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Palisaded neutrophilic granulomatous dermatitis and its associations with autoimmune diseases
No full textPalisaded neutrophilic granulomatous dermatitis and its associations with autoimmune disease
Segregation analysis revealed hemizygotic causative mutations in a pseudoxanthoma elasticum patient
No full textPseudoxanthoma elasticum (PXE, OMIM 264800) is an autosomal recessive disorder in which elastic fibers of skin, eyes, and cardiovascular system become progressively calcified, causing a spectrum of manifestations with a variable phenotype. The proposed prevalence of PXE is 1/25 000, but this might be an underestimate. PXE is associated with mutations in the ABCC6 (ATP binding cassette subtype C number 6) gene. This article is protected by copyright. All rights reserved
Cutaneous manifestations of adult-onset Still's disease: a case report and review of literature
No full textAdult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology and pathogenesis characterized by high spiking fever, arthralgia or arthritis, sore throat, lymphadenopathy, hepatosplenomegaly, serositis, and transient cutaneous manifestations. Although more common in children, cases are seen also in adults. Cutaneous involvement is common and may be suggestive for the diagnosis. A case of AOSD in a 35-year-old man is reported here, presenting with urticarial maculopapular rash of trunk, high spiking fever, acute respiratory distress syndrome, and myopericarditis. Skin biopsy showed interstitial and perivascular mature CD15(+) neutrophils. A comprehensive review of literature showed that cutaneous involvement occurs in about 80 % of patients, with various clinical presentations. The most common skin manifestation is an evanescent salmon pink or erythematous maculopapular exanthema, predominantly on the trunk and proximal limbs, with rare involvement of face and distal limbs. Less common manifestations include persistent erythematous plaques and pustular lesions. A constant histopathologic finding is the presence of interstitial dermal neutrophils aligned between the collagen bundles. This pattern may provide an easy accessible clue for the definitive diagnosis of AOSD and exclude other diagnosis such as drug eruptions or infectious diseases
Radioiodine ablation: when and how
No full textDifferentiated thyroid cancer (DTC) is the most common endocrine malignancy with the highest mortality although with appropriate treatment has a good long-term prognosis and cure rate. Over the last 30 years there is a worldwide trend showing an increasing incidence of thyroid cancer. In DTC patients, total thyroidectomy has been for many decades routinely followed by the administration of radioiodine (131I) activity to destroy remnant thyroid tissue. Several reasons are in favour to routine ablation of postoperative thyroid remnants. The combination of both surgery and radioiodine has proven as a safe and effective treatment, resulting in improved life expectation and reduced recurrence rate for DTC patients. Recently, however, 131I ablation is not uniformly recommended for cancers smaller than 10 mm, and its use is debated for papillary tumours with diameter between 10 and 20 mm. Indeed, the decision about subsequent 131I thyroid remnant ablation is recommended as "individualized and selective". Even if new evidence has emerged that provides additional support for performing 131I treatment, the possible presence of radioiodine-associated side effects should be not overlooked. Moreover, a lot of discussion has taken place as to whether, and to what extent, 131I may cause secondary malignancies. Blood-based dosimetry is important to avoid surplus bone marrow toxicity while treating DTC patients. In this regard, the availability of a genetically engineered version of recombinant human TSH (rhTSH) provides an alternative tool to enhance serum TSH levels without inducing hypothyroidism. The administration of rhTSH to thyroid cancer patients still on LT4 therapy promotes radioiodine uptake and thyroglobulin production by thyroid cells to an extent comparable with hypothyroidism, preserving patients' quality of life, increasing the renal clearance of 131I and decreasing both the whole body and the blood dose. In this review the authors will discuss the pros and cons of postoperative radioiodine-induced thyroid remnant ablation
Urticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria: Part I. Cutaneous diseases
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Lichen planopilaris coexisting with plaque psoriasis effectively treated with brodalumab
No full textLichen planopilaris coexisting with plaque psoriasis effectively treated with brodaluma
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