Xavier Fustà-Novell

Clinical and photobiological response in eight patients with solar urticaria under treatment with omalizumab, and review of the literature

Solar urticaria (SU) is a rare photodermatosis. Treatment is challenging, and outcomes are often disappointing. Omalizumab is an anti‐IgE, currently approved for treatment of chronic spontaneous urticaria. We sought to evaluate therapy with omalizumab in refractory SU and describe predictive factors for response.

Dermographism in a patient with fever

A woman in her 30s presented with a 2-week history of high spiking fever (>39 °C), arthralgia, and sore throat. She reported that recurrent transient erythematous linear lesions were associated with her systemic symptoms. These lesions appeared at scratching sites and faded within hours. She did not recall having similar eruptions in the past. Physical examination revealed linear erythematous urticarial lesions on the trunk and upper arms (Fig. 1). Generalized pathological lymph nodes and hepatosplenomegaly were also found.

Rituximab for the treatment of autoimmune subepidermal blistering diseases

Dear Editor, Conventional therapy for autoimmune subepidermal blistering diseases (ASBD) includes topical or systemic corticosteroids (SC) and adjuvants such as immunosuppressive agents (ISA), dapsone, plasma exchange, or tetracyclines. Experience regarding rituximab (RTX) in blistering diseases mainly comes from pemphigus, where it is highly effective. RTX is usually reserved to treat ASBD refractory to conventional therapy. Evidence about its efficacy in ASBD is mainly documented in case series and case reports (Cho, Chu, & Wang, 2015, Maley et al., 2016). The aim of this manuscript was to evaluate RTX therapy in refractory ASBD. Retrospective cohort of 10 patients treated with RTX for ASBD at a single tertiary referral center between March 2007 and April 2017. Clinical outcomes were defined following the international consensus criteria (Murrell et al., 2012, 2015) NC16-BP180 levels and CD20+ B-cells were measured in all patients before RTX administration. Two patients with bullous pemphigoid (BP), 1 chronic pemphigoid gestationis (CPG), 5 mucous membrane pemphigoid (MMP), and 2 epidermolysis bullosa acquisita (EBA) were included. Median age was 60.5 (interquartile range [IQR], 50–71) years. Clinical manifestations, previous and concomitant treatments, RTX dosages, follow-up, and outcomes are detailed in Table 1. All patients were unresponsive to SC and to at least one systemic adjuvant agent before introducing RTX. After a median follow up of 20.5 (IQR 9–48) months, the 2 patients with BP were in complete remission off treatment (CR-off ), and the patient with CPG did not respond. After treatment, BP180 antibodies were undetectable in patients with both BP and CPG. Of the 5 patients with MMP, 4 had favorable outcomes: 2 patients were in CR, 1 of them on treatment (CR-on), and 2 patients presented partial remission on treatment (PR-on). One patient did not respond. Both patients with EBA remained with a PR-on. Scarring, blindness or severe strictures were not observed after RTX treatment. No patient within the cohort achieved CR-off after a single RTX cycle. The 2 patients with sustained CR-off, presented disease flare-ups at 6 months (patient 4, MMP) and 12 months (patient 1, BP) after the first cycle. They received additional doses of RTX, requiring no further treatment. No adverse reactions or deaths were observed. Our findings, consistent with those reported in the literature, show an overall improvement in most patients with ASBD treated with RTX, and a better response to this agent in BP than in MMP or EBA. Although RTX did not lead to long-term remission in all patients with MMP or EBA, it appears to be beneficial in stabilizing and preventing progression and therefore, secondary scarring associated with these diseases as previously reported (Shetty & Ahmed, 2013). Once inflammation is under control after receiving RTX, patients could continue with conventional therapy or RTX as a maintenance regimen (Heelan, Walsh, & Shear, 2013). While our patient diagnosed with CPG did not respond, there have been cases of successful treatment of persistent CPG with RTX (Cianchini et al., 2007). Recently, successful preventive treatment with RTX of CPG in a pregnant woman has been published (Tourte, Brunet-Possenti, Mignot, & Gavard, 2016). Promising results with RTX associated with monthly regimens of immunoglobulins, have been reported in case series. (Ahmed, Shetty, Kaveri, & Spigelman, 2016; Foster, Chang, & Ahmed, 2010). Further studies are needed to explore the cost-effectiveness of administering RTX as first-line therapy in high-risk patients with MMP and EBA. This could halt the inflammatory response and thus avoid scarring and complications associated with conventional treatment. It is yet to be defined, which is the optimal adjuvant and/or the convenience of a maintenance regimen with RTX.

Actinic Keratosis—Can Dermoscopy or RCM Differentiate AK (Not Full Thickness Atypia) from Full-Thickness Atypia/Invasive SCC?

Purpose of ReviewActinic keratosis (AK) is the most common sun-induced preneoplastic lesion, and together with photo-aging and skin cancer, it places a huge burden on healthcare systems. The clinical distinction between AK and incipient squamous cell carcinoma (SCC) can be difficult, and therefore, clinical diagnosis is not always reliable and certain. Skin biopsies are sometimes mandatory, and physicians must be aware of the importance of accurate diagnosis and management, as other malignant neoplasms (melanoma, basal cell carcinoma) cannot always be reliably distinguished from AK, especially when pigmented and inflamed.Recent FindingsAlthough histopathology remains the gold standard for differentiation of AK from SCC, some non-invasive optical technologies such as dermoscopy and reflectance confocal microscopy have recently been applied to enhance clinical diagnosis accuracy and to obtain an in vivo characterization of these lesions.SummaryThe combination of dermoscopy with reflectance confocal microscopy improves the clinical assessment and diagnosis of equivocal keratinizing tumors, allows the selection of the most suspicious areas for biopsy, and permits non-invasive determination of treatment outcomes.

Systemic allergic dermatitis caused by disulfiram (Antabuse) in a patient previously sensitized to rubber accelerators

Disulfiram (tetraethylthiuram disulfide; Antabuse) is an aldehyde dehydrogenase inhibitor widely used for the treatment of alcoholism. Chemically, it is a thiuram, and it is also used as rubber accelerator. Thiuram mix is composed of 4 thiurams, including disulfiram, all of which are used in rubber manufacturing and are included in most patch test baseline series. Thiurams can be found in industrial and domestic rubber products, and are common causes of allergic contact dermatitis (ACD). Concomitant sensitization between thiurams is frequent.

Sífilis maligna, una presentación de sífilis a tener en cuenta

Malignant syphilis is an uncommon form of secondary syphilis associated with HIV infection. Clinically, it is characterized by necrotic nodules and generalized ulcerated lesions. We present 4 cases of malignant syphilis diagnosed after evaluating syphilis cases diagnosed at our hospital between 2012 and 2016. We describe the epidemiologic, clinical, histiopathologic, and serologic characteristics of malignant syphilis and explore its response to treatment and association with HIV infection. Although malignant syphilis is uncommon, there has been an increase in the number of cases published in recent years, particularly in young HIV-positive patients. Malignant syphilis must be contemplated in the differential diagnosis of HIV patients with ulcerated, necrotic lesions.

Porokeratosis ptychotropica responding to photodynamic therapy: An alternative treatment for a refractory disease

Porokeratosis ptychotropica (PP) is a rare variant of porokeratosis with a special predisposition to affect body folds, particularly the intergluteal cleft. This disease is resistant to most topical and systemic treatments, as shown in the review of the literature we provide here. Itching and discomfort are often a difficult problem to solve.

Abdominal violaceous skin lesions of a 47-year-old woman following a geometric pattern

We present the case of an obese 47-year-old woman from the Dominican Republic, living in Spain since 2003. She came to our outpatient department reporting a 15-day-history of painful nodules and ulcerations on the abdomen that had been treated by her general practitioner with amoxicilin/clavulanate 875/125 mg t.i.d. for 7 days with no improvement. She mentioned that 8 weeks previously, during a vacation trip to her country, she had undergone a mesotherapy session with injections of a liquid formula containing phosphatidylcholine to reduce the fat from her abdomen. On physical examination redness, swelling, drainage and ulceration were seen at the sites of mesotherapy injection (Fig. 1). Ultrasound imaging revealed subcutaneous edema, but no signs of deep abscessification, and MRI (T2) showed fat necrosis at the sites of injection. A punch biopsy was performed. Histopathologic examination revealed only mild dermoepidermal edema and a mixed infiltrate on the dermis without granulomas (Fig. 2). Periodic-Acid Schiff (PAS) and silver staining were negative as well as Ziehl-Neelsen. Mycobacterium abscessus was identified on skin cultures. Antibiogram is shown in Table 1.

Doxiciclina, una alternativa efectiva, bien tolerada y de bajo coste como tratamiento de primera línea del penfigoide ampolloso

Please cite this article as: Morgado-Carrasco D, Riquelme-Mc Loughlin C, Fustà-Novell X, Iranzo P. Doxiciclina, una alternativa efectiva, bien tolerada y de bajo coste como tratamiento de primera línea del penfigoide ampolloso. Actas Dermosifiliogr. 2018;109:549--550. ∗ Corresponding author. E-mail address: piranzo@clinic.cat (P. Iranzo). corticosteroids have been used at doses of 0.5-1.5 mg/kg, but it has been shown that high-potency topical corticosteroids (HPTCs) applied over the entire body have a similar effectiveness with fewer side effects, and so these are recommended as first-line treatment of bullous pemphigoid. However, use of these agents can be difficult due to lack of therapeutic adherence given the patient characteristics, logistic constraints, and adverse effects associated with systemic absorption. Among other treatment alternatives, tetracyclines have been widely used for treatment of bullous pemphigoid, thanks to their anti-inflammatory effect (Table 1), low cost, and good tolerability; however, robust evidence of their efficacy has not been available. Recently, Williams et al. published the results of a randomized noninferiority clinical trial (defining an effectiveness margin of 37% as acceptable) comparing doxycycline (200 mg/day, 132 patients) with prednisolone (0.5 mg/kg/day, 121 patients). Both groups were allowed to apply HPTCs to cutaneous lesions only, without exceeding 30 g/week, for the first 3 weeks. The mean age of the patients was 77 years and 68% had moderate or severe bullous pemphigoid. The main outcome measures were effectiveness at 6 weeks, where effectiveness was defined as having ≤ 3 blisters, and safety, determined by severe adverse effects at 52 weeks. In the doxycycline group, 74% achieved total control of bullous pemphigoid at 6 weeks compared with 91% in the prednisolone group (adjusted difference of 18.6% [90% CI: 11.1%-26.1%]), enabling the conclusion to be drawn that doxycycline was not inferior to prednisolone. Severe adverse effects were reported in 18%