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Notes: Background Borrelia burgdorferi can be isolated from the skin of patients with acrodermatitis chronica atrophicans (ACA), a late-stage manifestation of Lyme borreliosis; despite a marked T-cell infiltrate in lesional skin and high antibody titres in patients’ sera. Objectives To determine whether antigen-presenting Langerhans cells (LCs), which reportedly show signs of injury in erythema chronicum migrans (ECM), the early stage of disease, are altered in ACA. Patients/Methods We studied the immunophenotype of cutaneous leucocytes on cryostat sections of lesional skin from both ECM and ACA patients. Results The total number of CD1a+ cells evaluated by semiautomatic image analysis was lower in ECM (594 ± 263 cells mm−2 epidermis) than in ACA (835 ± 317 cells mm−2 epidermis). HLA-DR expression was remarkably downregulated on CD1a+ LCs to 29% in ECM and 18% in ACA, whereas in normal skin, most of the epidermal CD1a+ dendritic cells were HLA-DR+. The inflammatory infiltrate was mainly composed of CD68+ macrophages and CD45RO+ memory T cells, with a predominance of CD4+ helper T cells. Conclusions It is conceivable that the downregulation of major histocompatibility complex class II molecules on LC in both the early and late skin manifestations of Lyme borreliosis is indicative of a poorly effective anti-B. burgdorferi immune response and thus at least partly responsible for the insufficient elimination of this micro-organism from ACA skin.

Notes: A 43-year-old white man presented with a generalized eruption of lichen planus and tense blisters within the lichenoid lesions and also on clinically normal skin. Direct immunofluorescence (IF) studies revealed immunological and histopathological characteristics of lichen planus in the lichenoid lesions and of bullous pemphigoid in the bullous lesions, and indirect IF studies showed that the patient had circulating antibasement membrane antibodies. The coexistence of both disorders may indicate a possible link between the pathology in the junctional zone in lichen planus and the appearance of antibasement membrane zone antibodies and bullous lesions, respectively.

Notes: Summary Management of the increasing frequency of aciclovir-resistant herpes simplex virus (HSV) infections among immunocompromised human immunodeficiency virus-infected people demands additional treatment options. We report the case of a 38-year-old patient with acquired immune deficiency syndrome who suffered from a perianal butterfly ulcer, which was HSV-2 positive by polymerase chain reaction (PCR) analysis. The ulcer appeared during treatment of a cytomegalovirus (CMV) pneumonitis with ganciclovir. Despite additional valaciclovir therapy the lesion gradually progressed in size. Investigations including histology, PCR analysis and in situ hybridization of a biopsy from the growing ulcer margin confirmed the presence of HSV-2 infection. Importantly, HSV isolates from this specimen were resistant to aciclovir. Based on a report about the successful treatment of aciclovir-resistant HSV infection with cidofovir, our patient received this drug intravenously at a dose of 5 mg kg−1 body weight once weekly for a total of 3 weeks. Concomitant oral probenecid and prehydration were administered to minimize nephrotoxicity. Within 30 days of treatment the ulcer had almost (〉 95%) completely healed. We conclude that cidofovir is a potent antiviral drug with a potential usefulness in the treatment of aciclovir-resistant HSV-2 infection. It deserves further investigation in clinical trials.

Notes: Background After exposure, 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) is excreted via the faeces, breast milk and epidermal lipids. Objectives To determine to what extent TCDD is eliminated via the skin and to evaluate whether cutaneous elimination can be accelerated by the application of petrolatum. Methods In two patients severely intoxicated with TCDD, material obtained from the skin surface and, in one patient, cerumen and the content of epithelial cysts, was analysed for TCDD. Results The TCDD concentration in the initial blood sample taken was 144 000 pg g−1 blood fat in patient 1, and 26 000 pg g−1 blood fat in patient 2. Six months later, when the skin tests were performed, the blood TCDD levels had decreased to 80 900 and 16 100 pg g−1 blood fat, respectively. In the two samples of pooled cyst contents from patient 1, TCDD levels of 34 400 and 18 600 pg g−1 fat were found. A cerumen sample contained TCDD at 20 500 pg g−1 fat. In the material collected from the skin surface we observed a linear increase of the amount of TCDD measured per test field with time, indicating a continuous elimination of TCDD via the skin. The daily amount of TCDD eliminated via the skin was 1·51 pg cm−2 in patient 1 and 0·57 pg cm−2 in patient 2. Application of petrolatum led to a twofold increase in the amount of TCDD measured in patient 1, but had no significant effect in patient 2. Conclusions In our patients, elimination of TCDD via the skin, most probably through desquamating scales, represented 1–2% of the overall daily TCDD elimination rate, with regard to the body surface and when calculated on the basis of the half-life of TCDD at the time of the skin test. If a more typical overall elimination half-life of 7 years is used as the basis for the calculation, the skin would account for 9%(patient 1) and 15%(patient 2) of the overall elimination. Although we observed an increase in TCDD in material derived from the skin surface of up to 100% after application of petrolatum in patient 1, such an approach appears not to be a feasible means to increase elimination. Owing to the small amount of TCDD measured in skin-surface material, as well as in the cyst contents and cerumen obtained from one patient, contamination of the environment and other persons appears highly unlikely.

Notes: Severe pustular psoriasis von Zumbusch type is a therapeutic challenge not only in adults, but even more in children. We report a 3½-year-old boy who developed a generalized flare of diffusely scattered pustules on erythematous skin which rapidly progressed to large exuding areas. The clinical presentation and investigations including histopathological examination of a biopsy and negative bacterial cultures were consistent with the diagnosis of pustular psoriasis von Zumbusch type. Upon initial treatment with methylprednisolone, acitretin and antibiotics the extent of the disease declined. However, several attempts to reduce the dose of the oral corticosteroid were followed by immediate severe flares. Additional treatment with narrowband ultraviolet B (NB-UVB, 311–313 nm UVB) resulted in a rapid arrest of disease activity and allowed the corticosteroid to be tapered off. After 10 irradiations the patient was both off steroid and disease free. NB-UVB therapy was subsequently reduced to twice-weekly exposures and acitretin gradually diminished to a maintenance dose of 0·3 mg kg−1 daily. We conclude that NB-UVB in conjunction with acitretin is a potent therapeutic regimen for the treatment of severe pustular psoriasis von Zumbusch type in childhood.