormal C2 and 1 subnormal C4 and C2 activities; the latter patient had HLA alleles A11;B35;Dw1 associated with type II C2 deficiency and therefore most likely had a defect at the C2 locus. One of the patients with diffuse SSC (n = 12) had subnormal C4 and 1 subnormal C4 and C2 activities.

Restrictive dermopathy. Report of 12 cases. Dutch Task Force on Genodermatology.

Background: This study describes 12 cases of restrictive dermopathy seen during a period of 8 years by the Dutch Task Force on Genodermatology. We present these unique consecutive cases to provide more insight into the clinical picture and pathogenesis of the disease.

Observations: Clinical features in more than 85% of these children were prematurity, fixed facial expression, micrognathia, mouth in O position, rigid and tense skin with erosions and denudations, and multiple joint contractures.

Melanosis naeviformis of Becker and scoliosis: a coincidence?

Melanosis naeviformis of Becker (MNB) can be associated with hypoplasia of soft tissue or extremities, spina bifida and scoliosis of the vertebral column. We have investigated 50 patients (42 men, 8 women) with MNB radiologically. Scoliosis was diagnosed in 13 patients (26%).

Leucocytoclastic vasculitis induced by prolonged exercise.

Many people develop skin symptoms after long-distance walks, but little is known about the aetiology of these. In this study we took 11 biopsies from 10 long-distance walkers who walked 80 km. All biopsies originated from purpuric lesions on the lower legs, which had appeared during walking.

Incomplete functional deficiencies of the fourth (C4) and second (C2) components of complement in a patient with linear frontoparietal scleroderma and his family. Deficiencies determined by a gene not linked to human leukocyte antigen system.

Background: In a previous study, a patient suffering from linear frontoparietal scleroderma and some of his family members were found to have an incomplete functional deficiency of the second component (C2) of complement (C). In this study, the proband and the rest of his family members were investigated for functional deficiencies of C2 and the fourth component of C (C4). A search for null alleles of C2 (C2*Q0) and C4 (C4*Q0) was made to find out whether their occurrence is responsible for incomplete functional deficiencies.

Mosaic expression of hypohidrotic ectodermal dysplasia in an isolated affected female child.

Background: Hypohidrotic ectodermal dysplasia (HED) is characterized by hypotrichosis, hypodontia, onychodysplasia and, as the most striking feature, hypohidrosis. The X-linked recessive form of HED, also known as Christ-Siemens-Touraine syndrome, is the most frequent and widely documented form. A clinically identical autosomal recessive form of HED has also been described.

[Bilateral linear temporoparietal scleroderma en coup de sabre].

Bilateral linear frontoparietal scleroderma en coup de sabre is an extremely rare form of localized linear scleroderma. In this paper the case of a 35-year-old woman with bilateral linear temporoparietal scleroderma en coup de sabre is presented. The manifestations that can be associated with linear scleroderma and the serologic abnormalities it involves are briefly discussed.

Successful treatment of pyoderma gangrenosum with topical 5-aminosalicylic acid.

Pyoderma gangrenosum in a twenty-nine-year-old woman with associated long-standing Crohn's disease was treated with topical 5-aminosalicylic acid cream. Significant improvement in the ulceration occurred despite progressive deterioration of the inflammatory bowel disease. The possible mode of action is reviewed.

Adhesion molecule expression in venous leg ulcers.

The final stage of chronic venous insufficiency (CVI) is ulceration. Several theories have been suggested to explain the skin changes and ulcerations. However, none of these can fully account for all the alterations which occur.

[Proteus syndrome. Expansion of the phenotype. Apropos of 3 pediatric cases].

In 1979 Cohen et Hayden and in 1983 Wiedemann et al. delineated a syndrome consisting of partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affections. Hitherto the literature pertaining to this syndrome consists of somewhat more than 100 cases of which some, that have been described previously or subsequently under other headings, were rediagnosed as being Proteus syndromes.

[Replacement of Kathon CG by Euxyl K 400 in cosmetics; from the frying pan into the fire?].

The recent negative publicity on the cosmetics preservative Kathon CG has made many cosmetic manufacturers look for safer alternatives. The most popular substitute appears to be Euxyl K 400, containing phenoxyethanol and methyldibromoglutaronitrile. Unfortunately, this preservative also induces allergic reactions to cosmetics and (at least in the Netherlands) to 'moist toilet paper'.

Evaluation of the frequency of contact allergic reactions to Kathon CG in the Maastricht area--1987-1990.

In the period 1987-1990, 999 patients suspected of a contact allergy were patch-tested with Kathon CG 100 ppm active ingredients aq. Of these patients, 84 (8.4%) showed a positive reaction (70% females).

Hereditary deficiency of C5 in association with discoid lupus erythematosus.

A 29-year-old woman with discoid lupus erythematosus had undetectable classic pathway complement activity. Hypocomplementemia was due to selective deficiency of C5. One of her children was also deficient.