A short obese woman with a cushingoid appearance has a history of painful spontaneous ecchymoses on her legs and arms. Her medical history is significant for hypertension, bipolar depression, sleep apnea, and chronic joint and musculoskeletal pain attributed to fibromyalgia. Laboratory evaluation reveals an erythrocyte sedimentation rate of 60 mm/hr. A complete blood count, bleeding times, von Willebrand testing, dexamethasone suppression testing, vitamin C level, antinuclear antibody determination, and rheumatoid screens with anti-cyclic citrullinated peptide (anti-CCP) testing were unremarkable. Antineutrophil cytoplasmic antibodies (both perinuclear and cytoplasmic) were negative, ruling out vasculitis.
Subcutaneous nodules biopsied in the office were negative. I have considered amyloidosis and sarcoidosis, but I am at a loss. I eagerly await any suggestions.
—John Spence, MD, Marianna, Fla.
I would suggest asking about other evidence of a bleeding diathesis, such as bleeding after surgery, epistaxis, gum bleeding, or menorrhagia. The physical exam can narrow the workup, as mucosal bleeding, petechiae, and small superficial ecchymoses are typically due to platelet disorders while large palpable ecchymoses and deep muscle hematomas suggest coagulation problems. The physical exam is also helpful to look for an underlying connective tissue disorder, such as Ehlers-Danlos syndrome. I would then scrutinize the patient’s medication list for possible culprits. Selective serotonin reuptake inhibitors, for example, can disrupt normal platelet aggregation by blocking uptake of serotonin into platelets. Herbal remedies, such as garlic and fish oil, and OTC medications, such as nonsteroidal anti-inflammatory drugs and aspirin, can also lead to easy bruising. You said your patient’s platelet count and bleeding time are normal. Are her prothrombin time (PT) and partial thromboplastin time (PTT) normal as well? Prolongation of either suggests abnormalities in the coagulation cascade and can further guide testing.
If all her testing is negative, a diagnosis of exclusion would be psychogenic purpura, also known as Gardner-Diamond syndrome, a poorly understood and rare condition involving unexplained ecchymoses usually in the context of patients (mostly women) with mental illness. Laboratory tests, including platelet count/morphology, PT, and PTT, are normal in these patients, and the pathophysiology is not understood. Finally, I would look into the possibility of abuse, including self-inflicted injury.
—Susan Kashaf, MD, MPH (116-20)