Nora Wutte

CXCL13 chemokine in pediatric and adult neuroborreliosis

Wutte N, Berghold A, Löffler S, Zenz W, Daghofer E, Krainberger I, Kleinert G, Aberer E. CXCL13 chemokine in pediatric and adult neuroborreliosis. 
Acta Neurol Scand: 2011: 124: 321–328. 
© 2011 John Wiley & Sons A/S.

Within European margins

In June, 2008, an 88-year-old woman presented to us with a 4-month history of dark red, partly ulcerated cutaneous nodules and plaques on both feet (fi gure A). We suspected large B-cell lymphoma of the leg, cutaneous follicle centre lymphoma, or merkel-cell carcinoma. Full blood count was normal. Serum protein electrophoresis suggested a monoclonal IgG λ band. No antibodies to lymphotropic viruses, Toxoplasma gondii, or Helicobacter pylori were detected, but IgG antibodies to Borrelia burgdorferi were found. Sonography, chest radiography, and whole-body CT were normal. Biopsy samples from the plaques showed dense infi ltrates with CD20-positive lymphocytes mixed with lympho plasmacytoid plasma cells and small reactive lymphocytes (fi gure B). Dutcher bodies (PAS-positive, diastaseresistant nuclear pseudoinclusions found in malignant plasma cells) were observed, and also a monoclonal κ light-chain restriction of plasma cells. The presence of B burgdorferi DNA was shown by PCR targeting of the OspA gene. Sequence analysis of the amplifi ed DNA showed that the strain belonged to the Borrelia afzelii genospecies. Our patient did not recall a tick bite, and she had no history of erythema migrans, borrelia lymphocytoma, acrodermatitis chronica atrophicans, or other symptoms of Lyme borreliosis. Primary cutaneous marginal-zone lymphoma with plasmacytic diff erentiation induced by B afzelii was diagnosed. Ceftriaxone 2 g intravenously for 3 weeks was started. 2 months after the start of treatment, the plaques had substantially regressed. After 18 months, red, scaling, hyperpigmented eczematous lesions persisted only on the left foot. On histological examination, Dutcher bodies were greatly reduced. B burgdorferi antibody titres had decreased. On serum electrophoresis, there was a switch from λ to κ light chain, which was in line with the histological fi ndings of monoclonal κ light chain proliferation. Our patient’s lesions regressed gradually, but erythema was still present after 24 months. This phenomenon of slow regression is also seen in late Lyme borreliosis and suggests that the eff ectiveness of antibiotics might be misinterpreted. Antibiotic therapy should be considered as a fi rst-line treatment option in patients with cutaneous marginalzone lymphoma, in the presence of B burgdorferi infection, or local radiotherapy in doses of 10–50 Gy. We did not give radiotherapy because of our patient’s age and stable disease state. B burgdorferi sensu lato has been shown by PCR in cutaneous B-cell lymphomas in European, but not in American, or Asian patients. Response to antibiotic treatment has been reported. The phylogenetic link to the B afzelii genospecies has not been made for skin lymphomas. In our patient, B afzelii specifi c DNA was reported for the fi rst time in skin lymphoma. The predominant species isolated from acrodermatitis chronica atrophicans and borrelia lymphocytoma in Europe is B afzelii. Since B afzelii is not present in the USA, cutaneous B-cell lymphoma induced by this genospecies is probably a European phenomenon. B burgdorferi serology can be borderline or negative, even in patients with lymphoma with B burgdorferi positive PCR. Acrodermatitis chronica atrophicans is a polyclonal lymphocytic proliferation, but clonal B-cell proliferation has been observed in erythema migrans. Further studies are needed to show whether malignant lympho proliferation is a unique phenomenon of B afzelii or is also induced by other B burgdorferi genotypes.

Impaired quality of life in patients with systemic sclerosis compared to the general population and chronic dermatoses

BackgroundSystemic sclerosis (SSc) is a rare and potentially life threatening autoimmune disorder. The burden of disease compared to other dermatoses is unknown. The purpose of this study was to assess both the quality of life in patients with SSc and the variables that are associated with poor quality of life. Forty-one patients with systemic sclerosis (29 limited, 2 diffuse, 10 undifferentiated forms) were assessed with respect to their health status and compared to published data for the normal population, SSc patients from other studies, and patients with chronic skin diseases.ResultsFor the most part, our SSc patients had better outcomes in all 8 dimensions of the SF-36 than SSc patients from other studies, and poorer scores than the healthy population and those with occupational contact dermatitis, ichthyosis, non-melanoma skin cancer, contact dermatitis, atopic eczema, chronic nail disease, vitiligo, health care workers with work-related disease, and those with other chronic skin diseases, but significantly better scores for mental health than those with nail disease, vitiligo, and health-care workers. Patients with atopic dermatitis, psoriasis and pemphigus had significantly poorer mean scores in social function and mental health than SSc patients. Patients with pemphigus were also significantly impaired in their physical and emotional roles. Patients with systemic lupus erythematosus (SLE) had the significantly poorest mean scores for QoL in all 8 domains except bodily pain and emotional role.ConclusionBesides SLE, SSc is one of the most severe chronic dermatologic diseases in terms of reduced QoL. Since SSc cannot be cured, treatment strategies should include therapeutic interventions such as psychotherapy, social support, physiotherapy, and spiritual care. Their beneficial effects could be studied in future.

CXCL13 and B-cell activating factor as putative biomarkers in systemic sclerosis

1 Baran R, Dupr e A, Sayag J et al. Bowen disease of the nail apparatus. Report of 5 cases and review of the 20 cases of the literature. Ann Dermatol Venereol 1979; 106:227–33. 2 Weisenseel P, Prinz J, Korting H. Treatment of paraungual HPV73-positive Bowen disease with imiquimod cream. Hautarzt 2006; 57:309–10. 3 Cox NH, Eedy DJ, Morton CA. Guidelines for management of Bowen’s disease: 2006 update. Br J Dermatol 2007; 156:11–21. 4 Kim MG, Kim WS, Lee JH et al. Carbon dioxide laser de-epithelialization: selective removal of the epidermis with minimal change to the basement membrane zone. Dermatol Surg 2006; 32:329–31.

Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study

Pulmonary arterial hypertension (PAH) is a feared complication of systemic sclerosis. In this prospective cohort study, we monitored the changes in resting and exercise pulmonary haemodynamics of scleroderma patients without initial PAH over a mean follow-up period of ∼4 years. All patients underwent exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. A subgroup underwent exercise right heart catheter (RHC) investigations. The primary end-point was the echocardiographic systolic pulmonary arterial pressure at 50 W exercise (sPAP50). We included 99 patients, of whom 58 had a complete dataset. Three out of 99 patients developed RHC-confirmed PAH (0.75 cases per 100 patient-years). sPAP50 increased (p<0.001) and peak oxygen uptake (secondary end-point) decreased significantly (p=0.001) during follow-up, but there was no significant change in resting sPAP (p=0.38). In the RHC subgroup (n=28), mean (m)PAP and pulmonary vascular resistance at 50 W increased significantly (p=0.02 and p=0.002, respectively), but resting mPAP was unchanged. Scleroderma patients without PAH develop a mild but significant deterioration of pulmonary exercise haemodynamics and exercise capacity over a 4-year follow-up period, indicating a progression of pulmonary vascular disease. The manifestation rate of RHC-confirmed PAH was 0.75 cases per 100 patient-years. Scleroderma patients without pulmonary hypertension develop mild deterioration in pulmonary exercise haemodynamics http://ow.ly/hva930aEW4E

Dapsone as Second-Line Treatment for Cutaneous Lupus Erythematosus? A Retrospective Analysis of 34 Patients and a Review of the Literature

Background: A variety of therapeutic options have been reported for cutaneous lupus erythematosus (CLE); one of these is dapsone. However, no high priority has been given to this drug. Objective: To determine whether dapsone is an effective and safe treatment alternative in patients with LE. Methods: We reviewed the literature and analyzed retrospectively 34 CLE patients who were treated with dapsone as monotherapy or combined with antimalarials. We assessed the course of the disease under treatment, response, concomitant treatment and side effects. Results: Six patients went into remission, 14 patients improved, and in 6 patients disease remained constant during the observation period. Dapsone was discontinued in 9 patients: in 4 due to reversible side effects and in 5 patients due to poor efficacy. Conclusion: Our data confirm that dapsone with/without antimalarials is effective in more than 50% of patients with CLE and could be used as second-line therapy for CLE.

The influence of religious/spiritual well-being on quality of life in dermatological disease

DEAR EDITOR, Although the association between spirituality and parameters of health and disease has been investigated extensively, little evidence is available for its potential role in dermatology. In order to lay some groundwork, we analysed religious and existential parameters and correlated these with the quality-of-life (QoL) subscales of physical and mental characteristics in patients with different skin diseases. Specifically, we sought to address what effect religiosity/spirituality had on human health, particularly in light of the ongoing secularization and changing social needs of Western Europe in particular. Spirituality as one of the key elements of religion has been confirmed as an integral aspect of QoL and in palliative and end-of-life care. Religion as being related to various parameters of mental health and illness was described prominently as ‘the search for significance in ways related to the sacred’. Recently, we have shown that, among patients with chronic skin diseases, those with systemic sclerosis (SSc) and lupus erythematosus (LE) have the poorest QoL. In a single-centre observational prospective study we investigated the patients’ spiritual needs and the way in which they related to QoL in 149 outpatients (107 women). These patients had SSc (n = 44, 37 women, age range 23–80 years), cutaneous LE (n = 48, 41 women, age range 24–78 years) and malignant melanoma (MM) (n = 57, 29 women, age range 23– 80 years). Patients were classified for clinical SSc subtypes according to the criteria of LeRoy and Medsger, and the different types of cutaneous LE according to the Duesseldorfer classification. A comparison group with early-stage MM (stage I–II, without metastasis) was recruited in order to differentiate chronic diseases (SSc, LE) from a nonchronic disease (MM). Furthermore, all dermatological samples were compared with normative nonclinical samples. More detailed patient data (e.g. marital status, children and religion) were published in a recent paper. Ethical approval (EK no. 25-280 ex12/13) was given by the ethical committee of the Medical University of Graz, Austria. Patients were interviewed using the Austrian–German standardized and normalized Multidimensional Inventory of Religious/Spiritual Well-Being. Spiritual well-being, a subjective phenomenon, consists in equal measures of existential wellbeing and religious well-being with different subscales (Table 1). Religious/spiritual well-being (RSWB) has been defined as ‘the ability to experience and integrate meaning and purpose in existence through a connectedness with self, others, or a power greater than oneself. QoL was measured by the Medical Outcomes Study 36-Item Short Form Health Survey (SF-36), with two standardized global scores: the physical component score (PCS) and the mental component score (MCS), consisting of QoL and various subscales (Table 1). Compared with the general population, dermatological patients showed lower levels of RSWB, with low scores in patients with LE (P < 0 001), and low values for Experience of Sense and Meaning (P < 0 001). Patients with MM had significantly lower scores for General Religiosity and Connectedness, but higher scores for Forgiveness (P = 0 003) and Hope Transcendent (P = 0 002) (Table 1). QoL was substantially lower in patients with SSc and LE, but was not reduced in patients with MM. In patients with SSc we found a reduced amount of physical role functioning (P < 0 001), vitality (P < 0 001) and emotional role functioning (P < 0 001), and a low score in total PCS (P < 0 01). Furthermore, patients with LE exhibited particularly low scores for general health (P < 0 001) and vitality (P < 0 001). As revealed by correlation and regression analysis (Table 2), spiritual dimensions such as Hope Immanent (hope for a better future) and Hope Transcendent (hope for a better afterlife) turned out to be positively related with general health (both P < 0 001), as well as with vitality (both P < 0 001), social functioning (P < 0 001 and P < 0 01, respectively), emotional role functioning (both P < 0 001) and total MCS (both P < 0 001). Moreover, substantial correlations were found between mental health (P < 0 001) and the MCS (P < 0 001) with total RSWB. Generally, existential well-being was a strong predictor (b = 0 42) of general health. We found that all mental scores of the SF-36 are significantly associated with existential and, to a minor extent, general RSWB. However, we registered a lower level of RSWB for patients with SSc or LE, which is related to their poor general health and vitality, and a lack of emotional role functioning. Spiritual issues were reported predominantly as being related to existential crises in terminally ill patients or those at the end of their lives. However, these existential

Spirituelles Wohlbefinden und Coping bei Sklerodermie, Lupus erythematodes und malignem Melanom

Religiös‐spirituelles Wohlbefinden ist verbunden mit höherer Vitalität und verminderter Depressionsneigung. In unserer Studie untersuchten wir die Strategien zur Krankheitsbewältigung und die Rolle von Religiosität‐Spiritualität (R‐S) zur Verbesserung des subjektiven Wohlbefindens.

Spiritual well‐being and coping in scleroderma, lupus erythematosus, and melanoma

Religious/spiritual (R‐S) well‐being is associated with greater vitality and lower depression scores. In this study, we investigated strategies for coping with disease and the role of religiosity/spirituality with respect to improving subjective well‐being.

Frontal hair loss and facial skin changes

Figure 1 Confluent scaly erythematous flat papules and plaques on the patient’s nose and cheek (a). Recession of the temporal hairline with “lonely hairs” in an alopecia patch (b). Figure 2 A biopsy from the face reveals a dense superficial and deep lichenoid interface dermatitis consistent with lupus dermatitis (hematoxylin-eosin stain, original magnification x 40). the frontotemporal region, we observed alopecia with effacement of follicular orifices and perifollicular erythema (Figures 1a, b).