Clemens Painsi

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.

Psoriasis Area and Severity Index 75 rate of classical inpatient dithranol therapy under daily life conditions

DEAR EDITOR, Dithranol (known in the U.S.A. as anthralin and in Germany as cignolin) is one of the oldest and safest longterm topical antipsoriatic treatments. However, established evidence for dithranol’s therapeutic efficacy is scant, and data regarding the extent to which dithranol may reduce the Psoriasis Area and Severity Index (PASI), the current standard parameter for assessing therapeutic efficacy in treated patients with psoriasis, are minimal. To help fill this gap, the aim of this study was to analyse retrospective data on the efficacy of classical dithranol therapy (CID) in terms of PASI reduction under daily life conditions. Data from 110 patients [75 men (68 2%) and 35 women (31 8%)] were reported to the Psoriasis Registry Austria (http://www.psoriasisregistry.at) and analysed retrospectively. Full details of the methods are provided in Data S1 (see Supporting Information). The median patient age was 48 3 years (range 13 4–97 7). Median body mass index (BMI) was 27 0 (range 18 1–38 8). Psoriasis types included chronic plaque psoriasis in 77 patients (70 0%) and guttate psoriasis in 33 (30 0%). Median disease duration was 10 2 years (range 0 1–84 0), and the median PASI score at treatment initiation was 13 0 (range 2 8–40 0). A family history of psoriasis was reported in 28 patients (25 4%), no family history of psoriasis in 36 (32 7%) and an uncertain family history in 46 (41 8%). Comorbidities included obesity in 40 patients (36 4%), fatty liver disease in 28 (25 4%), insulinor noninsulin-dependent diabetes mellitus in 16 (14 5%), hyperlipidaemia in 16 (14 5%), arterial hypertension in 30 (27 3%), coronary heart disease in six (5 4%) and kidney disease in three patients (2 7%). CID was administered in the formulations described in Table S1 (see Supporting Information). Seventy-seven patients (70 0%) received CID alone and 33 (30 0%) received CID in combination with ultraviolet (UV) B irradiation at 311 nm. The median number of UV exposures was five (range 1–11). Other than a between-group difference in the relative percentage of patients with plaque vs. guttate psoriasis (i.e. 75 3% vs. 24 7% in the monotherapy group compared with 57 6% vs. 42 4% in the combination therapy group; Fisher’s exact test P = 0 07), there were no major differences in clinical characteristics between the treatment groups. Ninety-one patients (82 7%) completed treatment with CID and achieved ≥ 75% reduction in PASI score (PASI 75; Fig. 1). The median time to PASI 75 was 14 days (range 3–25 days). In 19 patients (17 3%), treatment with CID was terminated early (i.e. before achieving PASI 75) for reasons including lack of efficacy [i.e. PASI reduction < 50% after at least 10 days of therapy (n = 5; 4 5%)]; local irritation [burning sensation of the skin and erythema (n = 3; 2 7%); and patient decision (n = 11; 10 0%). The median time to early treatment termination in those 19 patients was 5 days (range 2–19) (Fig. 1). Analysis revealed no difference in the rates of efficacy (i.e. PASI 75) and early treatment termination between the monotherapy and combination (irradiated) treatment group (data not shown), and therefore they were pooled for analysis. There was a weak but statistically significant positive correlation between baseline PASI and time to PASI 75 (P < 0 01; Fig. 2). This became particularly evident for patients with a PASI score < 10 at start of treatment, as visualized by the strong slope of the LOWESS line for this range (Fig. 2). The median time to reach PASI 75 was 10 2 days in patients with a PASI score < 10 (n = 30) and 13 6 days in patients with a PASI score > 10 (n = 61) (Wilcoxon test P < 0 01).

A case of Schöpf-Schulz-Passarge syndrome caused by c.1135C>T WNT10A missense mutation

An 86-year-old female patient was referred to our clinic for evaluation of plantar erosions. She reported a lifelong history of palmoplantar keratoderma, which, in recent years, had evolved into painful hyperkeratotic papules and erosions, as well as oligodontia of primary and permanent teeth, and massive palmoplantar hyperhidrosis, which had disappeared in early adulthood. Her medical records revealed that numerous neurocutaneous fi bromas (on the trunk) had been excised over the years. At the age of 50, a squamous cell carcinoma had been removed from the right palm. The patient was born to healthy parents, with no known ectodermal abnormalities or consanguineous background.

Herpes zoster in an infant without postnatal varicella infection

We report the case of a five-month-old female infant who presented with a two-day history of small blisters on the abdomen and the right thigh. At the time of examination, the infant was in good general health and exhibited ageappropriate development without any signs of pain or fever. Her medical history did not reveal any evidence of a prior varicella infection. Up to this point, the infant had received all vaccinations recommended in the Austrian immunization schedule (rotavirus, diphtheria, tetanus, pertussis, polio, Haemophilus influenza type b, hepatitis B, pneumococci); the MMR vaccination (recommended after the 10th month of life according to the Austrian immunization schedule) was yet to be administered. Clinically, the patient showed grouped vesicles on an erythematous base that were clearly confined to dermatomes T12–L2 (right side) (Figure 1). Using polymerase chain reaction, the diagnosis was confirmed by the detection of varicella-zoster virus (VZV) nucleic acids. Serum VZV IgG was elevated (201 mU/mL; normal value < 149 mU/mL); VZV IgM was within normal limits. Humoral or cellular immune deficiency was ruled out by measuring the various subclasses of serum immunoglobulins, as well as by another pediatric consult. Over the course of seven days, the infant subsequently received an oral acyclovir suspension (15 mg/kg, five times daily). Topical therapy initially included a zinc lotion, later panthenol ointment (Bepanthen®). Follow-up ten days after the start of treatment showed significant clinical improvement with only minor persistent erythema in the area previously covered by vesicles (dermatomes T12–L2 right side). Upon repeated questioning of the patient’s mother, an Indian migrant, and review of her medical history, we were able to ascertain a (documented) mild VZV infection with a polymorphous rash (at typical location) in the 4th gestational week.

Patient perspectives on treating psoriasis with classic inpatient dithranol therapy: a retrospective patient survey

Evidence of the efficacy of dithranol and patient perspectives on the treatment is scant.

Multiple miliary osteoma cutis of the scalp

A 76-year-old patient presented with a 7-year history of asymptomatic scalp lesions, which had been gradually increasing in size. One year earlier, he had undergone topical treatment with diclofenac (Solaraze®) followed by photodynamic therapy for multiple actinic keratoses. The patient denied any past history of skin disorders or trauma to the scalp. In addition to actinic damage, examination of the skin revealed multiple disseminated, firm, movable, whitish papules, measuring up to 5 mm (Figure 1). Histology of a completely excised papule showed a sharply demarcated area of mature lamellar bone in the upper and mid dermis. The epidermis was unremarkable (Figure 2a). The lamellar bone had several medullary spaces peripherally lined with osteoblasts (Figure 2b) and some isolated osteoclasts (Figure 2c). Lab tests for alkaline phosphatase, calcium, phosphate, and parathyroid hormone were within normal limits. Bone remodeling parameters such as ß-CrossLaps and osteocalcin, as well as mutation analysis of the GNAS gene, were also unremarkable. A cranial CT scan revealed extraskeletal bone formation on the scalp (cutaneous bone deposition without connection to the skull) (Figure 3). The skull itself as well as the bones of the hand – as illustrated by X-rays – were without pathological findings. Correlation of the clinical presentation and histopathology established the diagnosis of multiple miliary osteoma cutis of the scalp. As the patient was symptom-free, he declined any therapeutic intervention. Multiple miliary osteoma cutis is a very rare condition characterized by extraskeletal bone formation in the skin and/or subcutis. In their review of 51 cases of multiple miliary osteoma cutis, Myllyla et al. reported that the majority of patients were women (n = 42), with a large percentage of

The Prevalence of Periodontitis Is Increased in Psoriasis and Linked to Its Inverse Subtype

Periodontitis and psoriasis are suggested to be co-occurring, chronic inflammatory conditions with overlapping characteristics. However, respective evidence is rare and data on risk factors of periodontitis in psoriasis patients are minimal. The aim of this study was to expand the evidence of psoriasis-associated periodontitis and establish a potential risk profile for periodontitis. In total, data from 209 exacerbated psoriasis patients were retrospectively analyzed on recordings of periodontitis and compared with those of 91 patients with chronic spontaneous urticaria (CSU). Analysis showed a significantly increased prevalence of periodontitis in psoriasis compared to CSU patients with an odds ratio of 3.76 (95% CI = 1.60-10.27, p = 0.001). Within the psoriatic subtypes, the presence of the inverse type (affecting intertriginous body areas) was strongly linked to periodontitis with an odds ratio of 5.11 (95% CI = 1.36-20.38, p = 0.006). These results are enlarging the evidence for psoriasis-associated periodontitis and identify a link between the inverse type of psoriasis and periodontitis.

Image Gallery: Cutaneous signs of acute pancreatitis

DEAR EDITOR, An 86-year-old septic patient with acute pancreatitis showed dirty green-greyish discolorations on the periumbilical and pubic regions (a) and on the flanks and upper parts of the thighs (b) beside a heparin injection-related haematoma (marked *). Four weeks later the patient died of multiorgan failure. The discolorations are based on subcutaneous ecchymosis caused by liberated pancreatic enzymes or retroperitoneal bleeding. Cullen (periumbilical, a), Stabler (pubic area, a), Turner (flanks, b) and Fox (thighs, b) signs, named after their first describers, are observed in < 3% of patients with acute pancreatitis and are associated with a fulminant progress (mortality rate up to 37%).

Prognostischer Wert der Fläche und Dichte von Lymphgefäßen bei kutanem Plattenepithelkarzinom.

Kutane Plattenepithelkarzinome (SCC) sind bekannt für ihre Fähigkeit, über Lymphgefäße zu metastasieren. In neueren Studien wird das Ausmaß der Lymphangiogenese als möglicher prognostischer Faktor bei einigen Hauttumoren genannt. Ziel dieser Studie war die Quantifizierung der Lymphangiogenese bei SCC entweder durch computergestützte Bildanalyse oder mithilfe der Zählmethode nach Chalkley. Gefäßparameter wurden im Hinblick auf ihre Vorhersagekraft für die Bildung von Tumormetastasen beurteilt und verglichen.

Prognostic value of the area and density of lymphatic vessels in cutaneous squamous cell carcinoma.

Cutaneous squamous cell carcinoma (SCC) is known for its capacity to metastasize via lymphatic vessels. In recent studies, the level of lymphangiogenesis has been reported as a potential prognostic factor for several skin tumors. The aim of this study was to quantify lymphangiogenesis in SCC using either computer‐assisted image analysis or the Chalkley count technique. Vascular parameters were evaluated and compared with respect to their predictive power for tumor metastasis.