Bolli Bjarnason

Childhood, neonatal, and stillborn pemphigus vulgaris

Background Childhood, neonatal, and stillborn cases of pemphigus vulgaris were reviewed.

Childhood Pemphigus Vulgaris Treated with Dapsone: A Case Report

Abstract: A 5‐year‐old boy developed hemorrhagic mucocutaneous blisters on various parts of the body leading to fetor, dysphagia, dysuria, anal pruritus, pain on defecation, and weight loss. The histopathology showed the classic features of pemphigus vulgaris, and direct immunofluorescence showed intercellular deposition of IgG and C3 in the epidermis. Circulating pemphigus antibodies were also detected. He was treated with a combination of systemic prednisone and dapsone which induced a rapid remission and controlled the disease well. He has been in remission for 1 year and 7 months with no immunosuppressive therapy except for the use of topical agents for the oral lesions. An adjuvant to corticosteroids has been used only once before in children with pemphigus vulgaris under the age of 12 years. This is the third and the youngest child in the literature treated in this fashion.

Assessment of budesonide patch tests.

Patch‐test technique for budesonide needs improvement. 20 subjects with positive or questionable patch‐test responses to budesonide were retested for 3 to 96 h (4 days [D]) with polyester patches coated with budesonide in serial doses (150 to 0.074 μ g/cm2). Multiple readings were taken visually and with a laser Doppler perfusion imaging technique up to 264 h (day [D]11). Additionally, all subjects were tested with 0.1% budesonide in petrolatum in Finn Chambers® for 48 h (2D) with readings taken at 72 (D3), 96 (D4) and 168 h (D7). Different dose levels and application times affected unpredictably highest assessments of reactions. No clear suppression of reactivity was observed at high doses. Time points of highest assessments of reactions varied between subjects but were generally the same for each subject with both reading methods regardless of dose levels or application times. Positive and negative subjects during the study were easily distinguished with all serial doses, regardless of assessment technique. At 2.0 μ g/cm2, the lowest dose level tested on all subjects, longer applications than 24 h (1D) were required to detect all positive subjects. 48‐h (2‐D) applications required 2 readings, optimally at 96 (D4) and 216 h (D9). The only test technique with Finn Chambers® used here did not make such distinction possible.

Objective non-invasive assessment of patch tests with the laser Doppler perfusion scanning technique

The laser Doppler perfusion scanning technique is an objective, non‐invasive assessment method that may be used to assess patch tests. The purpose of this paper is to focus on its clinical use in individuals with light skin, tested with non‐pigmented test materials. A laser Doppler perfusion imager® PIM 1.0 (LDPI) is used to study different set‐ups of the instrument that may affect readings. These set‐ups are studied through simulated light‐absorbing patch tests. The results have served as a base for clinical experiments comparing visual and LDPI assessments of normal skin, irritant patch‐test reactions, and more than 25,000 allergic patch‐test assessments of reactions of different intensities and control patches. This paper reviews the clinical experiments to suggest a set‐up of the LDPI for patch‐test readings. Such a set‐up makes it possible to compare intra‐ and inter‐individual test results and to obtain meaningful assessment values among users. Some subject‐related factors that may affect reading results are studied, and the effect of non‐subject related factors on readings are considered. Use of the technique is illustrated by images of perfusion and a perfusion profile of a patch‐test reaction followed over time.

Patch testing with neomycin sulfate

The recommended patch test concentration of neomycin sulfate is 20% in petrolatum applied occluded for 48 h (2 days [D]). In the current study, the efficiency of such a test is compared to results with other techniques using lower allergen dose than obtained by approximately 20 μl of the 20% substance. Efficiency of petrolatum and demineralized water as vehicles are compared. 16 neomycin‐sulfate positive subjects were retested with serial doses ranging from 0.4 to 0.0085 mg/cm2 neomycin sulfate in cellulose printed on polyester squares and applied by both tapes and plastic foils. Additionally, tests were performed with the 20% petrolatum substance in Finn Chambers®. Tests containing the 20% substance in petrolatum and in water were applied directly with transparent foils. Readings ranged from 3 to 264 h (11D) following applications. Results show that foil applications of a polyester square dose, that is 7% of the dose obtained with the 20% substance, distinguished between perfusion of subjects who were visually positive and not with any test in the study. With the same test technique and visual assessments, 2 subjects were false‐negative but developed doubtful test responses with the Finn Chambers®.

Influence of patch-test application tape on reactions to sodium dodecyl sulfate

There remains much room for improvement in the accuracy of the patch test procedure. There has been a lack of knowledge regarding the possible relationship between the intensity of test reactions and the quality of the tape used to apply patch tests. Using different brands of tape, patches coated with 2 mg/cm2 sodium dodecyl sulfate (SLS) were applied for 24 h to the backs of 10 volunteers. The tape specimens varied in terms of manufacture, elasticity, adhesive strength, and water permeability. The intensity of patch test reactions, evaluated visually and objectively with both the high resolution laser Doppler perfusion imaging technique (HR‐LDPI) and transepidermal water loss measurements (TEWL), varied significantly with the different brands of tape. Observed variations in the intensity of reactions to the patch tests could not be explained by any of the 4 tape parameters investigated. In order to attain optimal test quality in the future, both the tape and patches used in the testing system should be standardized and clearly labelled.

A Non-Epidermolytic Epidermal Naevus of a Soft, Papillomatous Type with Transitional Cell Cancer of the Bladder: A Case Report and a Review of Non-cutaneous Cancers Associated with the Epidermal Naevi

Sir, We describe here the case of a man aged 65-years at our first examination with non-epidermolytic epidermal naevus of a soft, papillomatous type covering large areas of the left side of his body and scalp. He reported bilateral hearing loss and earlier frequent mastoiditis. He was of short stature and had had dyspnoea since childhood affecting his sporting activities, reduced touch sensation on the soles of his feet and reduced vibration sensitivity of his toes. His upper teeth were extracted at a young age. He had had amblyopia of the left eye since childhood and an attack of paralysis of that eye. At the age of 22 years, an asymptomatic gross haematuria was discovered caused by a large bladder papilloma at the opening of the left ureter. Lost to follow-up, he presented 2 years later with intermittent gross haematuria and a cancer at the same site. The cancer was confirmed by our review of the pathological report to be of a transitional cell type. The patient had no familial history of epidermal naevi. The term “epidermal naevus syndrome” has, in the past, been used to refer to the association between epidermal naevi and abnormalities in other organ systems (1). Several distinct birth defects have been lumped together under this designation (2). All epidermal naevus syndromes are mosaic phenotypes (2). Because of the understanding of the concepts of genetic mosaicism, that there are potentially many different epidermal naevus syndromes, or syndromes of which an epidermal naevus is a cutaneous feature, it has been argued that the term “epidermal naevus syndrome” to describe a disease entity should be abandoned (3, 4). Thus far, at least 7 different epidermal naevus syndromes have been identified; viz., naevus sebaceous syndrome, Proteus syndrome, CHILD syndrome, naevus comedonicus syndrome, Becker naevus syndrome, phakomatosis pigmentokeratotica (5, 6); and the last one was first described by Schauder et al. (7) and later termed “angora hair naevus syndrome” (1). A Non-Epidermolytic Epidermal Naevus of a Soft, Papillomatous Type with Transitional Cell Cancer of the Bladder: A Case Report and a Review of Non-cutaneous Cancers Associated with the Epidermal Naevi

Assessment of balsam of Peru patch tests

To find an ideal test technique for as low a dose of balsam of Peru (Myroxylon Pereirae) as possible, subjects testing positive to balsam of Peru are re‐tested with a 25% concentration of balsam of Peru in petrolatum. Applications are with Finn Chambers® for 6 different application times, and directly by foils for 96 h (4 days (D)). The goals are to confirm which subjects are positive and which are not, and, using that information, to see if it is possible to distinguish between these 2 groups, tested concomitantly at much lower serial dose levels, in terms of perfusion or by visual assessments. 5 different serial doses are applied with strips for 3–96 h (4D) and with foils for 96 h (4D). The Finn Chamber® tests allow a distinction between visually positive and negative subjects supported by perfusion assessments. With the foils, a 24× lower serial dose level than with the 25% test substance is sufficient to distinguish between positive and negative subjects in terms of perfusion values. This approach requires readings up to 9 days. With this test, the visual approach yields only 3 of 10 positive subjects. This study demonstrates that a lower test dose is possible with perfusion assessments compared to visual ones.

Effect of test techniques on perfusion of neomycin sulfate patch tests : A comparative study with visual assessments

Effects of test techniques on neomycin patch test results have not been thoroughly investigated. This study focuses on effects of dose and application time of neomycin sulfate patch tests on test results. The effects are assessed both visually and by perfusion. 16 subjects positive to neomycin are retested with neomycin sulfate in various doses and vehicles applied with different application devices for variable time intervals. All subjects were tested with a serial‐dose series for time intervals ranging from 3 to 96 h (4 days [D]). The results show very good agreement between perfusion assessments and positive and negative visual assessments of reactions; however, a gray zone was detected where reactions with variable perfusion were associated with visually questionable test responses. 1:27 serial dilutions and wide application time intervals show a positive response with perfusion assessments and partly with visual assessments. Regardless of reading technique, highest reactivity of reactions was detected at either 96 h (day [D] 4) or 168 h (D7) in each subject with minor exceptions, regardless of dose, vehicle, application device or application time. Some findings of the study support the idea that reactions with papules alone are positive tests. Possible effect of tapes and foils on test results are discussed.

Effect of dark test‐substance pigmentation on skin perfusion assessments and effect of test technique on balsam of Peru patch‐test results

13 balsam of Peru (Myroxylon Pereirae) patch‐test‐positive subjects are re‐tested with 25% balsam of Peru in petrolatum and with serial doses printed on polyester squares. All substances are applied with tape strips for 3, 6, 24 (1 day [D]), 48 (2D), 72 (3D) and 96 h (4D) on each subject and for 96 h (4D) with plastic foils. Tests are followed visually and with perfusion assessments from 3 h to 9 days. Results show that pigment remnants following detachment of patches affect perfusion assessments. Such effect due to pigment is supported by readings of patch tests through the petrolatum test substance while applied with transparent foils. For most reactions, good agreement is observed between the assessment techniques when peak assessment values of reactions are compared. There is inter‐individual variation in perfusion with identical tests. With the petrolatum test substance, increased visible reactivity was observed when the application time was extended up to 24 h (1D), while extension of application time increased perfusion in most cases except for an extension from 24 (2D) to 48 h (4D) where decreased perfusion resulted in most cases. Dose and application time did not affect the timing of highest reactivity of reactions in most cases.