Inter- and Intra-rater Agreement of Dermatologic Ultrasound for the Diagnosis of Lobular and Septal Panniculitis.

OBJECTIVES: Dermatologic ultrasound (US) may aid in the diagnosis and classification of panniculitis. The purpose of this study was to assess the capability of dermatologic US for subtyping mainly septal/lobular panniculitis. METHODS: A multicentric and prospective study of the inter- and intra-rater agreement of dermatologic US for subtyping panniculitis was conducted among 4 clinicians with experience in dermatologic US and a radiologist specialized in dermatologic US. Clinicians recruited patients and performed dermatologic US examinations of the most substantial lesion and punch biopsies. A histologic study was considered the reference standard. Then the images were blindly evaluated by all researchers. For intra- and inter-rater agreement, Cohen and Fleiss κ values were calculated. RESULTS: Sixty-four patients were included. The Cohen intra-rater κ was 0.74. Sensitivity and specificity for lobular panniculitis were 85.19 and 88.57, respectively. The Fleiss inter-rater κ was 0.47. Limitations of the study included the small number of patients and differences in evaluators and their dermatologic US equipment. CONCLUSIONS: This study supports the use of US for diagnosing panniculitis. For subtyping panniculitis, the intra-rater correlation was good. Improvement of inter-rater agreement may depend on access to clinical information, dynamic images, a better definition of criteria, homogeneous configurations of the devices, and the expertise of dermatologic US operators.

Ultrasound Assessment of Psoriatic Onychopathy: A Cross-sectional Study Comparing Psoriatic Onychopathy with Onychomycosis.

This cross-sectional study evaluated the usefulness of an ultrasound technique in assessment of nail changes in 35 patients with psoriatic onychopathy and 25 with nail dystrophy secondary to onychomycosis. All patients underwent 3 examinations: a complete clinical assessment; a nail ultrasound study; and fungal culture. Nails of patients with psoriatic onychopathy presented a thinner nail plate and nail bed, measured by ultrasound, than did those with onychomycosis. The percentage of patients with a power Doppler signal ?2 at nail bed was significantly higher in psoriatic onychopathy than in onychomycosis, and structural bone lesions were more frequent in psoriatic onychopathy than in onychomycosis. These results suggest that the presence of structural damage and high-power Doppler signal are the main ultrasound findings supporting a diagnosis of psoriatic onychopathy.

Pleomorphic mastocytoma in an adult.

Cutaneous mastocytoma (CM) is a localized variant of mastocytosis, characterized by an over-accumulation of mast cells in the skin, without extra-cutaneous organ involvement. It is defined as the presence of up to 3 isolated mast-cell skin lesions and commonly develops in newborns and children. We report the case of a 35-year-old healthy Caucasian woman presenting with a 4-year history of a pruritic brown plaque on her left breast. Hematoxylin-eosin staining showed a dense dermal infiltrate of atypical mast cells extending to the subcutis. The cells presented a marked nuclear pleomorphism with bilobed and multilobed nuclei. Immunohistochemical studies revealed strongly expressed KIT (CD117) and CD25 proteins. Serum tryptase levels and bone marrow biopsy were normal. The diagnosis was a solitary cutaneous pleomorphic mastocytoma. This case can be added to 17 other cases of adult mastocytoma documented in the literature, although, unlike other reported cases, and as far as we are aware, this is the first case of pleomorphic mastocytoma in an adult.

Standards of care and quality indicators for multidisciplinary care models for psoriatic arthritis in Spain.

To define and give priority to standards of care and quality indicators of multidisciplinary care for patients with psoriatic arthritis (PsA). A systematic literature review on PsA standards of care and quality indicators was performed. An expert panel of rheumatologists and dermatologists who provide multidisciplinary care was established. In a consensus meeting group, the experts discussed and developed the standards of care and quality indicators and graded their priority, agreement and also the feasibility (only for quality indicators) following qualitative methodology and a Delphi process. Afterwards, these results were discussed with 2 focus groups, 1 with patients, another with health managers. A descriptive analysis is presented. We obtained 25 standards of care (9 of structure, 9 of process, 7 of results) and 24 quality indicators (2 of structure, 5 of process, 17 of results). Standards of care include relevant aspects in the multidisciplinary care of PsA patients like an appropriate physical infrastructure and technical equipment, the access to nursing care, labs and imaging techniques, other health professionals and treatments, or the development of care plans. Regarding quality indicators, the definition of multidisciplinary care model objectives and referral criteria, the establishment of responsibilities and coordination among professionals and the active evaluation of patients and data collection were given a high priority. Patients considered all of them as important. This set of standards of care and quality indicators for the multidisciplinary care of patients with PsA should help improve quality of care in these patients.

Identification and management of comorbidity in psoriatic arthritis: evidence- and expert-based recommendations from a multidisciplinary panel from Spain.

The objective is to establish recommendations, based on evidence and expert opinion, for the identification and management of comorbidities in patients with psoriatic arthritis (PsA). The following techniques were applied: discussion group, systematic review, and Delphi survey for agreement. A panel of professionals from four specialties defined the users, the sections of the document, possible recommendations, and what systematic reviews should be performed. A second discussion was held with the results of the systematic reviews. Recommendations were formulated in the second meeting and voted online from 1 (total disagreement) to 10 (total agreement). Agreement was considered if at least 70% voted ≥7. The level of evidence and grade of recommendation were assigned using the Oxford Centre for Evidence-Based Medicine guidance. The full document was critically appraised by the experts, and the project was supervised at all times by a methodologist. In a final step, the document was reviewed and commented by a patient and a health management specialist. Fourteen recommendations were produced, together with a checklist to facilitate the implementation. The items with the largest support from evidence were those related to cardiovascular disease and risk factors. The panel recommends paying special attention to obesity, smoking, and alcohol consumption, as they are all modifiable factors with an impact on treatment response or complications of PsA. Psychological and organizational aspects were also deemed important. We herein suggest practical recommendations for the management of comorbidities in PsA based on evidence and expert opinion.

Photodynamic therapy with intralesional methylene blue and a 635 nm light-emitting diode lamp in hidradenitis suppurativa: a retrospective follow-up study in 7 patients and a review of the literature.

Hidradenitis suppurativa is a chronic inflammatory skin disease which has an estimated prevalence of 1%. It is characterized by the formation of recurrent painful suppurative nodules and abscesses in the flexural areas of the body. It is believed that its pathogenesis involves an aberrant, genetically-determined activation of innate immunity against the bacterial commensal flora of intertriginous areas. It has been found that the formation of antibiotic-resistant bacterial biofilms is a common finding in hidradenitis lesions. Photodynamic therapy with different compounds and light sources has demonstrated its efficacy in a number of infectious diseases such as nail mycosis and chronic periodontitis. We retrospectively report our experience in the treatment of hidradenitis with photodynamic therapy using intralesional methylene blue and a 635 nm light-emitting diode lamp in 7 patients. Two patients received one session whereas 5 patients received two sessions. At one month follow-up good response was achieved in 6 patients. After 6 months, 5 patients (71%) maintained remission of the disease in the treated area. In view of the results and literature review, we regard methylene blue as an ideal photosensitizer for photodynamic therapy in this disease.

Toll-like receptor 9 promoter polymorphism as a predictive factor of narrow-band UVB phototherapy response in patients with psoriasis.

BACKGROUND: Prediction of response to ultraviolet B (UVB) phototherapy in psoriatic patients mainly relies on clinical criteria, although some genetic predictors have been identified. Toll-like receptors (TLRs) have been involved in psoriasis pathogenesis through activation of the innate immune system. Their polymorphisms may condition not only the clinical profile of psoriasis but also the response to therapy. METHODS: We analyzed the role of functional single-nucleotide polymorphisms (SNPs) of TLR2, 5, 4, and 9 in clinical response to a standard narrow-band UVB (NBUVB) therapy in 39 patients with moderate to severe psoriasis. RESULTS: We found a significant relationship between TLR9-1486T/C SNP variants and a better response to NBUVB phototherapy. Patients with TC and CC genotype showed a higher improvement of Psoriasis Area and Severity Index (PASI) than patients with TT genotype. Results of multivariate analysis indicate that the differences in PASI improvement at the end of phototherapy attributed to TRL9 SNP genotype were not dependent on the patients' phototype, age, gender, body mass index, basal PASI, or disease evolution. CONCLUSIONS: We describe a functional genetic variant in TLR9 gene that might affect the susceptibility to antipsoriatic treatment. The search of genetic predictive factors may be helpful in therapy selection and optimization of therapeutic regimes in psoriatic patients.

Assessment of cardiovascular risk in patients with moderate to severe plaque psoriasis.

BACKGROUND: Cardiovascular morbidity and mortality have been demonstrated to be greater in psoriasis patients than in the general population. Our study aimed to assess the 10-year cardiovascular risk in patients with moderate to severe psoriasis compared with those suffering from other dermatological diseases, using the calibrated Framingham risk score and the Systematic Coronary Risk Evaluation (SCORE) risk charts. METHODS: A cross-sectional, multicentre study was made of 477 patients, of whom 238 had moderate to severe psoriasis (cases) and 239 were diagnosed with another dermatological disease (controls). RESULTS: The proportion of patients with intermediate to high 10-year cardiovascular risk using the Framingham equation was significantly higher among psoriasis patients (38.5%; 80/208) than among the controls with other dermatological diseases (23.4%; 50/214, P<.05). No significant differences were observed between the 2 groups with respect to cardiovascular risk using the SCORE risk charts (P=.591). The case group included a greater proportion of obese and morbidly obese patients, as well as patients with higher triglyceride and low density lipoprotein cholesterol levels (P<.05); while high density lipoprotein cholesterol levels were significantly more favorable in patients in the control group (P<.05). CONCLUSIONS: Cardiovascular risk was greater in patients with moderate to severe psoriasis than in patients with other dermatological conditions, suggesting that early detection and tailored management of risk factors is essential to reducing cardiovascular morbidity in these patients.

Refractory childhood pemphigoid successfully treated with rituximab.

Bullous pemphigoid is an acquired autoimmune blistering disorder affecting mostly elderly people that is rare in children. There have been fewer than 100 cases of childhood bullous pemphigoid reported. Childhood bullous pemphigoid (CBP) generally has a good prognosis. Remission is usually achieved within several weeks to a few months, and relapses are rare, although refractory cases can occur, and the disease may be life threatening, particularly when lesions generalize. Herein we describe the case of an infant with severe CBP refractory to multiple treatments. In our case, therapy with azathioprine and intravenous immunoglobulin resulted in a slight clinical response, but only combined with high doses of prednisolone. Administration of rituximab led to a complete clinical response and allowed almost complete steroid tapering. There are no current guidelines indicating what doses and frequency of rituximab are safest or most effective in children with blistering diseases.

Localized xanthomas associated with primary lymphedema.

Cutaneous xanthomas arising in chronic lymphedema are rare. We present a case of verruciform xanthoma involving the left foot and toes of a 10-year-old boy who had developed a primary lymphedema (lymphedema praecox) in the left lower extremity. Laboratory studies demonstrated a normal lipid profile.

A 12-point recommendation framework to support advancement of the multidisciplinary care of psoriatic arthritis: A call to action.

OBJECTIVE: Making a differential diagnosis of psoriatic arthritis (PsA) is not straightforward. This is partly because of its heterogeneous presentation and partly because many patients with PsA are initially diagnosed with psoriasis and treated in primary care or by dermatologists, with referral to rheumatologists being delayed. Once diagnosed, optimal disease control requires frequent specialist monitoring, adjustment or switching of therapies, and management of comorbidities and concomitant diseases, as well as attention to patients' overall well-being. Given the breadth of expertise that diagnosis and management of PsA requires, we sought to define a collaborative, structured framework that supports the optimisation of multidisciplinary care for patients with PsA in Europe. METHODS: An expert panel comprising four rheumatologists, three dermatologists, two specialist nurses and one psychologist-from Spain, the United Kingdom, The Netherlands, Germany, France and Italy-met face-to-face to take part in a modified Delphi exercise. RESULTS: The result of this exercise is a set of recommendations that are based on combining published evidence with the panel's extensive clinical experience. Recommendations can be implemented in a number of ways, but the central call-to-action of this framework is the need for improved collaboration between dermatologists (or primary care physicians) and rheumatologists. This could occur in a variety of different formats: standard referral pathways, multidisciplinary physician meetings to discuss patient cases, or 'one stop', combined clinics. CONCLUSION: We anticipate that when the majority of patients with PsA receive regular multidisciplinary care, improved patient outcomes will follow, although robust research is needed to explore this assumption.

Current status of multidisciplinary care in psoriatic arthritis in Spain: NEXUS 2.0 project. / Estado actual de la atención multidisciplinar para pacientes con artritis psoriásica en España: proyecto NEXUS 2.0.

OBJECTIVE: 1) To analyze the implementation of multidisciplinary care models in psoriatic arthritis (PsA) patients, 2) To define minimum and excellent standards of care. METHODS: A survey was sent to clinicians who already performed multidisciplinary care or were in the process of undertaking it, asking: 1) Type of multidisciplinary care model implemented; 2) Degree, priority and feasibility of the implementation of quality standards in the structure, process and result for care. In 6 regional meetings the results of the survey were presented and discussed, and the ultimate priority of quality standards for care was defined. At a nominal meeting group, 11 experts (rheumatologists and dermatologists) analyzed the results of the survey and the regional meetings. With this information, they defined which standards of care are currently considered as minimum and which are excellent. RESULTS: The simultaneous and parallel models of multidisciplinary care are those most widely implemented, but the implementation of quality standards is highly variable. In terms of structure it ranges from 22% to 74%, in those related to process from 17% to 54% and in the results from 2% to 28%. Of the 25 original quality standards for care, 9 were considered only minimum, 4 were excellent and 12 defined criteria for minimum level and others for excellence. CONCLUSIONS: The definition of minimum and excellent quality standards for care will help achieve the goal of multidisciplinary care for patients with PAs, which is the best healthcare possible.

Hidradenitis suppurativa of the nape: Description of an atypical phenotype related to severe early-onset disease in men.

Some patients with hidradenitis suppurativa (HS) develop severe inflammatory lesions of the nape. Through a single-center, cross-sectional study with a total of 377 patients, we sought to compare patients with and without nape involvement, to determine whether disease severity is greater in these patients and to describe their clinical characteristics. Thirty patients (90% male) were identified as having nape involvement. Patients with versus without nape involvement had greater disease severity, earlier disease onset, a family history of HS, lower body mass, and higher Dermatological Life Quality Index and pain scores. Amongst them, involvement of the trunk and gluteal regions predominated. A significant univariate relationship existed between patients with nape involvement and male sex, early disease onset, family history, scalp and gluteal involvement, Canoui-Poitrine phenotypes II and III, and Dowling-Degos disease. A significant multivariate relationship existed with early disease onset, Dowling-Degos disease, and phenotypes II and III.

Navel melanoma: not always easy to detect, not always difficult to remove.

Melanoma diagnosis can be delayed when the tumor is present in areas that are poorly visualized. We present a peri-umbilical melanoma and discuss the use of Y-plasty in this area.

Multidisciplinary Care Models for Patients With Psoriatic Arthritis. / Modelos de atención multidisciplinar en pacientes con artritis psoriásica en España.

OBJETIVE: To describe (structure, processes) of the multidisciplinary care models in psoriatic arthritis (PsA) in Spain, as well as barriers and facilitators of their implementation. METHODS: A qualitative study was performed following structured interviews with 24 professionals (12 rheumatologists, 12 dermatologists who provide multidisciplinary care for patients with PsA). We collected data related to the hospital, department, population and multidisciplinary care model (type, physical and human resources, professional requirements, objectives, referral criteria, agendas, protocols, responsibilities, decision- making, research and education, clinical sessions, development and planning of the model, advantages and disadvantages of the model, barriers and facilitators in the implementation of the model. The models characteristics are described. RESULTS: We analyzed 12 multidisciplinary care models in PsA, with at least 1-2 years of experience, and 3 subtypes of models, face-to-face, parallel, and preferential circuit. All are adapted to the hospital and professionals characteristics. A proper implementation planning is essential. The involvement and empathy between professionals and an access and well-defined referral criteria are important facilitators in the implementation of a model. The management of agendas and data collection to measure the multidisciplinary care models health outcomes are the main barriers. CONCLUSIONS: There are different multidisciplinary care models in PsA that can improve patient outcomes, system efficiency and collaboration between specialists.

Post-infectious acute renal failure due to calciphylaxis--when processes go the wrong way round.

Calciphylaxis is a rare life threatening disorder of small- and medium-sized vessel calcification that leads to cutaneous necrosis. While its pathogenesis is uncertain, nearly all cases have been described in patients with end-stage renal disease (ESRD) on dialysis or following renal transplantation which is why the lesion has also been referred to as calcific uremic arteriolopathy. We describe a patient with alcoholic cirrhosis and normal renal function who developed calciphylaxis. Due to infected cutaneous lesions, he developed an acute post-infectious glomerulonephritis with extra capillary proliferation.