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Biomedical subjects

R J Koopman

Publications and source records attributed to R J Koopman.

At least 19 recordsLinked to original sources
PubMed1997

Becker nevus syndrome.

The new term Becker nevus syndrome is proposed for a phenotype characterized by the presence of a particular type of organoid epithelial nevus showing hyperpigmentation, increased hairiness and hamartomatous augmentation of smooth muscle fibers, and other developmental defects such as ipsilateral hypoplasia of breast and skeletal anomalies including scoliosis, spina bifida occulta, or ipsilateral hypoplasia of a limb. The present review includes 23 cases that can be categorized under this designation. The Becker nevus syndrome usually occurs sporadically. The associated anomalies tend to show a definite regional correspondence, suggesting a common origin from an early postzygotic mutation.

Adolescent↗
PubMed1997

[D-penicillamine in treatment of scleroderma "en coup de sabre"].

A ten year old boy with linear erythema above the right eyebrow and a groove in the underlying skull bone is described. Histological examination of a biopsy revealed a spotty, periadnexal, perivascular, lymphocytic infiltrate. The papillary dermis was almost absent and the collagen fibres were slightly thickened. Based on the clinical appearance and the histology a diagnosis of linear scleroderma (en coup de sabre) was made. As the signs and symptoms were progressive, treatment with D-penicillamine was started. Subsequently the erythema disappeared. The shallow groove in the skull bone remained palpable. After thirteen months, treatment was stopped. The patient is currently free of signs and symptoms after a follow-up period of three and a half years. We feel that the strong clinical improvement is due to treatment with D-penicillamine rather than to the natural course of the disease.

Adolescent↗
PubMed1995

[Erythema ab igne].

A 47-year-old woman suffering from chronic abdominal pain developed erythema ab igne (reticular hyperpigmentation of the skin caused by exposure to heat). Histological examination revealed premalignant changes in two specimens of affected skin. The incidence of erythema ab igne is declining since fewer people use open fires for heating. Nowadays it is found mostly in patients suffering from chronic pain using hot water bottles, infrared lamps or heating pads. The disease is an important clinical indication for underlying chronic diseases. The affected skin runs a higher risk of development of actinic keratoses. Bowen's disease and squamous cell carcinomas. Dysplastic features of keratinocytes can be found histologically in lesions which are clinically not under suspicion.

Biopsy↗
PubMed1994

Juvenile xanthogranuloma and acute leukemia: a case report.

Juvenile xanthogranuloma (JXG) is a rare benign disease of the skin. It is seen in combination with juvenile chronic myelo-monocytic leukemia (JCML) and/or neurofibromatosis type 1 (NF1). The association with acute lymphoblastic leukemia is hardly mentioned in the literature. A case report of this rare combination is described and a review of the literature is given.

Child, Preschool↗
PubMed1994

Cellulitis as first clinical presentation of disseminated cryptococcosis in renal transplant recipients.

Two renal transplant recipients with cellulitis due to Cryptococcus neoformans are described. The patients were treated empirically for a presumed bacterial erysipelas, but without response. Examination of skin biopsies revealed C. neoformans as the causative organism. In both patients the cellulitis was the presenting clinical manifestation of disseminated cryptococcosis. Therapy with antifungal agents was successful. Disseminated cryptococcal disease occurs mainly in immunocompromised patients. When left untreated, it nearly always has a fatal course. Early diagnosis and appropriate therapy are therefore essential.

Adult↗
PubMed1993

MIDAS syndrome (microphthalmia, dermal aplasia, and sclerocornea): an X-linked phenotype distinct from Goltz syndrome.

Bilateral microphthalmia with blepharophimosis, linear lesions of dermal aplasia involving the face, and microcephaly were present in a newborn girl who died at age 9 months from cardiomyopathy resulting in ventricular fibrillation. Autopsy showed an atrial septum defect, persistent gross trabeculation of the left ventricle, and an arteria lusoria. This case represents a further example of a new entity for which we propose the term MIDAS syndrome. The acronym stands for microphthalmia, dermal aplasia, and sclerocornea. Our patient is the second with this syndrome to have a major congenital heart defect. Cytogenetic studies reported in previous cases indicate that the underlying gene defect can be assigned to Xp22.3. This new X-linked male-lethal trait should be distinguished from focal dermal hypoplasia that will be found to map elsewhere on the X-chromosome.

Abnormalities, Multiple↗
PubMed1993

Rapid diagnosis of acute meningococcal infections by needle aspiration or biopsy of skin lesions.

OBJECTIVES: To evaluate the usefulness of Gram staining and culture of skin lesions in patients with acute meningococcal infections. DESIGN: Retrospective study. SETTING: Community hospital and intensive care unit of a teaching hospital. SUBJECTS: 51 patients admitted from 1989 to 1993 with proved meningococcal infections and microbiological examination of specimens from skin lesions. INTERVENTIONS: Needle aspiration of a skin lesion before start of antibiotic treatment in 26 patients in the community hospital; punch biopsy of skin lesion after start of antibiotic treatment in 25 patients in the teaching hospital. MAIN OUTCOME MEASURES: Detection of meningococci by Gram staining of specimens from skin lesions according to category of infection (meningococcaemia, meningitis, meningitis with shock, or septic shock without meningitis). RESULTS: Bacteria were detected in the specimen from haemorrhagic skin lesions by culture or Gram staining, or both in 32 (63%) patients. The sensitivity of the Gram stain was 51% and did not differ significantly from its sensitivity in detecting bacteria in cerebrospinal fluid. In meningococcal sepsis, however, a Gram stained skin lesion was significantly more sensitive (72%) than Gram stained cerebrospinal fluid (22%). In patients with meningitis skin lesions gave positive results on staining more often if shock was present. The results for punch biopsy specimens were not affected by antibiotics as Gram staining gave positive results up to 45 hours after the start of treatment and culture gave positive results up to 13 hours. CONCLUSION: Microbiological examination of skin lesions is informative, especially in patients with sepsis and inconclusive results from cerebrospinal fluid, and may provide a diagnosis in such patients within 45 minutes. It differentiates well between meningitis with and without haemodynamic complications, and the result is not affected by previous antibiotic treatment.

Acute Disease↗
PubMed1993

Sarcoidosis.

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Adult↗
PubMed1993

Calciphylaxis.

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Calciphylaxis↗
PubMed1992

[Subfascial lipoma of the forehead].

Three patients with subfascial lipoma of the forehead are presented. These deeply placed lipomas are seen in up to fifty percent of the lipomas of the scalp. A characteristic feature is the intact skin line pattern over the tumour. Surgical treatment involves incision of the aponeurosis of the M. occipitofrontalis.

Adult↗
PubMed1992

Erysipelas-like skin lesions associated with Campylobacter jejuni septicemia in patients with hypogammaglobulinemia.

Three cases are reported of hypogammaglobulinemic males with recurrent Campylobacter jejuni septicemia and erysipelas-like cellulitis without diarrhoea. In one patient Campylobacter jejuni grew from skin biopsy specimens. The findings in another patient were strongly suggestive of osteomyelitis caused by Campylobacter jejuni. Since the susceptibility of hypogammaglobulinemic patients to infection with Campylobacter jejuni is probably related to a lack of serum bactericidal activity against Campylobacter jejuni due to lack of IgM, two patients in whom previous antimicrobial treatment failed were treated with plasma infusions. This regimen supplemented with imipenem resulted in cure of these relapsing infections. Campylobacter jejuni septicemia must be considered in hypogammaglobulinemic patients who present with periodic fever and cellulitis.

Adult↗