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Notes: Viral infections are thought to play a part in some cutaneous drug reactions. Human herpesvirus 6 (HHV6), which is the agent of exanthema subitum (sixth disease), has never been implicated in a drug reaction. We report a patient with severe phenobarbital-induced anticonvulsant hypersensitivity syndrome in whom a fulminant haemophagocytic syndrome was associated with HHV6 infection. We discuss the possible role of HHV6 in this reactive condition.

Notes: Summary There is a known relationship between the use of immunosuppressive therapies and the development of lymphoproliferative malignancies. These lymphomas are mainly B-cell nonHodgkin's lymphomas associated with Epstein–Barr virus. Most cases concern classical immunosuppressive treatments including ciclosporin and methotrexate. A relationship between the new antitumour necrosis factor (TNF)-α agents and lymphoproliferative malignancies is debated. Patients with psoriasis on immunosuppressive therapies, mainly ciclosporin, are considered to have a low risk of developing lymphoid proliferation. We report a patient with erythrodermic psoriasis treated with ciclosporin and infliximab who developed a CD30+ T-cell lymphoma. This lymphoma regressed after stopping these treatments. In this case, the anti-TNF-α agent may have played a role in association with ciclosporin in the development of the lymphoproliferative disorder. Whereas the combination of anti-TNF-α therapies with methotrexate has been well studied, their combination with ciclosporin has been evaluated only in a few patients. Psoriatic patients who may require anti-TNF-α treatment have often been or will be treated with ciclosporin. The combination of ciclosporin and anti-TNF-α warrants further investigation.

Notes: The concurrence of systemic scleroderma and porphyria cutanea tarda (PCT) seems very rare. Only six cases have been reported to our knowledge. On the other hand, cutaneous sclerodermatous lesions in PCT occur more frequently and can affect 1·8-18% of cases.1,2 These observations raise the problem of a possible physiopathological relation between scleroderma and PCT. In fact, Grossman and colleagues3 have found anti-nuclear antibodies (ANA) in 38% of patients so tested. In the present report, the association of systemic scleroderma and PCT with Sjögren's syndrome and anti-RNP antibodies raises further questions concerning the relationship between PCT and auto-immune diseases.

Notes: We report a new type of paraneoplastic mixed bullous skin disease in a patient with a B-cell lymphoma associated with monoclonal IgM kappa paraproteinaemia. The patient’s clinical and histological features were reminiscent of bullous pemphigoid. Characterization of antiepidermal antibodies by immunoblotting and indirect immunoelectron microscopy demonstrated a novel pattern of reactivity: IgG antibodies were directed against desmoplakins I–II and BPAG2, and were associated with antidesmoglein 3 polyclonal IgM antibodies. Such an autoreactive pattern involving antidesmoplakins, anti-BPAG2 and antidesmoglein 3 antibodies has not been previously reported. It reflects the breakdown of tolerance to multiple epidermal antigens observed in some patients with malignancy.

Notes: Background  Psoriasis is a T-cell-mediated immunological disease characterized by epidermal proliferation. The nature of the antigen(s) responsible for T-cell activation is still unknown. It has been suggested that the human papillomaviruses (HPVs) associated with epidermodysplasia verruciformis (EV), including the oncogenic HPV5, may contribute to the pathogenesis of psoriasis.Objectives  To determine whether EV-HPVs may play a role early in the disease, we searched for these viruses in children with psoriasis. The influence of clinical data on EV-HPV infection was investigated.Methods  We studied scrapings of involved skin from 26 children aged 1·5–13 years with psoriasis. As controls, we analysed scrapings from 28 adults with psoriasis and 15 children with atopic dermatitis, as well as scrapings from normal skin of 28 adults with no known history of HPV infection. We searched for EV-HPV DNA sequences with a nested polymerase chain reaction method using degenerate primers specific for EV-HPVs and primers specific for HPV5 and HPV36, two EV-HPVs frequently detected in adults with psoriasis.Results  Similar high prevalences were observed in children and adults with psoriasis for EV-HPVs (38·5% vs. 35·7%), HPV5 (46·2% vs. 46·4%) and HPV36 (15·4% vs. 25·0%). As in adults, we found several EV-HPV genotypes and HPV5 and HPV36 variants. A novel HPV36 subtype, HPV36b, was identified. Lower prevalences were observed in children with atopic dermatitis and in adults from the general population (6·7–10·1%). No correlation was observed between frequency of detection of HPVs and clinical data. It is noteworthy that HPV5 was identified in an 18-month-old girl and in a boy with psoriasis developing for only 1 week.Conclusions  The early detection of several EV-HPV genotypes in children further supports the link between psoriasis and EV-HPVs and suggests a putative role for these viruses in the pathogenesis of psoriasis.

Notes: Background  Few prospective studies are available on the incidence and analysis of the characteristics of adverse cutaneous drug reactions in hospital settings.Objectives  A 6-month prospective study was managed in our hospital among hospitalized patients to: (i) evaluate the incidence of cutaneous allergic reactions from systemic drugs; (ii) study characteristics of patients with cutaneous drug reactions; (iii) describe the adverse cutaneous reactions; and (iv) evaluate drug reaction imputability and preventability.Methods  All suspected allergic cutaneous reactions to systemic drugs were collected during a 6-month period (November 2000 to May 2001). Exhaustivity of recording was ensured by regular dissemination of information about this study to the practitioners; a simple method for inclusion by fax was established. Inclusion criteria were suspected cutaneous allergic reactions induced by systemic drugs responsible for hospitalization or developed during hospitalization. A physical examination was done by a dermatologist who completed a standardized questionnaire. Requested information included patient characteristics (associated disorders, severity score), drug intake (list and chronology of the drug intake during the 3 weeks preceding the adverse reaction) and characteristics of the skin reaction (type, course). A group comprising dermatologists and pharmacologists evaluated the drug imputability and preventability.Results  Forty-eight cases were collected. A prevalence of 3·6/1000 among hospitalized patients was estimated. The prevalence rate was higher in patients hospitalized in medical departments (0·5%) than in surgical departments (0·01%) (P 〈 0·001). The cases were mostly recruited in departments of infectious diseases and dermatology. The most frequent associated disorders were: human immunodeficiency virus (HIV) infection (19%), connective tissue disease (10%) and viral or autoimmune hepatitis (12%). Of these patients, 31% had had a previous immunological drug reaction. Adverse cutaneous drug reactions were principally exanthematous (56%). Reactions were considered severe in 34% of cases because they were responsible for hospitalization (18%), increased the duration of hospitalization (14%) or were life threatening (2%). Principal imputable drugs were antibiotics, mainly penicillins. An imputability score was likely in 56% of cases, but it was only possible to conclude definitively in 44% of patients. In 15% of the cases, the side-effect was considered to be preventable.Conclusions  This study finds a lower incidence than other studies that reported an incidence of 2% of cutaneous drug reactions in hospitalized patients, but only allergic adverse cutaneous reactions induced by systemic drugs were collected in this study. The previous studies were principally done in selected patients hospitalized only in general internal medicine or medical divisions. Our results confirm some data already known about skin drug reactions: HIV infection as a risk factor (P 〈 0·0001), high prevalence of adverse cutaneous reactions due to antibiotics, and difficulty in ascertaining the imputability of a drug. A high proportion (34%) of these reactions was severe and 15% were avoidable; these two facts justify the development of an intensive programme of clinical pharmacology.

Notes: Background  Kaposi's sarcoma (KS) is a potentially life-threatening multifocal neoplasm. Despite the significant decline in the incidence of acquired immune deficiency syndrome (AIDS)-related KS with the use of highly active antiretroviral therapy (HAART), some patients, even those with a good immune restoration, still have aggressive disease. Liposomal anthracyclines or combination chemotherapy are widely used but adverse effects limit their utilization.Objectives  We studied the efficacy and tolerance of docetaxel in the treatment of AIDS-related KS after pretreatment with anthracycline.Patients/methods and main outcome measure  A retrospective cohort study was done. Nine human immunodeficiency virus (HIV)-infected patients were treated from 1997 to 2002 with docetaxel. Tumour response was evaluated using the AIDS Clinical Trial Group (ACTG) staging criteria. Clinical and biological toxicity was evaluated. AIDS status with HIV viral load and CD4 T-cell count were measured at the beginning and at the end of the treatment.Results  A major (complete or partial) response and a stabilization of the disease were demonstrated in seven and two patients, respectively. Grade 4 neutropenia and thrombocytopenia were observed in four of nine and one of nine patients, respectively. One patient died after sepsis.Conclusions  Docetaxel has a good and rapid efficacy in anthracycline-pretreated patients with severe AIDS-related KS. Phase II/III trials should be done to compare docetaxel with liposomal anthracyclines as a first-line treatment.

Notes: Background  Atopic dermatitis (AD) is a chronic or chronically relapsing inflammatory skin condition that can have a considerable impact on those affected. There are a number of instruments available to measure outcome in dermatological conditions but none have been developed specifically for AD. In addition, most measure symptoms and/or daily functioning, which are potential influences on quality of life (QoL) rather than assessments of the construct itself.Objectives  The aim of the current study was to develop a new instrument specifically designed to measure QoL in adults with AD—the Quality of Life Index for Atopic Dermatitis (QoLIAD).Methods  The instrument was developed based on the needs-based model of QoL and was produced in several different countries simultaneously. Its content was derived from 65 in-depth interviews with relevant patients in the U.K., Italy and the Netherlands. The initial version of the measure was produced in U.K. English and translations were produced for the Netherlands, Italy, Germany, France and the U.S.A. using a dual translation panel methodology. A Spanish version was developed using the same adaptation process after the instrument was finalized. Field-test interviews were conducted with approximately 20 patients in each country to assess face and content validity. The instrument [in addition to the Dermatology Life Quality Index (DLQI) and the Psychological General Well-Being Schedule (PGWB)] was then administered to up to 300 AD patients in each country at two time points to finalize the instrument and test its psychometric properties.Results  The initial version of the QoLIAD had 56 items that reflected the areas of need fulfilment identified in the qualitative interviews as having been affected by AD: mental and emotional stimulation, physical and emotional stability, security, sharing and belonging, self-esteem, personal development and fulfilment. Comments from patients in field-test interviews resulted in the removal of 14 items, to leave a 42-item instrument that was considered relevant and acceptable. The number of patients participating in the survey were 286 in the U.K., 46 in the Netherlands, 213 in France, 187 in Germany, 178 in the U.S.A. and 83 in Spain. Application of the Rasch model to these data identified the final 25-item QoLIAD. Unidimensionality was confirmed, with deviation of the total scale from the Rasch model evident at a single time point in one country only (the U.K.). All language versions, with the exception of the Dutch measure, had test–retest reliability coefficients in excess of 0·85. The test–retest in the Netherlands was 0·80. However, this country had the smallest sample size and the corresponding reliability for the DLQI was only 0·40. The QoLIAD had adequate internal consistency and the initial indications of construct validity were good. The levels of association with the DLQI indicated that the two instruments measure related but distinct constructs.Conclusions  The QoLIAD is a practical, reliable, valid and culturally applicable instrument for measuring the impact of AD and its treatment on QoL in clinical trials or in routine clinical practice.