People are commonly misdiagnosed with fibromyalgia, bleeding disorders, or other disorders that can mimic EDS symptoms. Because of these similar disorders and complications that can arise from an unmonitored case of EDS, a correct diagnosis is important. Pseudoxanthoma elasticum is worth consideration in diagnosis. Management
No cure for Ehlers–Danlos syndromes is known, and treatment is supportive. Close monitoring of the cardiovascular system, physiotherapy, occupational therapy, and orthopedic instruments (e.g., wheelchairs, bracing, casting) may be helpful. This can help stabilize the joints and prevent injury. Orthopedic instruments are helpful for the prevention of further joint damage, especially for long distances. People should avoid activities that cause the joint to lock or overextend.A physician may prescribe casting to stabilize joints. Physicians may refer a person to an orthotist for orthotic treatment (bracing). Physicians may also consult a physical and/or occupational therapist to help strengthen muscles and to teach people how to properly use and preserve their joints.Aquatic therapy promotes muscular development and coordination. With manual therapy, the joint is gently mobilized within the range of motion and/or manipulations. If conservative therapy is not helpful, surgical joint repair may be necessary. Medication to decrease pain or manage cardiac, digestive, or other related conditions may be prescribed. To decrease bruising and improve wound healing, some people have responded to vitamin C. Medical care workers often take special precautions because of the sheer number of complications that tend to arise in people with EDS.
In HS, the erythrocytes fail to pass through fenestrations, and this is where resident splenic macrophages sample, or "bite," part of the stuck spherocytes plasma membranes. The macrophages recognize that the spherocyte is not normal, and the macrophage is "programmed" to destroy irregularities filtered at the spleen from the blood. The resident splenic macrophages therefore phagocytose the spherocytes, causing extravascular hemolysis. This leads to both splenomegaly and anemia. Should this process continue unchecked chronically, inappropriate regulation of erythropoeisis leads to extramedullary hematopoiesis. Clinical Presentation
HS patients present in a vast array of presentations from being asymptomatic, to the extreme situations of splenic rupture and/or hemolytic crisis, or in-utero demise. Asymptomatic HS (mild): 20-30% of patients. Infantile-Onset HS (moderate): 60-75% of patients. Neonatal or In-Utero Onset HS (severe): <5% of patients.The most common presentation will demonstrate jaundice (due to increased unconjugated bilirubin), anemia (with secondary pallor), and a palpable spleen sometimes with concomitant tenderness (due to splenic congestion and splenomegaly). Its worth noting that a subsection of HS patients will also have incidental black pigmented gallstones (made of calcium bilirubinate consequence of the extravascular hemolysis), and some of these patients will develop cholelithiasis and/or the potential complex sequelae of this condition, i.e. cholecystisis, choledocholithiasis, etc. Diagnostics
Laboratory Testing
Available lab testing that may aid in the diagnosis of HS is as follows:

Coombs Test
Osmotic Fragility Test
Acidified Glycerol Lysis Test
Supportive blood work: mean cell volume (MCV), mean corpuscular hemoglobin concentration (MCHC), red blood cell distribution width (RDW), red blood cell count (RBC), reticulocytes, unconjugated bilirubin, haptoglobin, lactate dehydrogenase (LDH).