In 1975, rodnan et al 2 proposed the name eosinophilic fasciitis after evaluating a laboratorial aspect of the disease. Upshawshulman syndrome definition of upshawshulman. Successful treatment of severe shulmans syndrome by. Micro syndrome is an autosomal recessive disorder that is characterised by mental retardation, microcephaly, congenital cataract, microcornea, microphthalmia, agenesishypoplasia of the corpus callosum and hypogenitalism. In 1975, shulman described eosinophilic fasciitis ef as a rare connective tissue disease characterized by a symmetrical and painful swelling with a progressive induration of the skin and soft tissues 1, 2. Ramsay hunt syndrome in a patient with metastatic lung cancer to brain. Eosinophilic fasciitis is a skin disease that causes thickening and inflammation of the skin and fascia. Shulmans practice includes both adult and pediatric retinal disorders, as well as posterior uveitis. What is shulmans syndrome with 3 ideas on treating it. Shapiroshulman and sturgeweber syndromes pediatric neurology. Treatment for eosinophilic fasciitis aims to eliminate inflammation through the use of aspirin, nsaids, and cortisone. We report a case of bilateral lupus choroidopathy and central nervous system cns vasculitis in a 43yearold female with systemic lupus erythematous.
In 1974 shulman described a rare sclerodermalike syndrome designated as eosinophilic fasciitis ef. Fasciitis with hypereosinophilia shulman syndrome in a 34 year old woman. Eosinophilic fasciitis shulman s syndrome center eosinophilic fasciitis is a skin disease that causes thickening and inflammation of the skin and fascia. Eosinophilic fasciitis ef, also called shulman syndrome is an uncommon disorder of unknown etiology and poorly understood pathogenesis. Also known as moschcowitzs disease explanation of shulman upshaw syndrome. Given the central role of recurrent abdominal pain in ibs, we evaluated the relationships of pediatric ibs and abdominal pain with intestinal microbes and fecal metabolites using a comprehensive clinical characterization and multiomics strategy. Jean schulman, md, a highly rated medical oncology specialist in peoria, az. Unlike other diseases in that category, it is limited to the arms and legs, and usually resolves. The onset of illness has been associated with a period of unusual physical exertion. This leads to symptoms of progressive thickening and often redness, warmth, and hardness of the skin surface.
Shulman treats this condition more than 96% of their peers view all cardiac care treatments check dr. These manifestations are replaced by fascial fibrosis, leading to a cobblestone appearance or peau dorange. Immunophenotyping of blood lymphocytes in childhood. Also known as moschcowitzs disease explanation of shulmanupshaw syndrome. A syndrome characterised by acute onset of erythema and later induration of the connective tissues of the extremities, but sparing the hands, usually appearing following exertion. Kawasaki syndrome is an acute, selflimited vasculitis that occurs in children of all ages and presents a challenge for the clinician. Symptoms include redness, warmth, and hardening of the skin, as well as occasional tissue and joint pain. Nov 12, 2015 eosinophilic fasciitis is a very rare condition in which muscle tissue underneath the skin, called fascia, becomes swollen and thick. Eosinophilic fasciitis radiology reference article.
Intrinsic abnormalities of lymphocyte counts in children with down syndrome. Leveraging human microbiome features to diagnose and. Upshawschulman syndrome uss is the recessively inherited form of thrombotic thrombocytopenic purpura ttp, a rare and complex blood coagulation disease. Meet julia shulman, md nyc retina retina specialists. Shulman is a boardcertified ophthalmologist and vitreoretinal surgeon. Shulman syndrome definition of shulman syndrome by. Eosinophils are a particular type of white blood cells, usually representing a small percentage less than 8% of the total white blood cell population. Eosinophilic fasciitis, also known as shulman syndrome, is named after the physician who, in 1974, was the first to report on the disorder in the medical literature. Eosinophilic fasciitis shulmans syndrome center by. Department of health and human services center for disease control and prevention. Shapiroshulman and sturgeweber syndromes pediatric. The shulman syndrome is a symptom complex recently described in the rheumatology literature that is characterized by eosinophilia, hypergammaglobulinemia, and a diffuse sclerodermalike process of the extremities.
Eosinophilic fasciitis ef, also known as shulman syndrome, is an uncommon connective tissue disorder. Eosinophilic fasciitis shulmans syndrome medicinenet. Diagnosis, grading, and treatment recommendations for. We describe a patient with severe shulmans syndrome shs eosinophilic fasciitis. Eosinophilic fasciitis is a rare disease that leads to inflammation and thickening of the skin and fascia underneath. We report on a girl with cutaneous angioma and hydrocephalus who presented the characteristics of the condition described by shapiro and shulman. Choroiditis flared and escalation of therapy with chemo therapy drug achieved cns disease control. These manifestations are replaced by fascial fibrosis, leading to a cobblestone appearance or peau dorange, as. In the absence of international diagnostic criteria, diagnosis of ef is now based on the association of characteristic skin or subcutaneous abnormalities and a thickened. Aggressive forms of eosinophilic fasciitis may require the use of.
Shulmans syndrome is a rare disorder in which the tissues just beneath the skin get inflammation. The number of these cells eosinophil count increases in certain illnesses, including allergies, asthma, addisons disease, sarcoidosis, parasite. People with this condition have a buildup of eosinophils, a type of white blood cell, in the affected fascia and muscles. Shulmanupshaw syndrome article about shulmanupshaw. Shulman s experience treating your condition or procedure. Laboratory findings are eosinophilia, hypergammaglobulinaemia and a raised erythrocyte rate. Shulman pdf shulman builds his foundation for teaching reform on an idea of teaching that emphasizes comprehension and reasoning, transformation and re. Magnetic resonance angiography revealed anomalous intracranial venous drainage, which led to.
Accurate diagnosis and stratification of children with irritable bowel syndrome ibs remain challenging. Shulman graduated from mount sinai school of medicine in new york. In 1976, shapiro and shulman described a condition consisting of bilateral facial nevi involving the lower face and upper cervical areas, macrocrania, cephalic venous hypertension, and anomalous intracranial venous return 1. Mim226350 induration and edema of the connective tissues of the extremities, usually appearing following exertion. Eosinophilic fasciitis is rare disease characterized by subacute onset of erythema, edema, and induration of the skin and soft tissues of the limbs and trunk. We read with great interest the report by prats vinas et al. Rapid swelling can occur in the hands, arms, legs, and feet. At birth, she manifested extensive pink discoloration on her face, scalp, and back. When accompanied by a decrease in the circulating platelets, it is. In patients with eosinophilic fasciitis, the involved fascia is inflamed with the eosinophil type of white blood cells. The full text of this article is available in pdf format.
The patient improved systemically on high dose steroids and liver transplant drug. Joel shulman, md book an appointment fort lauderdale, fl. Shulman syndrome, first described in 1974 by shulman, 1 is characterized by a sudden onset of a symmetrical edema with induration at the extremities. Eosinophilic fasciitis shulman syndrome mdedge dermatology. Background micro syndrome was first reported by warburg et al. David lebeaux, camille frances, stephane barete, bertrand wechsler, odile dubourg, jerome renoux, thierry maisonobe, olivier benveniste, marc gatfosse, pierre bourgeois, zahir amoura, patrice cacoub, jeancharles piette, damien sene, eosinophilic fasciitis shulman disease. Some researchers believe that eosinophilic fasciitis is a variant of scleroderma systemic sclerosis, an autoimmune connective tissue disorder characterized by hardening of the skin. We present a case of eosinophilic fasciitis, or shulman syndrome, in a 35yearold man and discuss its clinical and histopathologic aspects, as well as its relationship to scleroderma. During infancy, she developed hydrocephalus, which later stabilized. This causes the skin to swell and slowly thicken and harden. The shulman syndrome jama dermatology jama network.
Shulman syndrome, is an uncommon one, evolving into sclerodermoid cutaneous infiltration, associated with peripheral. Evidence for a common pathway linking neurodegenerative diseases. Shulman syndrome definition of shulman syndrome by medical. Thrombi in blood vessels associated with deposits of hyaline substances in the walls and with thrombocytopenia. It causes a symmetrical hardening of the muscles, fascia, and skin. Unlike scleroderma, it spares the fingers and toes, exhibits elevated blood eosinophils, often onsets after prolonged strenuous exercise, and responds to treatment with systemic corticosteroids. Sinusoidal obstructive syndrome, also known as hepatic venoocclusive disease, is a potentially lifethreatening complication that occurs in children undergoing haemopoietic stemcell transplantation hsct. This autoimmune disease involved not only the skin and muscles, but the bone marrow as well thereby. Eosinophilic fasciitis is a very rare condition in which muscle tissue underneath the skin, called fascia, becomes swollen and thick. Eosinophilic fasciitis nord national organization for rare. Find out information about shulman upshaw syndrome. Differences in the incidence of genetic predisposition and clinical presentation of sinusoidal obstructive syndrome between children and adults have rendered the historical baltimore and. Ef is characterized in its early phase by limb or trunk erythema and edema and later by collagenous thickening of the subcutaneous fascia. Eosinophilic fasciitis is a very rare sclerodermalike illness.