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At present, no studies have evaluated the role of bronchoscopic transbronchial lung biopsy (TBLB) in the diagnosis of diffuse drug-induced lung disease (DILD), and there is no consensus for a definite diagnostic workup approach for this rare clinical entity. The aim of the present study was to evaluate the clinical usefulness of TBLB in diffuse DILD. This study was a retrospective analysis of patients with diffuse DILD, who underwent bronchoscopy. The role of TBLB was assessed to determine whether the histological results are useful for the final diagnosis. Over a 5-yr period, 44 patients underwent bronchoscopy, and all had a bronchoalveolar lavage (BAL). Thirty-three of the 44 patients underwent TBLB (75%), and the results of TBLB were diagnostically helpful in 25 (75.7%) of the procedures. No histopathologic abnormality was identified in 6 (18%) of the 33 patients. In 2 patients (6%) the obtained samples were not adequate, since no lung parenchyma was obtained. No severe complications related to bronchoscopic procedures occurred. In conclusion, TBLB is a safe procedure, and is diagnostically helpful in the majority of cases of patients with diffuse DILD. Histological data obtained by TBLB further corroborate clinical, laboratory, radiologic and BAL results for a definitive diagnosis of diffuse DILD.

The lung is prone to develop various complications in patients with hematological disorders due to its structural and functional properties. Genetic predisposition, although probably involved, is not yet well understood. The secondary involvement of lung parenchyma by a lymphoproliferative malignancy is not an unusual event. Furthermore, the advances in treatment options such as hematopoietic stem cell transplantation, radiation therapy, newer polychemotherapeutic regimens even including the most recent drugs, have significantly widened the spectrum of non-neoplastic complications that can rise up in these patients. Infections, drug/radiation-induced toxicity, and graft versus host disease account by now for most pulmonary problems in hematologic patients and represent a diagnostic challenge for the clinician. A specific diagnosis is, however, important for prompt initiation of the correct therapy. High resolution computed tomography followed by bronchoalveolar lavage and/or transbronchial lung biopsy have proved to be the most helpful diagnostic tools within the multidisciplinary approach to these disorders. The aim of this review is to highlight the clinico-pathologic spectrum of lung diseases which can occur in the setting of hematologic neoplasms, pointing up the clinical approach with the different diagnostic tools avaible. Pulmonary amyloidosis and lung complications in hemoglobinopathies, erythrocyte and coagulation disorders will not be examinated.

Granuloma annulare (GA) is a self-limited benign inflammatory dermatosis of unknown origin characterized by the development of papules arranged in an annular, circinate pattern, mainly involving the extensor surfaces of lower and upper extremities. The diagnosis depends on clinical and histological features. There are four main variants of GA: localized GA (LGA), subcutaneous GA (SGA), generalized GA (GGA) and perforating GA (PGA) and 2 uncommon subtypes: papular umbilicated GA and linear or segmental GA. Children most commonly present with the localized and subcutaneous forms. The aim of this review was to provide an update on the etiology, diagnosis and management of GA focusing on the pediatric age.

intravascular large B-cell lymphoma is a distinct subtype of mature B-cell neoplasms, with uncommon primary presentation in the lungs. Diagnosis could be very difficult due to the lack of detectable tumor masses and it is usually made by surgical lung biopsy or autopsy examination.two patients occurred primarily with interstitial lung disease and underwent a pulmonary biopsy using cryoprobes.the pathological analysis of the lung biopsies revealed in both cases a conclusive diagnosis of intravascular large B-cell lymphoma with primary lung involvement and patients have been safely diagnosed using transbronchial cryobiopsy for the first time in the literature.transbronchial cryobiopsy could be used as valid surrogate for surgical lung biopsy in lymphoprolipherative lung disorders (including intravascular lymphomas), as allows larger samples of tissue, greater diagnostic yield, no crush artifacts and much less complications than surgical biopsy.

IPF is a common form of interstitial lung disease for which there is no effective therapy and usually results in death. Two previous contradictory studies showed anticoagulant therapy to be associated with both improved and worsened survival, respectively.The objective of this retrospective cohort study was to evaluate the effect of anticoagulant therapy on the survival and disease progression of patients with idiopathic pulmonary fibrosis (IPF) in real clinical practice.We compared the clinical characteristics, time to disease progression, incidence of acute exacerbation, and survival of 25 (20%) IPF patients receiving anticoagulant therapy to the remaining 97 IPF patients not receiving anticoagulant therapy. In addition we conducted a sensitivity analysis using as comparator a group of 25 patients matched by age, sex, functional impairment, cardiac comorbidities and pulmonary hypertension.Patients on anticoagulant therapy had a worse 1- and 3-year survival (84% and 53% versus 89% and 64% in the non-anticoagulant group, respectively), a difference that persisted after adjusting for age and comorbidities (hazard ratio 3.1 - 95% confidence interval, 1.4 to 7.0; p=0.006) and after comparison with the matched group (adjusted HR=4.8, 95% CI: 1.8-12.8; p=0.002). IPF patients on anticoagulant therapy had a shorter interval to disease progression ( 0.7 years versus 1.6 years, adjusted HR 2.2 -95% CI, 0.96 to 5.1; p=0.063) confirmed also in the analysis with matched subgroups (HR=2.7 (95% CI: 1.2-6.5); p=0.023). The incidence of acute exacerbations did not differ in the two groups (22% versus 23%). Two patients (8%) experienced anticoagulant treatment related complications and included an episode of hemorrhagic shock.In this retrospective study patients treated with anticoagulants had a worse survival and a shorter interval to disease progression. This support the recent finding that warfarin worsen the respiratory status and survival of IPF patients.

EBUS-TBNA and EUS-FNA are minimally invasive techniques rapidly gaining ground in the non-surgical invasive diagnostic approach to thoracic diseases due to their high accuracy and low morbidity and mortality compared to surgical techniques. Moreover, in the diagnosis and staging of lung cancer the combination of the two techniques is superior to either test alone. In this review we focus on the role of EBUS-TBNA and EUS-FNA in both malignant and non-malignant thoracic diseases.

Melorheostosis (MEL) is a rare benign bone disorder that can be associated with several anomalies, including vascular abnormalities, nevus sebaceus, unilateral nevoid telangiectasia, linear scleroderma and hypertrichosis. We report the case of a 6-year-old patient who showed an unusual co-occurrence of bone hyperostosis and different skin lesions affecting the same side of the body: MEL, verrucous epidermal nevus, connective tissue nevus, linear scleroderma-like disorder, hyperpigmentation and hypertrichosis. The spatial co-occurrence of these conditions made us speculate as to whether they originated from a common genetic mechanism or if their co-occurrence was completely accidental.

Background Narrowband-ultraviolet B (NB-UVB) is widely used for the treatment of several dermatological diseases. A cutaneous carcinogenic effect has been hypothesized, but not proved. Methods We retrospectively reviewed the data of patients treated with NB-UVB between January 1998 and December 2013 at the Dermatology Unit of our University Hospital, to evaluate the cutaneous carcinogenic risk of NB-UVB. Results 375 patients were included, each receiving a mean follow-up of 6.9 years. Vitiligo and psoriasis were the most common diseases. 19 non-melanoma skin cancers (NMSCs) were diagnosed in 8 patients, after a mean latency of 5.2 years after the first radiation. No malignant melanoma (MM) was observed. The incidence rates of basal cell carcinoma (BCC) and squamous cell carcinoma (SCC) were 620.2/100 000 p/y and 116.3/100 000 p/y. NMSCs were more frequent in patients affected by psoriasis (p = 0.0232), with older age at the first radiation (mean = 68.8 years, p = 0.0001). Conclusion Despite the small number of patients and limited follow-up, our data suggest that NB-UVB may trigger cutaneous carcinogenesis, mainly in patients at risk for NMSCs, increasing their personal risk for single and multiple neoplasms, usually superficial BCCs. MM risk does not seem to be enhanced. This article is protected by copyright. All rights reserved.

BACKGROUND: Targeted therapies in melanoma have shown clinical benefit in incrementing the overall survival of metastatic patients. However, cutaneous adverse events have been frequently associated with these drugs. METHODS: We report our experience in the management of patients treated with dabrafenib for metastatic melanoma, focusing on the monitoring of pigmented lesions. Dermatologic evaluation was performed during the first visit, at the start of each treatment and subsequently after every four weeks. Global nevi count, videodermoscopy of suspected lesions, and surgical excisions when necessary were performed at the beginning of the treatment and every fourth week. All other cutaneous adverse events (cAEs) were noted and documented. Eleven patients were included. RESULTS: The most important cAEs included palmo-plantar hyperkeratosis, diffuse xerosis and pigmented lesion changes. Regarding the latter, in 6 patients, especially in the first months of treatment, we observed hyperpigmentation and hyperkeratosis of the nevi, of the pigmented mucosae and, in one patient, hyperkeratotic changes on a cutaneous metastasis. Histopathology of the excised lesions showed one ex novo melanoma occurrence and benign changes to pre-existing nevi. CONCLUSIONS: The awareness of the importance of sequential monitoring of pigmented lesions, with particular attention to the lesions of new onset, is crucial for the best management of these complex patients.

Background. Transbronchial lung biopsy (TBLB) is a valuable procedure used to obtain a parenchymal specimen in the evaluation of diffuse lung infiltrates. Large forceps are expected to result in larger specimens and improve diagnostic yield. Aim. The objective of this study was to evaluate diagnostic yield of TBLB using large modified flexible gastroenterological forceps (“Jumbo forceps”) compared with ‘normal’ flexible forceps via rigid bronchoscopy in patients with diffuse parenchymal lung disease (DPLD). Methods. The study was a prospective analysis of 95 patients who underwent fluoroscopy guided TBLB over a two year period. Patients with a lung mass or solitary lung nodule undergoing TBLB were excluded. The larger and small forceps were used in a random sequence to avoid a reduction in diagnostic yield of the second series of biopsies related to possible bleeding by first series of biopsies. To minimize the consequence of haemorrhage, we performed every rigid bronchoscopy, placing a non inflated Fogarty balloon and a rigid aspirator (diameter 4 mm) in lobar bronchus near the biopsy segment. The Fogarty balloon has been inflated in case of bleeding. After the bleeding was controlled we continued to operate up to the biopsy segment. Results. Diagnostic yield of TBLB using Jumbo forceps was significantly higher than using normal flexible forceps via rigid bronchoscopy in patients with DPLD (p=0.001). In 74 out of 95 patients (78%) the diagnosis was placed with Jumbo forcep while the smaller forcep was diagnostic in 62 out of 95 patients (65%). Large forceps obtained significantly more tissue than the small forceps; the biopsy specimen taken with normal forcep measured in average 1.4 x 1.0 mm and the larger biopsy taken with jumbo forcep measured in average 2.5 x 1.9 mm (p < 0.005). Conclusion. The use of large biopsy forceps to perform TBLB via rigid bronchoscope can significantly increase diagnostic yield in the pathological diagnosis of diffuse infiltrative lung disease.

Lichen sclerosus (LS) shows a predilection for the genital area and occurs mostly in postmenopausal women and in prepubertal girls. We conducted a retrospective review of 15 young girls whit genital LS with onset before the menarcheal age and treated with topical clobetasol propionate 0.05%. The mean duration of follow-up was 4.7 years with relapses in nine patients after approximately 1 year from the first clearing. At the end of the study we observed that (i) potent topical steroids are safe and effective in childhood, (ii) an early aggressive treatment gives the best therapeutic response, (iii) one follow-up visit a year is required to monitor for relapsing.