Mastocytosis
58. Mastocytosis Definition Mastocytosis is a clonal disorder in which mast cells and the precursor cells accumulate in different tissues, such as bone marrow, skin, gastrointestinal tract, liver, and spleen….
Gardner Syndrome
38. Gardner Syndrome Definition Gardner syndrome is a type of familial adenomatous polyposis that also has extracolonic manifestations, including intestinal polyposis, desmoids, osteomas, and epidermoid cysts. The disorder is inherited…
Myasthenia Gravis
59. Myasthenia Gravis Definition Myasthenia gravis is an acquired autoimmune disorder of neuromuscular function marked by fatigue and exhaustion of the muscular system. Fluctuations in severity of muscular atrophy accompany…
Lymphomatoid Granulomatosis
54. Lymphomatoid Granulomatosis Definition Lymphomatoid granulomatosis (LYG) is a rare angiodestructive, lymphoproliferative disease with significant pulmonary involvement in the form of pulmonary angiitis and granulomatosis. LYG mimics Wegner’s granulomatosis (see…
Lymphangioleiomyomatosis
53. Lymphangioleiomyomatosis Definition Lymphangioleiomyomatosis (LAM) affects women during the childbearing years. It is characterized by proliferation of smooth muscle phenotypic neoplastic cells in the lungs, kidneys, and axial lymphatics. The…
Lown-Ganong-Levine Syndrome
51. Lown-Ganong-Levine Syndrome Definition Lown-Ganong-Levine (LGL) syndrome is a preexcitation syndrome producing supraventricular tachycardia that is neither atrial fibrillation nor atrial flutter. This syndrome is characterized by a short P-R…
Long QT Syndrome
50. Long QT Syndrome Definition Long QT syndrome (LQTS) is prolonged duration of the QT interval in the cardiac cycle. This syndrome can be congenital or acquired. The congenital form…
Ludwig’s Angina
52. Ludwig’s Angina Definition Ludwig’s angina is cellulitis of the mouth floor. It expands very rapidly and can spread to other areas of the body, such as the mediastinum or…
Incontinentia Pigmenti
47. Incontinentia Pigmenti Definition Incontinentia pigmenti is a rare, X-linked, dominant inherited disorder involving skin pigmentation. Melanin is lost from the basal cells of the epidermis and collects in the…
Ehlers-Danlos Syndrome
27. Ehlers-Danlos Syndrome Definition Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders that vary according to clinical and biochemical evidence, inheritance mode, and disorder severity (mild to…
