Linkage between patients' characteristics and prescribed systemic treatments for psoriasis: a semantic connectivity map analysis of the Swiss Dermatology Network for Targeted Therapies registry.

BACKGROUND: Several treatment options are currently available for the treatment of psoriasis. OBJECTIVE: To explore the main associations between patients' characteristics and systemic treatments prescribed for psoriasis in a large group of patients observed in real-life clinical practice. METHODS: This was a retrospective analysis of baseline data collected within the Swiss Dermatology Network for Targeted Therapies registry in Switzerland between March 2011 and December 2017. Semantic map analysis was used in order to capture the best associations between variables taking into account other covariates in the system. RESULTS: A total of 549 patients (mean age 46.7 ± 14.7 years) were included in the analysis. Conventional therapies such as retinoids and methotrexate were associated with no previous systemic therapies for psoriasis, a moderate quality of life (QoL) at therapy onset and older age (≥60 years). Fumaric acid derivatives were associated with mild psoriasis (psoriasis area severity index < 10) and long disease duration (≥20 years). On the other side, cyclosporine and psoralen and ultraviolet A/ultraviolet B treatments were linked to a more severe condition, including impaired QoL, hospitalization and inability to work. Regarding biological therapies, both infliximab and adalimumab were connected to the presence of psoriatic arthritis, severe disease condition and other comorbidities, including chronic liver or kidney diseases and tuberculosis. Etanercept, ustekinumab and secukinumab were all connected to a complex history of previous systemic treatments for psoriasis, moderate disease condition, overweight and university education. CONCLUSIONS: The analysis shows multifaceted associations between patients' characteristics, comorbidities, disease severity and systemic treatments prescribed for psoriasis. In particular, our semantic map indicates that comorbidities play a central role in decision-making of systemic treatments usage for psoriasis. Future studies should further investigate specific connections emerging from our data.

Gender and age significantly determine patient needs and treatment goals in psoriasis - a lesson for practice.

BACKGROUND: Though patient needs are key drivers of treatment decisions, they are rarely systematically investigated in routine care. OBJECTIVE: This study aimed at analysing needs and expectations from the patient perspective in the German and Swiss psoriasis registries PsoBest and Swiss Dermatology Network of Targeted Therapies (SDNTT) with respect to treatment choice, age and gender. METHODS: The German and Swiss psoriasis registries observe patients recruited at first-time use of systemic drugs. Within 10 years, clinical [Psoriasis Area Severity Index (PASI), Body Surface Area (BSA)] and patient-reported outcomes are documented, including the Dermatology Quality of Life Index (DLQI) and the Patient Benefit Index (PBI), characterizing patient needs for treatment. The analysis data set includes n = 4894 patients from PsoBest and n = 449 from SDNTT with mean follow-up time of 7.5 months. RESULTS: A total of 5343 patients registered between 2008 and 2016 were included in the analyses (at baseline: 59.6% male, mean age 47.6 years ± 14.5, PASI 14.2 ± 9.7, BSA 22.7 ± 19.7, DLQI 11.3 ± 7.2). The most important patient needs were to 'get better skin quickly' and to 'be healed of all skin defects'. Subgroup analyses by age revealed significant differences in needs, especially higher needs regarding social impairments in patients younger than 65 years. Patients 65 years or older attributed more importance to sleep quality, less dependency on medical visits, fewer side-effects and confidence in the therapy. Out of 25 items reflecting patient needs, 20 items were rated significantly more important by women than men, with the greatest differences regarding feeling of depression, sleep quality and everyday productivity. Divided by treatment, needs were rated differently, recommending individualized and targeted choice of therapy. CONCLUSION: Age and gender stratify patient needs. Women showed higher expectations and rated specific needs in psoriasis treatment higher than men. Analysing the patient needs on an individual level will facilitate shared decisions by patient and physician in finding the optimal personalized treatment.

Swiss S1 guideline for the treatment of rosacea.

Rosacea (in German sometimes called 'Kupferfinne', in French 'Couperose' and in Italian 'Copparosa') is a chronic and frequently relapsing inflammatory skin disease primarily affecting the central areas of the face. Its geographic prevalence varies from 1% to 22%. The differential diagnosis is wide, and the treatment is sometimes difficult and varies by stage of rosacea. For erythematous lesions and telangiectasia, intense pulsed light (IPL) therapy and lasers are popular treatment option. In addition, a vasoconstrictor agent, brimonidine, has recently been developed. For papulopustular rosacea, topical antibiotics, topical and systemic retinoids, as well as systemic antibiotics are used. A topical acaricidal agent, ivermectin, has undergone clinical development and is now on the market. In the later stages, hyperplasia of the sebaceous glands develops, resulting in phymatous growths such as the frequently observed bulbous nose or rhinophyma. Ablative laser treatments have largely replaced classical abrasive tools. Here, we reviewed the current evidence on the treatment of rosacea, provide a guideline (S1 level) and discuss the differential diagnosis of rosacea.

Genetic susceptibility to cutaneous melanoma in southern Switzerland: role of CDKN2A, MC1R and MITF.

BACKGROUND: Nearly 10% of all cases of cutaneous melanoma (CM) occur in patients with a personal or family history of the disease. OBJECTIVES: To obtain information about genetic predisposition to CM in Ticino, the southern region of Switzerland, a zone with moderate-to-high CM incidence. METHODS: We identified germline mutations in highly CM-associated genes (CDKN2A and CDK4) and low/medium-penetrance variants (MC1R and MITF) in patients with multiple primary CMs or individuals with one or more CM and a positive family history for CM or pancreatic cancer among first- or second-degree relatives. Healthy blood donors (n = 146) were included as a control group. RESULTS: From July 2010 to July 2012, 57 patients (41 pedigrees) were included. Twenty-six were melanoma-prone families (with at least two cases) and 15 had multiple CMs. Pancreatic cancer was found in six families. The CDKN2A mutation p.V126D was identified in seven patients (four families) with a founder effect, whereas CDKN2A A148T was detected in seven cases (five families) and seven healthy donors (odds ratio 2·76, 95% confidence interval 0·83-9·20). At least one MC1R melanoma-associated polymorphism was detected in 32 patients (78%) and 97 healthy donors (66%), with more than one polymorphism in 12 patients (29%) and 25 healthy donors (17%). The MITF variant p.E318K was identified in four patients from three additional pedigrees (7%) and one healthy control (0·7%). CONCLUSIONS: Inclusion criteria for the Ticino population for genetic assessment should follow the rule of two (two affected individuals in a family or a patient with multiple CMs), as we detected a CDKN2A mutation in almost 10% of our pedigrees (four of 41), MITF p.E318K in 7% (three of 41) and a higher number of MC1R variants than in the control population.

The clinical spectrum of syphilitic balanitis of Follmann: report of five cases and a review of the literature.

BACKGROUND: Eugène Follmann first described syphilitic balanitis as a manifestation of primary syphilis in 1948 and since then it has been known as syphilitic balanitis of Follmann (SBF). So far, SBF has rarely been described in literature. OBJECTIVES: We are reporting five additional cases of SBF considering the broad differential diagnosis of balanitis. METHODS: A review of the available literature of SBF was performed and five additional cases analyzed. RESULTS: In our case series, the clinical appearance of SBF shows a heterogeneous spectrum varying from painful oedematous balanoposthitis with beginning paraphimosis to superficial erosive balanitis and even to painless induration of the glans. CONCLUSIONS: SBF might be seen more frequently than has been described due to misinterpretation. Therefore primary syphilis should be included in the differential diagnosis of balanitis and balanoposthitis.

Fumaric acid esters in psoriasis and multiple sclerosis.

Fumaric acid esters (FAEs) are effective in patients with moderate to severe psoriasis. Recent studies also report the efficacy of one FAE component, dimethylfumarate, in relapsing forms of multiple sclerosis (MS). We describe the case of a patient with MS who developed severe plaque psoriasis during interferon-ß-1a treatment for MS. The psoriasis was unresponsive to usual topical treatments and phototherapy. The patient was started on FAE 720 mg daily, with complete remission of the psoriatic lesions and neurological stabilization at follow-up at 24 months. Our case suggests that FAEs could represent a therapeutic option for patients with MS who develop plaque psoriasis following exposure to immune-modulating agents.

Diagnostic pitfall: pigmented lesion of the nipple--correlation between dermoscopy, reflectance confocal microscopy and histopathology.

We present an unusual case of a nevus of the nipple changing during pregnancy which caused a diagnostic pitfall. Nevi on the nipple and areola are infrequent, and diagnostic criteria for clinical, dermoscopy or reflectance confocal microscopy examination for nevi in this 'special location' are still missing. We comment on the literature on dermoscopic findings in mammary lesions and their management during pregnancy, as well as the challenging histopathology of nevi along the milk line. Finally, we focus on two main limitations of reflectance confocal microscopy: the misinterpretation of dendritic cells and the limitation of the imaging depth.

Precalcaneal congenital fibrolipomatous hamartomas: report of 3 additional cases and discussion of the differential diagnosis.

We report 3 cases of otherwise healthy infants with nodules on their soles. The nodules were present at birth or developed in early childhood. This condition has first been described in 1977 and was termed precalcaneal congenital fibrolipomatous hamartoma (PCFH). Since then the same entity has been reported under various denominations. The incidence of PCFH is underestimated, and it is important to recognize the benign nature of this disorder.

Eradication of Borrelia burgdorferi infection in primary marginal zone B-cell lymphoma of the skin.

Primary cutaneous B-cell lymphomas have been associated with Borrelia burgdorferi, the spirochete responsible for Lyme disease. Recently, cutaneous marginal zone B-cell lymphoma has been proposed as a distinct clinical-pathological entity. We report a case of primary cutaneous marginal zone lymphoma, associated with B burgdorferi infection. Polymerase chain reaction (PCR) amplification of the third complementarity determining region (CDR3) of the immunoglobulin heavy chain gene showed the presence of a monoclonal lymphoproliferation, therefore strengthening the histological diagnosis of a malignant process. B burgdorfer-specific hbb gene sequences were detected by PCR in the lymphoma tissue at diagnosis but not after antibiotic treatment. A nearly complete clinical and histological regression was observed after B burgdorferi eradication, with immunohistochemistry studies showing disappearance of plasma cell differentiation and a marked decline in the number of CD3+ T cells and Ki-67+ cells. Our case confirms the link between B burgdorferi and some cutaneous lymphomas. The disappearance of the microorganism accompanied by the unequivocal decrease of most indicators of active T- and B-cell immune response strongly supported a pathogenetic role for B burgdorferi in sustaining an antigen-driven development and growth of this cutaneous marginal zone lymphoma. Antibiotic therapy (analogous to Helicobacter pylori infection in gastric MALT lymphoma) might be helpful with the aim of averting or at least deferring the indication for more aggressive treatment.

[Solid persistent facial edema of the young adult. Melkersson-Rosenthal syndrome]. / Oedème facial solide persistant du sujet jeune. Syndrome de Melkersson-Rosenthal.

We report a case of solid persistent facial oedema in a 18-year-old woman. A biopsy specimen from the nose demonstrating lympho-epithelioid granulomas and an electro-neuro-myographic examination revealing sub-clinical sequellae from an old right facial palsy, led to the definite diagnosis of Melkersson-Rosenthal syndrome (MRS). We focus on the differential diagnosis of persistent facial oedema and outline the difficulties to establish the diagnosis of MRS when all features of the classic triad "recurrent labial edema-facial nerve palsy-lingua plicata" are not present.

[Cutaneous manifestations of tuberculosis]. / Manifestations cutanées de la tuberculose.

Tuberculous lesions of the skin occur rarely nowadays. We therefore have mostly lost the knowledge to recognize this entity. Furthermore, the great variability of the clinical and particularly dermatologic manifestations of the disease can tax the most astute clinician. The challenge is even greater, when the patient has an intercurrent condition such as a malignancy or AIDS. In order to make understanding easier, we replace all descriptive terms, some carried over from the last century, by a useful pathogenetic and clinical, algorithmic classification. Finally the necessity of biopsy and specific cultures for proper diagnosis and treatment of any skin lesion consistent with skin tuberculosis is emphasized.

L-tryptophan-induced eosinophilia-myalgia syndrome. II. Partial correction of abnormal tryptophan metabolism by pyridoxine.

Abnormal metabolism of tryptophan is one of the possible aetiological factors in the L-tryptophan-induced eosinophilia-myalgia syndrome (EMS). We studied the plasma levels of tryptophan and serotonin and the urinary excretion of kynurenine and 5-hydroxyindoleacetic acid after oral intake of L-tryptophan in 1 subject with EMS and 2 healthy subjects. The test was repeated with concomitant administration of pyridoxine. In the patient there were elevated levels of plasma tryptophan during the loading and increased elimination of kynurenine in the urine both during and after the L-tryptophan test. During pyridoxine administration tryptophan levels and kynurenine elimination were much reduced, and kynurenine elimination was similar to that of controls. This study (i) confirms that an abnormal metabolism of L-tryptophan occurs in EMS patients and (ii) shows that this can be corrected by pyridoxine.

Hip surgery skin cellulitis.

Of 583 erysipelas/cellulitis seen in our Department between 1981 and 1991, six (1.04%) were localized to the buttock and hip. Five patients had had a dynamic hip screw implanted for coxarthritis on the side where the skin infection developed. This does not appear to be secondary to superinfection of the surgical wound as the erysipelas/cellulitis occurred weeks (7 to 520) after surgery. The possible surgical compromise of the venous/lymphatic circulation appears to be the causative factor. In all patients, the course was satisfactory upon systemic antibiotic therapy and no recurrence was seen after a mean follow up of 28 months.

Classical (HIV-negative) cutaneous Kaposi's sarcoma: a case report and a short review of the literature.

Classical Kaposi's sarcoma is a sporadic disease with a markedly higher incidence in the Mediterranean area. The two original forms of this disease, the Caucasian (classical) and the African, are now well-documented entities. The immunosuppression-related form is a new, recently described subset of the disease, arising from the human immune deficiency virus (HIV) infection and/or the widespread use of immunosuppressive treatments. Cutaneous lesions are the most common manifestation of classical Kaposi's sarcoma. The case described in the present report is characterised by a history of slowly evolving disease affecting the lower extremities without spread to thoracic or abdominal organs, a lengthy remission following initial radiation therapy, and local relapse with a further remission after a second course of radiation. Current treatments, in particular radiation therapy, achieve disease control or at least control of disease-related symptoms (mainly pain) in most cases, even when cure is not possible.

L-tryptophan-induced eosinophilia-myalgia syndrome. I. Report of two cases with pseudoxanthoma-elasticum-like skin changes.

Two female patients who fulfilled the criteria for L-tryptophan-induced eosinophilia-myalgia syndrome (EMS) had, together with morphea-like and fasciitis-like sclerotic changes of the skin, lesions that clinically mimicked pseudoxanthoma elasticum (PXE). Histology was compatible with the diagnosis; electron microscopy did not reveal calcium deposits. PXE-like changes may represent an additional feature of the pleomorphic L-tryptophan-induced EMS.

Localized childhood vulval pemphigoid treated with tacrolimus ointment.

BACKGROUND: Localized vulval childhood pemphigoid is a rare variant within the pemphigoid group. Although its prognosis seems favorable, the best therapeutic strategy remains unclear. OBSERVATION: We here describe the case of an 8-year-old girl presenting with a 5-year history of relapsing vulval pain and lesions suggestive of lichen sclerosus. Clinical features, light microscopy and direct immunofluorescence microscopy were consistent with vulval cicatricial pemphigoid, although the autoantigen(s) involved could not be characterized. Her disease responded to treatment with topical tacrolimus ointment 0.1% within 3 months without any evidence for disease activity, except for slight residual scarring. After 12 months, her treatment was stopped without relapse. CONCLUSION: This observation suggests that in this rare immune-mediated blistering disease topical tacrolimus is an interesting therapeutic option without the adverse effects associated with topical steroids.

L-tryptophan-induced eosinophilia-myalgia syndrome associated with primary cutaneous malignant fibrous histiocytoma and extraabdominal desmoid tumor.

A 57-year-old woman with L-tryptophan-induced eosinophilia-myalgia syndrome, 23 months after the onset of symptoms, experienced development of a parasternal malignant fibrous histiocytoma on previously scleroderma-like skin areas and, almost concurrently, an extraabdominal desmoid tumor of the left arm muscle fascia. The malignant fibrous histiocytoma was treated by surgical excision without sign of recurrence or metastasis 19 months later. Radiation therapy was performed on the extraabdominal desmoid tumor. We suspect that these two connective tissue tumors in this patient were related to the exposure to contaminated L-tryptophan, which interfered with connective tissue metabolism.

Topical haemotherapy for leg ulcers.

Several growth factors, present in the plasma or delivered by blood cells, are involved in wound healing. We have treated 15 chronic atonic leg ulcers by topical application of autologous heparinized blood under a hydrocolloidal dressing. This 'topical haemotherapy' (THT) was applied every other day. After the first THT, 30% of the fibrinonecrotic material had already been removed. After a mean of 9 THT applications (range: 5-18), granulation tissue covered up to 75% of the surface of the ulcer (range: 30-100%) allowing autologous skin grafting. No local or systemic side-effects occurred during the treatment. THT is feasible and well tolerated; it is an easy and inexpensive treatment of chronic leg ulcers that rapidly induces granulation tissue. Growth factors and proteases produced by the blood cells could play a relevant role in this process.

Pulse of methylprednisolone in alopecia areata.

In an attempt to stop the evolution of recent-onset severe alopecia areata (AA), we tested pulse corticotherapy on 9 patients. Acceptance into the study was based on the following criteria: recent-onset AA (< 1 year), AA in an active state, bald surface > 30% of the scalp, no contraindication to pulse corticotherapy. Each patient was given 250 mg i.v. of methylprednisolone twice a day on 3 successive days. In 8 patients the course of the ongoing episode of AA was stopped. At the 6-month follow-up, a regrowth on 80-100% of the bald surface was observed in 6 patients. One patient did not respond to treatment, and 2 had less than 50% of regrowth. This open study suggests that pulse corticotherapy: (1) can stop the course of severe AA in an active state, (2) is well tolerated without major side effects and (3) does not permit a stable control of AA of more than 1 year duration. This treatment seems to be indicated for severe AA of recent onset.