Loin pain hematuria syndrome (LPHS) is the combination of debilitating unilateral or bilateral flank pain and microscopic or macroscopic amounts of blood in the urine that is otherwise unexplained.Loin pain-hematuria syndrome (LPHS) is a poorly defined disorder characterized by recurrent or persistent loin (flank) pain and hematuria that appears to represent glomerular bleeding. Most patients present with both manifestations, but some present with loin pain or hematuria alone. Pain episodes are rarely associated with low-grade fever and dysuria, but urinary tract infection is not present. The major causes of flank pain and hematuria, such as nephrolithiasis and blood clot, are typically not present. Renal arteriography may suggest focally impaired cortical perfusion, while renal biopsy may show interstitial fibrosis and arterial sclerosis.The pain is typically severe, and narcotic therapy is often prescribed as a way to manage chronic pain. Sleep can be difficult because the supine position increases pressure on the flank. The onset of pain is often associated with nausea and vomiting, making pain management by oral opiates complicated. Cause
The cause of LPHS is not known. One theory proposes that it is caused by a thin glomerular basement membrane and red blood cell (RBC) renal tubular congestion that leads to swelling of the kidney and distension of the renal fascia resulting in pain.Researchers have hypothesized that the syndrome may be due to blood vessel diseases of the kidney, spasms of the kidney vessels, or other bleeding disorders (coagulopathy). The hematuria in LPHS may be due to an abnormal (thick or thin) glomerular basement membrane.
A cutaneous myxoma, or superficial angiomyxoma, consists of a multilobulated myxoid mass containing stellate or spindled fibroblasts with pools of mucin forming cleft-like spaces. There is often a proliferation of blood vessels and an inflammatory infiltrate. Staining is positive for vimentin, negative for cytokeratin and desmin, and variable for CD34, Factor VIIIa, SMA, MSA and S-100.Clinically, it may present as solitary or multiple flesh-colored nodules on the face, trunk, or extremities. It may occur as part of the Carney complex, and is sometimes the first sign. Local recurrence is common. See also
Myxoma
Skin lesion


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