DLQI-R scoring improves the discriminatory power of the Dermatology Life Quality Index in patients with psoriasis, pemphigus and morphea.

BACKGROUND: The Dermatology Life Quality Index (DLQI) rates 'not relevant' responses (NRRs) as the item on the questionnaire having no impact on the patients' lives at all. The DLQI-Relevant (DLQI-R) is a recently developed scoring that adjusts the total score of the questionnaire for the number of NRRs indicated by a patient. OBJECTIVES: To compare the discriminatory power of the original and DLQI-R scoring approaches in terms of absolute and relative informativity. METHODS: Cross-sectional data from 637 patients with morphea, pemphigus and psoriasis were used for the analyses. To assess absolute and relative informativity, Shannon's index and Shannon's evenness index were calculated for the 10 items on the questionnaire and for DLQI and DLQI-R total scores. RESULTS: Mean DLQI and DLQI-R scores of patients were 6·13 vs. 6·91. In the subset of patients with NRRs (n = 261, 41%), absolute informativity was higher with the DLQI-R scoring for all eight items with NRR options in all three conditions. The DLQI-R exhibited a better relative informativity in 8, 8 and 6 items in pemphigus, morphea and psoriasis, respectively. The DLQI-R led to an improvement in average item-level informativity in all DLQI score bands up to 20 points. Regarding total scores, the DLQI-R produced both a higher absolute and relative informativity in all three conditions. CONCLUSIONS: In patients with morphea, pemphigus and psoriasis, DLQI-R scoring improves the discriminatory power of the questionnaire by benefiting from the additional information in NRRs. DLQI-R scoring may be useful both in clinical practice and research. A scoring chart has been developed to aid physicians with scoring. What's already known about this topic? The original scoring of the Dermatology Life Quality Index (DLQI) rates 'not relevant' responses as the item of the questionnaire having no impact on the patients' lives at all. DLQI-Relevant (DLQI-R) is a new scoring developed in 2018 that adjusts the total score of the questionnaire for the number of 'not relevant' responses indicated by patients. The discriminatory power of the DLQI-R compared with the DLQI has not yet been investigated. What does this study add? In patients with psoriasis, pemphigus and morphea, DLQI-R scoring improves the discriminatory power of the questionnaire by benefiting from the additional information in 'not relevant' responses. What are the clinical implications of this work? DLQI-R scoring may help to more accurately quantify patients' health-related quality of life both in clinical practice and research. A scoring chart has been developed to aid physicians with scoring.

Association between quality of life and clinical characteristics in patients with morphea.

BACKGROUND: Morphea can lead to considerable cosmetic or functional impairment; nevertheless, health-related quality of life (HRQoL) is rarely documented in adult morphea patients. OBJECTIVE: To investigate the impact of morphea on HRQoL and to identify determinants of impaired HRQoL. METHODS: A cross-sectional study has been carried out among adult morphea patients. HRQoL was evaluated by the Dermatology Life Quality Index (DLQI). The modified Localised Scleroderma Skin Severity Index (mLoSSI) and the Localised Scleroderma Damage Index (LoSDI) were applied to evaluate disease activity and damage, respectively. Physician Global Assessment of Activity and Damage (PGA-A, PGA-D) were also completed. Determinants of HRQoL were analysed by multiple regression. RESULTS: A total of 101 patients (84% females) entered the study, with a mean age of 56.8 ± 14.8 years. Median mLoSSI, LoSDI, PGA-A and PGA-D scores were 8, 5, 9 and 9 points, respectively. Patients with generalised localised (51%) and plaque-type morphea (45%) had median total DLQI scores of 4 and 1, respectively. Embarrassment (53%), itchy or painful skin (46%), and clothing issues (43%) were the most commonly reported problems in the DLQI. Female gender, generalised morphea, higher disease activity (PGA-A score) and involvement of hands and/or feet were significant predictors of impaired HRQoL (p < 0.05). CONCLUSION: This study represents the largest sample of adult morphea patients surveyed about their HRQoL in Europe. The frequent occurrence of embarrassment warrants an increased attention to improve patients' mental health. Care must be taken in case of involvement of functionally sensitive areas, as these cases might require more intensive treatment.

A detailed analysis of 'not relevant' responses on the DLQI in psoriasis: potential biases in treatment decisions.

BACKGROUND: Dermatology Life Quality Index (DLQI) is the most common health-related quality of life measure in dermatology that is widely used in treatment guidelines for psoriasis. Eight of the 10 questions of the DLQI offer a 'not relevant' response (NRR) option that is scored as the item had no impact on patients' life at all. OBJECTIVE: To explore the occurrence of NRRs on the DLQI in psoriasis patients and to examine the effect of several socio-demographic and clinical factors on giving NRRs. METHODS: Data were obtained from two cross-sectional surveys among psoriasis patients at two academic dermatology clinics in Hungary. Health-related quality of life was measured by employing DLQI and EQ-5D-3L, while disease severity was graded by Psoriasis Area and Severity Index (PASI). Multivariate logistic regression was applied to determine the predictors of providing NRRs. RESULTS: Mean age of the 428 patients was 49 years, and 65% were males. Mean PASI, DLQI and EQ-5D-3L index scores were 8.4 ± 9.5, 6.8 ± 7.4 and 0.74 ± 0.28, respectively. Overall, 38.8% of the patients had at least one NRR: 19.6% (one), 11.5% (two), 5.1% (three) and 2.6% (more than three). Most NRRs occurred in sport, sexual difficulties and working/studying items of the DLQI (28.4%, 16.4% and 14.0%, respectively). Female gender (OR 1.65; 95% CI 1.04-2.61), older age (OR 1.05; 95% CI 1.03-1.07) and higher PASI score (OR 1.03; 95% CI 1.01-1.06) were associated with providing more NRRs, whereas highly educated patients (OR 0.34; 95% CI 0.16-0.72) and those with a full-time job (OR 0.47; 95% CI 0.29-0.77) less frequently tended to tick NRRs. CONCLUSION: The high rate of psoriasis patients with NRRs, especially among women, less educated and elderly patients, indicates a content validity problem of the measure. A reconsideration of the use of the DLQI for medical and financial decision-making in psoriasis patients is suggested.

Folliculotropic mycosis fungoides: clinicopathological analysis of 17 patients.

BACKGROUND: Folliculotropic mycosis fungoides (FMF) represents a variant of MF characterized by hair follicle invasion of mature, CD4-positive small lymphoid cells with cerebriform nuclei. The disease displays resistance to standard treatment modalities and has an unfavourable course. OBJECTIVE: Clinical analysis of 17 patients with FMF collected between 2005 and 2012, investigation of tumour cells and involved hair follicle. METHODS: Re-evaluation of clinical data, wide panel immunohistochemistry investigation on paraffin-embedded biopsy material, T-cell receptor gene rearrangement analysis of the samples. RESULTS: Male and older age group predominance, frequent head-neck involvement, acneiform lesions, keratotic plugs, cysts, nodules, follicular papules, alopecia and classic mycosis fungoides-like plaques represented the main clinical characteristics. Treatment response showed a wide range from transient complete response to therapy resistance and death due to the disease. The pathological alterations: folliculotropism, mild epidermotropism, follicular plugging, mucinous degeneration of hair follicle, basaloid hyperplasia, syringotropism were similar to those observed previously. The first case of a CD8-positive folliculotropic mycosis fungoides - with unusual clinical presentation - is reported here. Nestin overexpression of mesenchymal cells of the isthmic and suprabulbar regions of hair follicle and the reappearance of dermal nestin-expressing cells were observed in association with immature dendritic cell hyperplasia. Altered CK19 expression was detected suggesting a potential role of follicular keratinocytes in the disease process. It was found that a proportion of neoplastic T cells constantly express programmed death-1 receptor in our patients contrary to classic mycosis fungoides. CONCLUSION: The spectrum of the clinical manifestation and the course of folliculotropic mycosis fungoides are broad and differ from classic mycosis fungoides. Folliculotropic neoplastic T-cell proliferation is associated with activation of inflammatory reactive T- and B-lymphoid cells, mesenchymal cells and changes in the hair follicle.

Periocular dermatitis: a report of 401 patients.

BACKGROUND: Periocular contact dermatitis may appear as contact conjunctivitis, contact allergic and/or irritative eyelid and periorbital dermatitis, or a combination of these symptoms. The clinical symptoms may be induced by several environmental and therapeutic contact allergens. OBJECTIVES: The aim of the present study was to map the eliciting contact allergens in 401 patients with periocular dermatitis (PD) by patch testing with environmental and ophthalmic contact allergens. METHODS: Following the methodics of international requirements, 401 patients were tested with contact allergens of the standard environmental series, 133 of 401 patients with the Brial ophthalmic basic and supplementary series as well. RESULTS: Contact hypersensitivity was detected in 34.4% of the patients. Highest prevalence was seen in cases of PD without other symptoms (51.18%), in patients of PD associated with ophthalmic complaints (OC; 30.4%), and PD associated with atopic dermatitis (AD; 27.9%). In the subgroup of PD associated with seborrhoea (S) and rosacea (R), contact hypersensitivity was confirmed in 17.6%. Most frequent sensitisers were nickel sulphate (in 8.9% of the tested 401 patients), fragrance mix I (4.5%), balsam of Peru (4.0%), paraphenylendiamine (PPD) (3.7%), and thiomersal (3.5%). By testing ophthalmic allergens, contact hypersensitivity was observed in nine patients (6.7% of the tested 133 patients). The most common confirmed ophthalmic allergens were cocamidopropyl betaine, idoxuridine, phenylephrine hydrochloride, Na chromoglycinate, and papaine. LIMITATIONS: Patients with symptoms of PD were tested from 1996 to 2006. CONCLUSIONS: The occurence of contact hypersensitivity in PD patients was in present study 34.4%. A relatively high occurrence was seen in cases of PD without other symptoms, in PD + OC and in PD + AD patients. The predominance of environmental contact allergens was remarkable: most frequent sensitizers were nickel sulphate, fragrance mix I, balsam of Peru, thiomersal, and PPD. The prevalence of contact hypersensitivity to ophthalmic allergens did not exceed l.5%.

Contact and aeroallergens in adulthood atopic dermatitis.

BACKGROUND: Atopic dermatitis (AD) is a clinically well-defined, chronic-intermittant, genetically predisposed skin disease. The increasing number of adult cases observed in the last years has turned the attention to ascertaining factors eliciting skin symptoms. Studies have revealed numerous environmental components (e.g. contact and aeroallergens) that may play an important role in sustaining the symptoms. The aim of the study was to search for contact allergens and aeroallergens triggering the skin disease in adult AD patients. METHODS: Patients over 18 years from our Atopy Outpatient Department were included in the study. Diagnosis of AD was based on the clinical criteria of Hanifin and Rajka. The distribution of skin symptoms was characteristic for adult AD: hands, shoulders, neck, flexures, face and eyelids. The extremities and the trunk were less involved. The potentially provoking contact and aeroallergens were examined in symptom and drug-free period with atopy patch and epicutaneous tests (APT, ET), which were supplemented by in vitro allergy and Prick tests. The relevance of sensibilization was evaluated by the comparison of in vivo and in vitro test results, medical history and skin symptoms. RESULTS: A total number of 34 cases of adult AD (23 women and 11 men) were studied. Four of them were classified into the intrinsic group (IG; 12%), and 30 were classified into the extrinsic group (EG; 88%). The incidence of contact sensitization to environmental allergens was remarkable: 13 of the EG, 1 of the IG (14 of 34, 41%). In the IG, a late thiomersal positivity was detected without clinical relevance. In the EG, epicutaneous standard series late positivity was seen in 13 patients, in four of them with multiple sensitivity. The allergens causing positivity were nickel (6 of 13), thiomersal (3 of 13), mercury-amidochlorate (3 of 13), mercury-chloride (2 of 13), iodine chlorhyrdoxyquin (1 of 13), lanalcolum (1 of 13) and fragrance mix (1 of 13). Among the detected allergens, the following were relevant: lanalcolum (1 of 13: cosmetics), fragrance mix (1 of 13: cosmetics), nickel (1 of 13: metal objects), thiomersal (1 of 13: eyedrops). No immediate response was seen with APT. Relevant late positivity was shown with APT test in one patient in the IG (1 of 4) to Dermatophagoides pteronyssinus. We observed late positivity in 18 patients in the EG (18 of 30). Among the detected allergens, the following were clinically relevant: D. pteronyssinus and/or Dermatophagoides farinae (15 of 18), cat epithel (4 of 18), timothy pollen (1 of 18) and dog epithel (1 of 18). Furthermore, we examined the relevance of APT, specific immunoglobulin E (IgE) tests and Prick tests. We observed multiplex positivity by specific IgE tests, APT and Prick tests in 14 patients in EG. Sensitization to D. pteronyssinus and/or D. farinae (11 of 14) cat epithel (4 of 14), dog epithel (1 of 14) and timothy pollen (1 of 14) proved to be clinically relevant with the atopic skin symptoms and medical history. CONCLUSION: The proportion of contact sensitization to environmental allergens in the 34 adult atopic patients was remarkable (14 of 34, 41%). Out of the verified contact allergens, nickel, fragrance mix, thiomersal and lanalcolum proved to be relevant. House dust mite and cat epithel proved to be the most common relevant aeroallergens. D. pteronyssinus and D. farinae sensibilization was high, particularly in patients with severe skin symptoms on the face, eyelids and hands. Pollens should be considered in patients with seasonal relapse of AD. Sensitization to animal epithel was usually indicated by the flare-up of skin symptoms upon contact with animals. The relevance of the eliciting effects of sensitization could easily be supported in most cases by the medical history and the distribution of skin symptoms. In some adult AD patients with long-lasting AD, the relevance of triggering factors is hard to determine.

Basophil CD63 expression assay on highly sensitized atopic donor leucocytes-a useful method in chronic autoimmune urticaria.

BACKGROUND: The autoimmune subclass of chronic idiopathic urticaria (CU) has been characterized by the occurrence of biologically relevant IgG antibodies against the IgE molecule or the alpha chain of the high-affinity Fcepsilon receptor (FcepsilonRIalpha) on basophils and mast cells. These antibodies are usually detected by autologous serum skin testing and confirmed by histamine release studies, immunoblotting, or enzyme-linked immunosorbent assay, but not always. OBJECTIVES: To detect autoantibodies to the FcepsilonRIalpha in sera of CU patients by a modified serum-induced basophil activation test measured by flow cytometry (FCM) and to evaluate the relationship between the in vitro functional test, the autologous serum skin test (ASST), and the serum levels of IgE, eosinophil cationic protein (ECP) and antithyroid antibodies. METHODS: Sera of 30 patients with CU and 26 patients with systemic autoimmune diseases (systemic lupus erythematosus, dermatomyositis) were tested for CD63 activation marker expression on basophils by FCM. Leucocytes from two highly sensitized atopic donors (D(A1,) D(A2)) and one non-atopic donor (D(NA)) were incubated with patients' sera and double-labelled with anti-IgE and anti-CD63 antibodies. Subsequently, the percentage of CD63-expressing basophils was determined by using FCM. In all CU patients an ASST was carried out and the serum IgE, and ECP levels and antithyroid antibodies were evaluated. RESULTS: Twelve patients had a positive ASST and 14 patients a positive CD63 expression assay. There was a strong correlation between the ASST and CD63 assay. Sera from patients with systemic autoimmune diseases did not raise positive CD63 expression on basophils. There was a moderate negative correlation between the occurrence of atopic serum markers (IgE, ECP) and the ability of sera to induce CD63 expression on basophil cells of D(A2) (P < 0.05). The female sex was preponderant and antithyroid antibodies were more frequent. CONCLUSIONS: Our new technical observation demonstrates that basophils of highly sensitized atopic donors can be successfully used without priming with IL-3 for the in-vitro flow cytofluorimetric diagnosis of CU. With this investigation the characterization of the autoimmune origin of CU is based on an objective in vitro technique.

The effect of heat-inactivated Helicobacter pylori on the blastogenic response of peripheral blood mononuclear cells of patients with chronic urticaria.

BACKGROUND: Helicobacter pylori, the most important etiologic factor of gastritis and peptic ulcer, has recently been associated with several extradigestive diseases. Previous studies reported conflicting results on H. pylori eradication in chronic urticaria, in that some studies showed a benefit, while others found no effect. METHODS: Peripheral blood mononuclear cells of 24 chronic urticaria patients (13 seropositive/11 seronegative for H. pylori) and 18 healthy controls (9 seropositive/9 seronegative) were stimulated with whole heat-inactivated H. pylori (8 x 10(5), 8 x 10(6 )and 8 x 10(7) bacteria/well), phytohemagglutinin (2 microg/ml) and pokeweed mitogen (5 microg/ml). The proliferative response was determined by (3)H-thymidine incorporation. Helicobacter-specific IgG antibody response was determined by ELISA. RESULTS: There were significantly higher proliferative responses to various concentrations of whole heat-inactivated H. pylori antigen in 6- to 7-day cultures of peripheral blood mononuclear cells of chronic urticaria patients compared to healthy controls. We found a tendency to exhibit a higher proliferative response to either Helicobacter antigens or mitogens in seropositive compared to seronegative patients. CONCLUSION: Our results support the hypothesis that there is an increased lymphocyte reactivity in chronic urticaria, perhaps further enhanced by the presence of H. pylori which, therefore, may be involved as a trigger in the pathogenesis of chronic urticaria.

[Interferon-alpha and PUVA therapy for mycosis fungoides]. / Mycosis fungoides interferon-alfa- és PUVA-kezelése.

14 patients suffering from early stage mycosis fungoides were treated with interferon alpha 2-a and PUVA/1 patient in stage I a, 3 patients in stage I b, 4 patients in stage II a and 6 patients in stage II b/during 3-21 months time course. Interferon alpha 2-a was administered 3 times a week, in escalating dose from 3 MU to 9 MU, determining the individual maximal tolerated dose. All of the patients responded well to the treatment. Partial remission was observed after 4-13 weeks of treatment. Total remission developed in 8 cases, after 8 weeks- 9 months of the treatment. Side effects occurred frequently: weight loss, pain, fever, fatigue, leucopenia, thrombopenia, liver enzyme elevation. Because of the side effects the dose of the interferon was reduced individually, the dose reduction did not cause relapse.