scleroderma

<body topmargin=0 leftmargin=0 marginheight=0 marginwidth=0> <table cellpadding=0 cellspacing=0 border=0><tr> <td valign="top" colspan=2 height=110 BGCOLOR="#666699" TEXT="#080000" bgproperties="fixed" background="04a00840erlcjm.png"> <div> <FONT SIZE="5" COLOR="#FFFFFF" FACE="Arial" ><B>usmle medecine part 2</B></FONT><B>     |     </B><A HREF="index.htm" TARGET="_top" TITLE="usmle medecine part 2"><U><B>home</B></U></A></div> <div> <A HREF="id18.htm" TARGET="_top" TITLE="crest syndrome"><U>crest syndrome</U></A>   |   <A HREF="id20.htm" TARGET="_top" TITLE="raynauld phenomenon"><U>raynauld phenomenon</U></A>   |   <B>scleroderma</B>   |   <A HREF="id22.htm" TARGET="_top" TITLE="The Iliac Crest Syndrome"><U>The Iliac Crest Syndrome</U></A>   |   <A HREF="id23.htm" TARGET="_top" TITLE="cardio"><U>cardio</U></A>   |   <A HREF="id24.htm" TARGET="_top" TITLE="hypoparathyroidism"><U>hypoparathyroidism</U></A>   |   <A HREF="id25.htm" TARGET="_top" TITLE="hypercalcemia"><U>hypercalcemia</U></A>   |   <A HREF="id26.htm" TARGET="_top" TITLE="hyperphosphatemia"><U>hyperphosphatemia</U></A>   |   <A HREF="id27.htm" TARGET="_top" TITLE="leukemia"><U>leukemia</U></A>   |   <A HREF="id21.htm" TARGET="_top" TITLE="glossary"><U>glossary</U></A>   |   <A HREF="id17.htm" TARGET="_top" TITLE="Contact Me"><U>Contact Me</U></A></div> <div> </div> </td> </tr><tr> <td valign="top" width=190 BGCOLOR="#CCCCFF" TEXT="#080000" background="06be95e3.jpg"> <div> <A HREF="id18.htm" TARGET="_top" TITLE="crest syndrome"><U><B>crest syndrome</B></U></A></div> <div> <A HREF="id20.htm" TARGET="_top" TITLE="raynauld phenomenon"><U><B>raynauld phenomenon</B></U></A></div> <div> <B>scleroderma</B></div> <div> <A HREF="id22.htm" TARGET="_top" TITLE="The Iliac Crest Syndrome"><U><B>The Iliac Crest Syndrome</B></U></A></div> <div> <A HREF="id23.htm" TARGET="_top" TITLE="cardio"><U><B>cardio</B></U></A></div> <div> <A HREF="id24.htm" TARGET="_top" TITLE="hypoparathyroidism"><U><B>hypoparathyroidism</B></U></A></div> <div> <A HREF="id25.htm" TARGET="_top" TITLE="hypercalcemia"><U><B>hypercalcemia</B></U></A></div> <div> <A HREF="id26.htm" TARGET="_top" TITLE="hyperphosphatemia"><U><B>hyperphosphatemia</B></U></A></div> <div> <A HREF="id27.htm" TARGET="_top" TITLE="leukemia"><U><B>leukemia</B></U></A></div> <div> <A HREF="id21.htm" TARGET="_top" TITLE="glossary"><U><B>glossary</B></U></A></div> <div> <A HREF="id17.htm" TARGET="_top" TITLE="Contact Me"><U><B>Contact Me</B></U></A><B>  <a href="http://counter.mycomputer.com/"><img alt="Counter" src="http://counter.mycomputer.com/c.count?u=dhammoud&c=1" border=0></a>   </B></div> <div> <B>  <a href="http://counter.mycomputer.com/"><img alt="Counter" src="http://counter.mycomputer.com/c.count?u=dhammoud&c=1" border=0></a>   </B></div> </td> <td valign="top" height=370 BGCOLOR="#FFFFFF" TEXT="#080000"> <div> scleroderma</div> <div> scleroderma[see also my<A HREF="http://pathologylectures.homestead.com" TARGET="_top" TITLE="http://pathologylectures.homestead.com"><U> pathology lectures</U></A> page]</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Scleroderma is a chronic autoimmune disease that involves the skin and connective tissue</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> There are two types, localized and systemic.</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0"> In the localized type, the skin shows one or more patches of sclerosis (thickening and hardening). </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">The systemic type involves the skin and the connective tissue</div> <bR> <div> Systemic forms of scleroderma include progressive systemic sclerosis (PSS), also known as systemic sclerosis (SS), and the CREST syndrome. Systemic scleroderma involves body systems such as the esophagus, intestines, lungs, heart and kidneys. </div> <bR> <div> Diffuse scleroderma is a term which describes systemic sclerosis and skin changes on many parts of the body. Tight, glossy skin may be present on the trunk and upper arms as well as on the face, chest and extremities. </div> <bR> <bR> <bR> <div> <IMG SRC="063c2430.jpg" border=0 width="194" height="323" ALIGN="BOTTOM" HSPACE="0" VSPACE="0"><IMG SRC="06461f30.jpg" border=0 width="353" height="243" ALIGN="BOTTOM" HSPACE="0" VSPACE="0"><IMG SRC="06571f30.jpg" border=0 width="369" height="243" ALIGN="BOTTOM" HSPACE="0" VSPACE="0"><IMG SRC="06770c80.jpg" border=0 width="112" height="200" ALIGN="BOTTOM" HSPACE="0" VSPACE="0"><IMG SRC="068e1a90.jpg" border=0 width="225" height="169" ALIGN="BOTTOM" HSPACE="0" VSPACE="0"><IMG SRC="066f76e0.jpg" border=0 width="247" height="366" ALIGN="BOTTOM" HSPACE="0" VSPACE="0"><IMG SRC="0699ac80.jpg" border=0 width="154" height="200" ALIGN="BOTTOM" HSPACE="0" VSPACE="0"></div> <div> progressive systemic sclerosis (PSS) </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">hypertrophy then atrophy of collagen fibers </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">4-6th decade, M:F = 1:3 </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">bones </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">punctate soft tissue ca++ (finger tips, shoulder, hips) </div> <div> <U><IMG BORDER="0" SRC="Symb075c00b700f0ff000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">acro-osteolysis</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > (63%) </FONT></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">intercarpal joint space narrowing (late) </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">chest </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">evident in 10-25% </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">pulmonary fibrosis with diffuse reticulate infiltrate </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">predominantly in lower lungs </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">GI </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">esophageal dilation and <FONT SIZE="3" COLOR="#0000FF" FACE="Arial" ><U>aperistalsis</U></FONT> (>50%) </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">hiatus hernia + GE reflux + esophagitis + distal esophageal stricture </div> <div> <U><IMG BORDER="0" SRC="Symb075c00b700f0ff000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">gastroparesis</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <div> <U><IMG BORDER="0" SRC="Symb075c00b700f0ff000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">dilation and dysmotility of small bowel</U><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">pseudosacculation and dysmotility of colon </div> <bR> <div> ANA present in 37-67%, pattern speckled, homogeneous, or nuclear +/- anti-DNA antibodies increased Ig RF seronegative </div> <bR> <div> Progressive systemic sclerosis (scleroderma) is a disorder of connective tissue dysfunction which affects whole organ systems including skin, lungs, GI, heart, kidneys and musculoskeletal.</div> <div>  The characteristic radiographic presentation is generally defined as <B>soft tissue atrophy, calcifications, and distal phalangeal resorption in the hand</B>.</div> <div>  In addition, recently described findings include erosion involving the distal interphalangeal disease (DIP) and less commonly proximal interphalangeal (PIP) joints. </div> <div> Although there has been no definite pathogenesis identified, it is felt that progressive systemic sclerosis secondary to a structural defect in the microvasculature.</div> <div> This is supported by the fact that there are often vascular changes identified in multiple tissues. </div> <div> Systemic sclerosis affects women approximately three to four times more commonly than it does men, with the onset between the third and fifth decades being most common.</div> <div>  Raynaud's phenomenon is a characteristic finding in these patients consisting of sudden pallor of the digits with associated pain when exposed to cold.</div> <bR> <div>  In addition, patients often complain of joint pain, erythema, and swelling. </div> <div> Skin involvement is the most characteristic clinical finding in systemic sclerosis consisting of bouts of edema, thickening of the dermis, and progressive rigidity with fixation to the subcutaneous tissues. </div> <div> A small percentage of patients will progress to develop significant atrophy of the skin as well as muscle and subcutaneous tissues. </div> <div> Tissue abnormalities in the hand and wrist are felt to be the most characteristic radiographic abnormalities found in systemic sclerosis. </div> <div> These are findings such as subcutaneous calcifications, soft tissue resorption, and osseous destruction. In addition, flexion contractures of the hands develop secondary to abnormal tightening of the skin.</div> <div>  The radiographically identifiable calcifications often involve the subcutaneous tissues, joint capsules, and/or synovial lining.</div> <div>  Osseous resorption is a characteristic finding in patients with systemic sclerosis and has been seen in as many as 80% of these patients.</div> <div> The resorption initially involve the palmar aspect of the distal phalanges with progressive resorption of the distal phalangeal tuft. </div> <div> Articular abnormalities are also identified in patients with systemic sclerosis including periarticular osteoporosis as well as bilateral asymmetric erosion. Erosions may be central or in a periarticular distribution often involving both the DIP and PIP joints. In addition, ankylosis may occur. </div> <div> The most important consideration in the differential diagnosis would be rheumatoid arthritis.</div> <div>  this may be especially true early in the course of the disease.</div> <div>  Osteoporosis, </div> <div> erosive changes,</div> <div>  and flexion deformities are characteristic findings found in both progressive systemic sclerosis and rheumatoid arthritis.</div> <div>  One possible differentiating feature is the relative sparing of the metacarpophalangeal joint in systemic sclerosis as opposed to the generally significant changes identified in rheumatoid arthritis.</div> <bR> <DIV ALIGN="LEFT"></DIV> <div> <I><B>MORPHEA</B></I><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <bR> <div> <I>Morphea happens when hard, oval-shape patches form on the skin. The patches are usually whitish with a purplish ring around them. They usually occur on the trunk, but can also occur on the face, arms, legs, and other parts of the body. Morphea often improves by itself, over time.</I><FONT SIZE="3" COLOR="#000000" FACE="Arial" ><I> </I></FONT></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">one or more oval or round circumscribed indurations that become hard and white </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">found in skin anywhere on trunk or extremities </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">characteristic violet border found early in disease </div> <bR> <DIV ALIGN="LEFT"></DIV> <div> <I><B>LINEAR</B></I><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <bR> <div> <I>Linear is a line of thickened skin that occurs in areas such as the arms, legs, or forehead. It can occur in more than one area. The line can extend deep into the skin and affect the bones and muscles underneath it. This can affect the motion of joints and muscles, as well as the growth of the affected area.</I><FONT SIZE="3" COLOR="#000000" FACE="Arial" ><I> </I></FONT></div> <bR> <div> <I>When the line forms a long crease on the head or neck, it is sometimes called en coup de sabre (the strike of the sword).</I><FONT SIZE="3" COLOR="#000000" FACE="Arial" ><I> </I></FONT></div> <div> <I>Linear scleroderma usually occurs in childhood.</I><FONT SIZE="3" COLOR="#000000" FACE="Arial" ><I> </I></FONT></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">one or more linear streaks on scalp, face, extremities </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">found in skin but involve underlying subcutaneous tissue, tendons, muscle, and bone -> contractions & deformities </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">scleroderma en coup de sabre - lesions of face or scalp </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">associated with significant local growth arrest -> atrophy of the skin, subcutaneous tissue, and bone </div> <bR> <bR> <DIV ALIGN="LEFT"></DIV> <div> <B>Visceral Manifestations</B></div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">occasionally develops late suggesting an evolution of the local to systemic forms of scleroderma </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.png" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">occasionally develop other connective tissue diseases: SLE, MCTD </div> <bR> <DIV ALIGN="LEFT"></DIV> <div> <I><B>GENERALIZED</B></I><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <bR> <div> <I>Generalized scleroderma affects many parts of the body. It can affect the skin as well as internal body parts, such as blood vessels, the digestive system (esophagus, stomach, and bowel), the heart, lungs, kidneys, muscles, and joints. The severity of this form depends on the organs affected and how much they are affected. In rare cases, scleroderma may affect only some internal system, leaving the skin and joints untouched.</I><FONT SIZE="3" COLOR="#000000" FACE="Arial" ><I> </I></FONT></div> <div> <I>There are two types of generalized scleroderma: </I><I><B>Limited (also called the</B></I><I> </I><I><B>CREST Syndrome)</B></I><I> and </I><I><B>Diffuse.</B></I><FONT SIZE="3" COLOR="#000000" FACE="Arial" ><I><B> </B></I></FONT></div> <bR> <div> <I><B>CREST </B></I><I>stands for a combination of symptoms: Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia. This type usually has a slow onset, with the first symptoms appearing 10 to 20 years before the full syndrome occurs. It usually affects the skin on the face, fingers, and hands. Later on, it may affect internal organs, such as the esophagus (the tube leading from the mouth to the stomach), the lungs and bowels.</I><FONT SIZE="3" COLOR="#000000" FACE="Arial" ><I> </I></FONT></div> <DIV ALIGN="LEFT"></DIV> <div> <I><B>DIFFUSE</B></I><FONT SIZE="3" COLOR="#000000" FACE="Arial" > </FONT></div> <bR> <div> <I>Diffuse occurs throughout the body. It usually affects the skin as well as other body parts, such as the lungs, kidneys, heart, bowels, blood vessels and joints. Depending on the areas affected, this type can cause problems such as high blood pressure, muscle weakness, trouble swallowing, or shortness of breath.</I><FONT SIZE="3" COLOR="#000000" FACE="Arial" ><I> </I></FONT></div> <bR> <div> <I>Diffuse scleroderma may progress slowly in some people and more rapidly in others. However, with proper management, it can be controlled.</I><FONT SIZE="3" COLOR="#000000" FACE="Arial" ><I> </I></FONT></div> <bR> <bR> </td> </tr></table></body>