Relationship Between Skin Shame, Psychological Distress and Quality of Life in Patients with Psoriasis: a Pilot Study.

Shame has been registered as a part of psychosocial distress in patients with psoriasis. This study investigated 44 patients with psoriasis and 88 age- and sex-matched individuals without skin disease. Skin shame, multifarious expressions of shame, psychological symptoms, overall health, dermatological quality of life, disease burden and disease severity were measured. Higher levels of skin shame correlated with a greater disease burden (r=0.63; p < 0.01), higher Dermatology Life Quality Index (r=0.33; p < 0.05), and lower mental quality of life (r=-0.30; p <´ 0.05). Patients had a higher level of skin shame (F=74.03; eta2= 0.36; p < 0.01) and less physical quality of life on the SF-36 (F=5.14; eta2= 0.04; p < 0.05) than non-dermatological controls. General shame was not related to disease burden or quality of life. While self-rated skin shame appears to be related to quality of life in patients with psoriasis, no association was registered between expert-rated Psoriasis Area and Severity Index and quality of life.

[German version of the Skin Shame Scale (SSS-24) : Validation with dermatological patients and dermatologically healthy individuals]. / Deutsche Version der Skin Shame Scale (SSS-24) : Validierung anhand dermatologischer Patienten und dermatologisch gesunder Personen.

BACKGROUND: Skin shame plays an important role in the psychological distress of dermatological patients. However, it is rarely examined in research or clinical practice due to the lack of availability of a robust measure of skin shame. This study sought to adapt and validate the Skin Shame Scale (SSS-24) for use in the German-speaking population. METHODS: The SSS-24 questionnaire was completed by 488 dermatologically healthy (DH) individuals (66.6% women) and 339 dermatological patients (DP; 66.7% women). Participants also completed measures of general shame (SHAME) and of psychological distress (BSI[Brief Symptom Inventory]-18). RESULTS: The SSS-24 showed adequate psychometric properties and a high internal consistency in both samples (DH: α = 0.91; DP: α = 0.95). DP reported more skin shame than DH (p < 0.001) but in both samples more skin shame was associated with more general shame and more psychological distress (p < 0.001). CONCLUSIONS: Overall, the psychometric properties of the English original version were replicated in the German version of the SSS-24. Due to the strong associations with general shame and psychological distress, skin shame should be considered an important parameter in psychodermatology. The SSS-24 enables further research into the role of shame in skin conditions and provides a useful tool for identifying patients who might benefit from psychosocial interventions.

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.

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.

Cyclosporine Reduces Sclerosis in Morphea: A Retrospective Study in 12 Patients and a Literature Review.

BACKGROUND: The treatment of severe morphea is challenging, and treatment experience concerning the use of immunosuppressive agents for this condition is limited. OBJECTIVE: The purpose of this study is to analyze the use of cyclosporine, its tolerability, and its effect on skin sclerosis. MATERIALS AND METHODS: Patients with severe morphea who underwent treatment with cyclosporine were studied retrospectively. RESULTS: Five of 12 patients with morphea showed complete remission and 6 patients had partial remission at the end of therapy (9-46 months, median 14) under a median cyclosporine dose of 2.4 mg/kg. The mean affected body surface area fell from 50% (2-80, median 65) to 17% (0-40, median 18). Side effects were hypertension, elevated transaminases, cholesterol, and weight gain. CONCLUSION: Cyclosporine can be effective in morphea. The side effects were reversible. However, the duration of treatment with cyclosporine is limited because of its potential permanent side effects. Prospective placebo-controlled studies are needed to establish the superiority of cyclosporine over other immunosuppressive drugs in this setting.

German guidelines for the diagnosis and therapy of localized scleroderma.

Localized scleroderma designates a heterogeneous group of sclerotic skin disorders. Depending on the subtype, severity, and site affected, adjacent structures such as adipose tissue, muscles, joints, and bones may be involved. This is an update of the existing German AWMF (Association of the Scientific Medical Societies in Germany) guidelines (classification: S2k). These guidelines provide an overview of the definition, epidemiology, classification, pathogenesis, laboratory workup, histopathology, clinical scoring systems, as well as imaging and device-based workup of localized scleroderma. Moreover, consensus-based recommendations are given on the management of localized scleroderma depending on its clinical subtype. Treatment recommendations are presented in a therapeutic algorithm. No financial support was given by any pharmaceutical company. The guidelines are valid until July 2019.

Spiritual well-being and coping in scleroderma, lupus erythematosus, and melanoma.

BACKGROUND AND OBJECTIVES: 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. PATIENTS AND METHODS: One hundred and forty-nine patients (107 women), 44 of whom with systemic sclerosis (SSc), 48 with lupus erythematosus (LE), and 57 with stage I or II malignant melanoma (MM) were surveyed using a self-designed questionnaire, which addressed subjective well-being and disease-related circumstances, as well as the Multidimensional Inventory for Religious/Spiritual Well-Being (MI-RSWB). RESULTS: At the time of diagnosis, disease burden is greater in LE patients than in patients with SSc and MM. Only after several years are SSc and LE patients able to accept their disease. Compared with healthy individuals, the overall score of R-S well-being is significantly lower in LE patients. In LE, photosensitivity and joint pain are inversely correlated with the ability to forgive. SSc patients with facial lesions and pulmonary involvement show greater religiosity. MM patients display significantly higher values for transcendental hope. CONCLUSION: Talks about the disease and psychological support are the most important needs of patients with SSc, LE, and MM. At present, programs aimed at improving R-S coping skills do not seem to play a significant role, but could be an important resource that should be addressed in the future.

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

HINTERGRUND UND ZIELE: 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. PATIENTEN UND METHODIK: 149 Patienten (107 Frauen), 44 mit systemischer Sklerodermie (SKL), 48 mit Lupus erythematodes (LE) und 57 mit malignem Melanom (MM), Stadium I-II, wurden mittels eines selbstentwickelten Fragebogens zum subjektiven Wohlbefinden, zu den mit der Erkrankung einhergehenden Umständen sowie mit dem Multidimensionalen Inventar (MI-RSB) zu R-S befragt. ERGEBNISSE: LE-Patienten sind zum Zeitpunkt der Diagnosestellung stärker belastet als SKL- und MM-Patienten. SKL- und LE-Patienten können erst nach Jahren die Erkrankung akzeptieren. Der Gesamtscore des religiös-spirituellen Befindens liegt bei LE-Patienten signifikant unter dem Wert der Normalbevölkerung. Fotosensitivität und Gelenksschmerzen sind bei LE-Patienten negativ assoziiert mit der Fähigkeit Vergeben zu können. SKL-Patienten mit Gesichtsveränderungen und Lungenbeteiligung zeigen höhere allgemeine Religiosität. MM-Patienten haben höhere Werte für transzendente Hoffnung. SCHLUSSFOLGERUNGEN: Vorträge über die Krankheit und psychologische Betreuung sind die wichtigsten Bedürfnisse von Patienten mit SKL, LE und MM an ihre Betreuer. Religiös-spirituelle Angebote zur Krankheitsverarbeitung scheinen derzeit eine untergeordnete Rolle zu spielen, könnten aber eine wichtige Ressource sein, der man in Zukunft mehr Aufmerksamkeit schenken sollte.

Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis.

BACKGROUND: Systemic sclerosis (SSc)-overlap syndromes are a very heterogeneous and remarkable subgroup of SSc-patients, who present at least two connective tissue diseases (CTD) at the same time, usually with a specific autoantibody status. OBJECTIVES: To determine whether patients, classified as overlap syndromes, show a disease course different from patients with limited SSc (lcSSc) or diffuse cutaneous SSc (dcSSc). METHODS: The data of 3240 prospectively included patients, registered in the database of the German Network for Systemic Scleroderma and followed between 2003 and 2013, were analysed. RESULTS: Among 3240 registered patients, 10% were diagnosed as SSc-overlap syndrome. Of these, 82.5% were female. SSc-overlap patients had a mean age of 48±1.2 years and carried significantly more often 'other antibodies' (68.0%; p<0.0001), including anti-U1RNP, -PmScl, -Ro, -La, as well as anti-Jo-1 and -Ku antibodies. These patients developed musculoskeletal involvement earlier and more frequently (62.5%) than patients diagnosed as lcSSc (32.2%) or dcSSc (43.3%) (p<0.0001). The onset of lung fibrosis and heart involvement in SSc-overlap patients was significantly earlier than in patients with lcSSc and occurred later than in patients with dcSSc. Oesophagus, kidney and PH progression was similar to lcSSc patients, whereas dcSSc patients had a significantly earlier onset. CONCLUSIONS: These data support the concept that SSc-overlap syndromes should be regarded as a separate SSc subset, distinct from lcSSc and dcSSc, due to a different progression of the disease, different proportional distribution of specific autoantibodies, and of different organ involvement.

Disease spectrum in patients with elevated serum tryptase levels.

BACKGROUND: Elevated serum tryptase levels can be a sign of mastocytosis, which is a rare disease associated with systemic and/or skin manifestations. OBJECTIVE: To investigate patients with elevated tryptase levels in regard to their underlying diseases, and to determine whether increased tryptase can be used as a diagnostic marker for underlying mastocytosis. METHODS: In a retrospective study the data of 96 patients with serum tryptase levels higher than 15 µg/L were systematically analysed. In 48 patients control investigations for baseline tryptase were performed. RESULTS: Fifty-three of the 96 patients had tryptase levels ≥20 µg/L. A mere 16% of the 96 patients suffered from mastocytosis and had the highest tryptase levels (P < 0.001). The remaining patients had anaphylaxis (36%), urticaria and angioedema (26%), local reactions to insect bites (4%), drug reactions (3%), or miscellaneous diagnoses (15%). Only 16 of these had acute symptoms at tryptase investigation. In all, 48 patients had a follow up; in 7/48 patients with acute symptoms normal tryptase levels were seen at control investigations, but 41/48 (85%) patients showed continuously elevated tryptase levels >15 µg/L and in 30 patients (62%) even values >20 µg/L; 11 of these patients had anaphylaxis, five urticaria, five other diagnoses and nine patients mastocytosis. CONCLUSION: More than 50% of patients with non-mastocytosis such as urticaria and angioedema, drug or anaphylactic reactions repeatedly had tryptase levels higher than 20 µg/L. Since baseline tryptase >20 µg/L is a minor criterion for mastocytosis, these patients should be inspected for skin lesions of mastocytosis and receive a diagnostic body work-up for systemic mastocytosis including a bone marrow biopsy.

Pulmonary arterial hypertension therapy may be safe and effective in patients with systemic sclerosis and borderline pulmonary artery pressure.

OBJECTIVE: Borderline pulmonary arterial hypertension (PAH), characterized by a marked exercise-induced increase in pulmonary artery pressure (PAP) with normal resting values, may precede overt PAH in systemic sclerosis (SSc). We undertook the present study to investigate whether PAH treatment is safe in these patients and might attenuate hemodynamic progression. METHODS: SSc patients with borderline PAH underwent right heart catheterization at baseline, after a 12-month observation period, and subsequently after 6 months of bosentan therapy. Changes in mean PAP at 50W during the observation period versus during therapy were compared. RESULTS: Ten patients completed the study. Mean PAP at rest, at 50W, and during maximal exercise increased significantly during the observation period (mean ± SD increases of 2.5 ± 3.0 mm Hg [P = 0.03], 4.0 ± 2.9 mm Hg [P = 0.002], and 6.8 ± 4.1 mm Hg [P = 0.0005], respectively) and tended to decrease during the treatment period (decreases of 2.5 ± 3.9 mm Hg [P = 0.07], 1.5 ± 4.5 mm Hg [P = 0.32], and 1.8 ± 7.0 mm Hg [P = 0.43], respectively). The changes during the observation period versus the therapy period were significantly different (P = 0.03 at rest, P = 0.01 at 50W [primary end point], and P = 0.02 during maximal exercise). The changes in resting pulmonary vascular resistance were also significantly different during the observation period (increase of 8 ± 25 dynes · seconds · cm(-5) ) versus during the therapy period (decrease of 45 ± 22 dynes · seconds · cm(-5) ) (P < 0.0005). Changes in resting pulmonary arterial wedge pressure were not significantly different between the observation period and the treatment period, despite the significant increase during the observation period (2.6 ± 2.5 mm Hg [P = 0.01]). No relevant adverse effects were reported. CONCLUSION: In SSc patients with borderline abnormal pulmonary hemodynamics, resting and exercise PAP may increase significantly within 1 year of observation. Bosentan might be safe and effective to attenuate these changes. Randomized controlled trials are warranted to confirm the exploratory findings of this hypothesis-generating pilot study.

Calcinosis cutis: part I. Diagnostic pathway.

Calcinosis cutis is characterized by the deposition of insoluble calcium salts in the skin and subcutaneous tissue. The syndrome is separated into five subtypes: dystrophic calcification, metastatic calcification, idiopathic calcification, iatrogenic calcification, and calciphylaxis. Dystrophic calcification appears as a result of local tissue damage with normal calcium and phosphate levels in serum. Metastatic calcification is characterized by an abnormal calcium and/or phosphate metabolism, leading to the precipitation of calcium in cutaneous and subcutaneous tissue. Idiopathic calcification occurs without any underlying tissue damage or metabolic disorder. Skin calcification in iatrogenic calcinosis cutis is a side effect of therapy. Calciphylaxis presents with small vessel calcification mainly affecting blood vessels of the dermis or subcutaneous fat. Disturbances in calcium and phosphate metabolism and hyperparathyroidism can be observed.

Calcinosis cutis: part II. Treatment options.

Because calcinosis cutis is a rare syndrome, there is a notable lack of controlled clinical trials on its treatment. The efficacy of calcinosis treatment has only been reported in single cases or small case series. No treatment has been generally accepted as standard therapy, although various treatments have been reported to be beneficial, including warfarin, bisphosphonates, minocycline, ceftriaxone, diltiazem, aluminium hydroxide, probenecid, intralesional corticosteroids, intravenous immunoglobulin, curettage, surgical excision, carbon dioxide laser, and extracorporeal shock wave lithotripsy.

Clinical Impact of Skin Lesions in Mastocytosis: A Multicenter Study of the European Competence Network on Mastocytosis.

Mastocytosis is a rare neoplasm characterized by the expansion and accumulation of mast cells in various organ systems. Systemic mastocytosis (SM) may or may not present with cutaneous lesions. To examine the frequency and clinical impact of cutaneous involvement, data on 1,510 patients with mastocytosis collected in the registry of the European Competence Network on Mastocytosis were analyzed. Cutaneous involvement was found in 1,195 of 1,510 patients (79.1%). Of these, 286 had cutaneous mastocytosis, and 721 had SM with skin involvement. Adult patients with skin involvement who did not have a bone marrow examination (n = 188) were defined as having mastocytosis in the skin. In 315 patients, SM without skin involvement was found. The percentage of cases with cutaneous involvement was higher in indolent SM (100%) and smoldering SM (87.9%) compared to aggressive SM (46.8%) or mast cell leukemia (38.5%). After a median follow-up of 5.6 years, no patient with cutaneous mastocytosis had died, but 2.6% of the patients with mastocytosis in the skin, 5.7% of the patients with SM with skin involvement, and 28.95% of the patients with SM without skin involvement had died. Overall survival was longer in patients with skin involvement (cutaneous mastocytosis and/or mastocytosis in the skin and/or SM with skin involvement) than in patients with SM without skin involvement (P < 0.0001). These data argue for a thorough examination of both the skin and bone marrow in adult patients with mastocytosis.

Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin.

BACKGROUND: Mastocytosis in adults often presents with skin lesions. A bone marrow biopsy is necessary to confirm or exclude the presence of systemic mastocytosis (SM) in these cases. When a bone marrow biopsy is not performed, the provisional diagnosis is mastocytosis in the skin (MIS). No generally accepted scoring system has been established to estimate the risk of SM in these patients. OBJECTIVE: To develop a risk score to predict SM in adults with MIS. METHODS: We examined 1145 patients with MIS from the European Competence Network on Mastocytosis Registry who underwent a bone marrow biopsy. A total of 944 patients had SM and 201 patients had cutaneous mastocytosis; 63.7% were female, and 36.3% were male. Median age was 44 ± 13.3 years. The median serum tryptase level amounted to 29.3 ± 81.9 ng/mL. We established a multivariate regression model using the whole population of patients as a training and validation set (bootstrapping). A risk score was developed and validated with receiver-operating curves. RESULTS: In the multivariate model, the tryptase level (P < .001), constitutional/cardiovascular symptoms (P = .014), and bone symptoms/osteoporosis (P < .001) were independent predictors of SM (P < .001; sensitivity, 90.7%; specificity, 69.1%). A 6-point risk score was established (risk, 10.7%-98.0%) and validated. CONCLUSIONS: Using a large data set of the European Competence Network on Mastocytosis Registry, we created a risk score to predict the presence of SM in patients with MIS. Although the score will need further validation in independent cohorts, our score seems to discriminate safely between patients with SM and with pure cutaneous mastocytosis.

Borderline pulmonary arterial pressure is associated with decreased exercise capacity in scleroderma.

RATIONALE: Pulmonary arterial hypertension is associated with impaired exercise capacity and decreased survival in patients with scleroderma. Randomized controlled studies showed significant benefit of targeted therapies in patients with a resting mean pulmonary arterial pressure (MPAP) greater than 25 mm Hg. The clinical relevance of pulmonary arterial pressure values in the upper normal range is unknown. OBJECTIVES: To examine the clinical relevance of pulmonary arterial pressure in scleroderma patients. METHODS: After a noninvasive screening program, 29 patients with systemic sclerosis without significant lung fibrosis and without known pulmonary arterial hypertension underwent right heart catheterization and simultaneous cardiopulmonary exercise test. A six-minute walk distance (6MWD) was determined within 48 hours. MEASUREMENTS AND MAIN RESULTS: A resting MPAP above the median (17 mm Hg) was associated with decreased 6MWD (396 +/- 71 vs. 488 +/- 76 m; P < 0.005) and peak Vo(2) (76 +/- 11% vs. 90 +/- 24%; P = 0.05). Resting pulmonary vascular resistance was inversely correlated with 6MWD (r = 0.45; P < 0.05). At 25 and 50W, MPAP above the median (23 and 28 mm Hg) was associated with decreased 6MWD (P < 0.005; P < 0.0005). At peak exercise, MPAP showed no association with 6MWD or peak Vo(2); however, cardiac index was positively (r = 0.45; P < 0.05) and pulmonary vascular resistance was negatively correlated with 6MWD (r = -0.38; P < 0.05). CONCLUSIONS: MPAP and resistance in the upper normal range at rest and moderate exercise are associated with decreased exercise capacity and may indicate early pulmonary vasculopathy in patients with systemic sclerosis. Investigations on the prognostic and therapeutic implications of such borderline findings are warranted. Clinical trial registered with http://www.clinicaltrials.gov (NCT00609349).

Pseudolymphomatous tumid lupus erythematosus of the oral mucosa.

Oral lesions are frequent complications of systemic lupus erythematosus, but only ulceration is included in the 1982 American College of Rheumatology revised criteria. Because the lack of a uniform classification, a range of ulcerative and keratotic lesions are typically described. In this report we describe a unique progressive irregularly cobblestoned and vegetating plaque of the oral mucosa with clinical and histological features mimicking a cutaneous lymphoma. Despite the papillomatous and extensive nature of the lesions and the dense lymphoid infiltrate with follicle formation suggesting a malignant lymphoproliferative process, the slow progression coupled with a mixed cell infiltrate and polyclonality supported a diagnosis of pseudolymphoma. Recognition of this entity is important to prevent diagnosing them as a malignant lymphoma. As well as with the other mucosal lesions in lupus erythematosus, this pseudolymphomatous variant should be added to the disease spectrum.