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LMD-MS/MS analysis of several dermal microdissected amyloid deposits demonstrated high spectra for KRT5/14 heterodimers, along with the amyloid signature proteins. Spectra of other known amyloid proteins were not detected; notably, no spectra for light chain immunoglobulins were detected, ruling out AL amyloidosis. gl The Chinese family included three symptomatic members (II.8, II.9 and II.10), who complained of pruritic lesions, highly suggestive of lichen amyloidosis, over arms, legs and back. LC-MS/MS and immuno-electron subtyping combined with genetics show that OSMR mutations cause amyloid deposition of keratins 5/14 in familial primary localized cutaneous amyloidosis. [Extracted from the article]

Background: Sarcoidosis, characterized by epithelioid granulomas, is considered to be caused by a complex interplay between genetics and environmental agents. It has been hypothesized that exogenous inorganic particles as crystalline silica could be a causal or adjuvant agent in sarcoidosis onset. Objectives: To investigate the location, frequency and physicochemical characteristics of foreign materials and mineral tissue deposits in the granulomatous area of cutaneous sarcoidosis. Methods: Skin biopsies (n = 14) from patients diagnosed with cutaneous sarcoidosis (mean age 43 years; 11 patients with extracutaneous involvement) were investigated using polarized light examination (PLE), μFourier Transform Infra‐Red (μFT‐IR) spectroscopy and Field Emission Scanning Electron Microscopy coupled with Energy Dispersive X‐ray Spectroscopy (FE‐SEM/EDX). Results: Combined PLE, μFT‐IR, FE‐SEM/EDX analysis allowed to characterize mineral deposits in 7/14 biopsies (50%). It identified crystalline silica (SiO2) inside granulomas in three biopsies and calcite (CaCO3) at their periphery in 4. Conclusion: This study emphasizes the need of using combined methods for assessment of mineral deposits in granulomatous diseases. According to the location and characteristics of deposits, we can hypothesize that SiO2 particles contribute to the granuloma formation, whereas CaCO3 deposits are related to the granuloma biology. However, the significance of the association between SiO2 deposits and sarcoidosis is still disputed. [ABSTRACT FROM AUTHOR]

Background Epidermal growth factor receptor (EGFR) inhibitors are part of the therapeutic arsenal available for advanced cancer. However, they are frequently associated with cutaneous side-effects, which can hamper compliance, lead to treatment refusal and impair quality of life. Objective To know the attitudes of French oncologists who deal with this skin toxicity. This work is one of the steps to build a therapeutic algorithm of side-effects induced by EGFR inhibitors taking both evidence-based medicine and standard practices into account. Methods Physicians completed a questionnaire as part of regional meetings, before any discussion. Questions concerned the management of 11 clinical situations in the context of EGFR inhibitor prescription. Results Sixty-seven questionnaires were analysed. The collaboration with dermatologists was especially planned for persisting or worsening lesions beyond 2 weeks, but never considered at the time of the introduction of targeted therapy. The results demonstrated the difficulties encountered in diagnosing and grading skin lesions. Attitudes of oncologists were uniform for preventive care and management of mild lesions for which moisturizing and cyclines were widely prescribed. Significant differences appeared in the treatment of less typical cases such as the involvement of skin appendages, secondary infections of folliculitis or cases associated with radiodermatitis. Discrepancies existed also for what to do in relation with maintenance or interruption of EGFR inhibitor mainly if they were responsible for severe lesions. Conclusion This original survey emphasizes the interest of greater multidisciplinary collaboration and the necessity to harmonize practice. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article on the probable drug rash with eosinophilia and systemic symptoms syndrome related to tetrazepam that was published in the previous issue.

A letter to the editor is presented in response to the article about an 82-year-old French woman who was diagnosed with lichen planus, which is described as an unusual cause of oesophageal stricture.

A letter to the editor which describes granulomatous tattoo reaction in a 39-year-old man treated with etanercept.

Streptococcus pneumoniae is a rare cause of cellulitis that develops mainly in patients with underlying chronic disease or those immunocompromised by drug or alcohol abuse. We report here a case of pneumococcal cellulitis in an immunocompetent patient. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented regarding the role of laptop computer in the development of erythema ab igne.

The article presents a case of a 46-year-old woman with sickle cell anaemia (SCA) who was hospitalized for dyspnea of progressive onset. It states that she suffered from bilateral leg ulcers and was treated with hydrogel and skin graft. The patient undergone bosentan therapy to treat her pulmonary hypertension which lasted for 10 months.It states that pulmonary hypertension is a complication of SCA and further infers that patients with leg ulcers also have pulmonary hypertension.

The role of mast cells has been questioned in sickle cell disease (SCD). We performed a prospective study evaluating plasma histamine and tryptase levels in a cohort of paediatric and adult patients, in steady state (n = 132) and during vaso-occlusive crisis (VOC) (n = 121). Histamine level was elevated in 18% of patients in steady state and in 61% during VOC. Median histamine level was significantly higher during VOC than in steady state (24·1 [7·0-45·0] vs 9·6 [6·2-14·4] nmol/l, P < 0·0001). Tryptase level was slightly increased during VOC without reaching pathological values. These results suggest a role of mast cell activation in SCD pathophysiology., (© 2019 British Society for Haematology and John Wiley & Sons Ltd.)

Cardiac involvement is well characterized in sickle cell anaemia (SCA) but cardiac features associated with Haemoglobin SC (HbSC) disease are mostly unknown. We compared 60 patients with HbSC disease (median age 31 years, 25 men) to 60 SCA patients and 60 controls matched for age and gender. Left ventricular ejection fraction (LVEF), left ventricle (LV) mass index (LVMi), cardiac index and peak tricuspid regurgitation velocity (TRV) were measured using echocardiography. LV filling pressures were assessed using the ratio of early diastolic transmitral velocity to tissue velocity (E/e' ratio). The LVMi was higher in both genotypes compared to controls. However, whereas LV hypertrophy was observed only in 3(5%) HbSC patients, this condition was diagnosed in 27(45%) SCA patients (P < 0·0001). While cardiac index and TRV were similar in HbSC compared to controls, SCA patients exhibited elevated cardiac output and TRV. LVEF was similar in the 3 groups. However, both genotypes had a higher E/e' ratio compared to controls. Cardiac involvement in SCA was related to anaemia and haemolysis, while LV diastolic dysfunction and TRV in HbSC disease patients were related to arterial hypertension and overweight comorbidities. In summary, cardiac involvement and its determinants are different in HbSC disease and SCA. Patient's genotype should be considered with regard to the echocardiographic indications and findings., (© 2018 John Wiley & Sons Ltd.)