ABSTRACT
The skin signs of benign and malignant endocrine and neuroendocrine tumors are manifold and early identification of these dermatologic features is crucial in initiating timely diagnosis and management. This article reviews the salient cutaneous features of these tumors that arise in the classic endocrine glands, lung and gastrointestinal tract either as individual neoplasms or as part of a syndrome.
Subject(s)
Endocrine Gland Neoplasms/pathology , Neuroendocrine Tumors/pathology , Paraneoplastic Syndromes/pathology , Adenocarcinoma/pathology , Adenoma/pathology , Carcinoma, Merkel Cell/pathology , Carcinoma, Merkel Cell/secondary , Carney Complex/pathology , Humans , Multiple Endocrine Neoplasia/pathology , Pancreatic Neoplasms/pathology , Paraneoplastic Syndromes/etiology , Parathyroid Neoplasms/pathology , Skin Neoplasms/pathology , Skin Neoplasms/secondaryABSTRACT
Nephrogenic systemic fibrosis, initially called nephrogenic fibrosing dermopathy, has been strongly linked to exposure to gadolinium-based contrast media used in magnetic resonance imaging in patients with renal insufficiency. This review discusses recent advances in our understanding of the pathophysiology and clinical approach to patients with chronic kidney disease who require diagnostic imaging with gadolinium-based contrast media.
ABSTRACT
Akhenaten was one of Egypt's most controversial pharaohs, in part because of his strange appearance in images produced after he had declared Aten, the Sun-disc, his one-and-only god. Whether these were symbolic representations or realistic ones that indicate a deforming genetic disorder is the subject of continuing debate. The authors present evidence that the bizarre physical features portrayed in these images are not only realistic but were shared by many members of Egypt's 18th Dynasty. The features are best explained by either 2 different familial disorders-the aromatase excess syndrome and the sagittal craniosynostosis syndrome-or a variant of the Antley-Bixler syndrome caused by a novel mutation in one of the genes controlling the P450 enzymes, which regulate steroidogenesis and cranial bone formation.
Subject(s)
Antley-Bixler Syndrome Phenotype/history , Craniosynostoses/history , Famous Persons , Metabolism, Inborn Errors/history , Antley-Bixler Syndrome Phenotype/genetics , Aromatase/genetics , Craniosynostoses/genetics , Egypt, Ancient , Female , History, Ancient , Humans , Male , Metabolism, Inborn Errors/genetics , Mutation , PaleopathologySubject(s)
Calcinosis/etiology , Raynaud Disease/etiology , Scleroderma, Limited/etiology , Skin/pathology , Telangiectasia, Hereditary Hemorrhagic/etiology , Antigens, CD/metabolism , Calcinosis/diagnosis , Calcinosis/metabolism , Diagnosis, Differential , Endoglin , Humans , Raynaud Disease/diagnosis , Raynaud Disease/metabolism , Receptors, Cell Surface/metabolism , Scleroderma, Limited/diagnosis , Scleroderma, Limited/metabolism , Skin/metabolism , Telangiectasia, Hereditary Hemorrhagic/diagnosis , Telangiectasia, Hereditary Hemorrhagic/metabolismABSTRACT
The coexistence in the same patient of epidermolysis bullosa acquisita (a rare, autoimmune, acquired mucocutaneous blistering disorder) and multiple myeloma (a plasma cell neoplasm) is extremely uncommon. We describe a patient in whom both of these diseases occurred simultaneously. Intravenous immunoglobulins were used to treat both diseases.
Subject(s)
Epidermolysis Bullosa Acquisita/complications , Epidermolysis Bullosa Acquisita/pathology , Multiple Myeloma/complications , Multiple Myeloma/pathology , Biopsy, Needle , Dexamethasone/administration & dosage , Drug Therapy, Combination , Epidermolysis Bullosa Acquisita/diagnosis , Follow-Up Studies , Humans , Immunoglobulins, Intravenous/administration & dosage , Immunohistochemistry , Male , Middle Aged , Multiple Myeloma/diagnosis , Risk AssessmentABSTRACT
This article concentrates on the major signs and syndromes that are associated with internal malignancies in the geriatric population. Included are cutaneous metastases, ectopic adrenocorticotropic hormone-producing syndromes, and disorders arising from APUD cell tumors. The major paraneoplastic disorders of dermatomyositis, generalized pruritus, Bazex's syndrome, and acanthosis nigricans also are discussed. Also included are Bowen's disease of skin; arsenical toxicity; and the Peutz-Jeghers', Gardner's, and Torre's syndromes, which are indicative of systemic or organ-related carcinogens.
