Down syndrome trisomy 21 syndrome goldenhar syndrome. Handschullerchristian disease bhargava d, bhargava k, hazarey. Nonlangerhans cell histiocytosis refers to a family of histiocytosis characterized by the absence of langerhans cells. It is associated with a triad of exophthalmos, lytic bone lesions often in the skull, and diabetes insipidus from pituitary stalk infiltration. Langerhans cell histiocytosis hematology and oncology merck. Handschullerchristian disease and erdheimchester disease. Letterersiwe disease, hand schuller christian disease, and eosinophilic granuloma. The clinical presentation of langerhans cell histiocytosis lch depends on the extent of dissemination. Hand schuller christian disease with a solitary swelling over left parietooccipital region of scalp, and increased thirst with polyuria. Crouzon syndrome craniofacial dysotosis dandy walker syndrome. It is usually manifested in childhood as well as in adulthood. Insipidus, exophthalmos and lytic bonetriad of diab. Cri du chat syndrome chromosome 5p syndrome criglernajjar syndrome. Related words hand schuller christian disease synonyms, antonyms, hypernyms and hyponyms.
Handschullerchristian disease an overview sciencedirect topics. Handschullerchristian disease a condition of multiple destructive histiocytosis of bone causing skull defects, pituitary gland disturbances with diabetes insipidus, middle ear infection and loss of teeth. Because these syndromes may be varied manifestations of the same. This is a rare case of hand schuller christian disease and affecting both the twin male children is rarest among the rare.
Tosssurvey of handschuller christiandisease in otolaryngology. The disease was first described in 1953 by lichtenstein under the term histiocytosis x. Get a printable copy pdf file of the complete article 1. As a suspicion of hand schiiller christian disease was entertained, decompression of the facial nerve and middle ear hygiene were omitted. The three classic symptoms are softened areas of the skull and other. Sep 07, 2018 histiocytosis, hand schuller christian. Insipidus, exophthalmos and lytic bone lesions is called hand schuller christian diseaselesions is called hand schuller christian disease 8. Because of its rarity, lch remains poorly understood. Schullerchristian disease article about schullerchristian. It is associated with a triad of exophthalmos, lytic bone lesions. Nelson md, faaos, carolyn taliaferro blauvelt, in a manual of.
In view of the extreme rarity of the condition, reference is made to the relevant literature on the subject. Langerhans cell histiocytosis, erdheimchester disease, handschullerchristian disease, central diabetes insipidus. Langerhanscell histiocytosis which can take the form of histiocytosis x, eosinophilic granuloma, abtletterersiwe disease, and hand schuller christian disease may involve the bone, lung, liver, hypothalamus, and pituitary 1. Handschullerchristian disease article about handschuller. Hashimotopritzker disease, also known as congenital selfhealing reticulohistiocytosis, is a very rare disease characterized by single or multiple redpurple or brown pimples papules and lumps nodules present at birth congenital or soon thereafter. Hashimotopritzker syndrome genetic and rare diseases.
Multifocal eosinophilic granuloma in an adult with 20year follow up. Unifocal bony lch is characterized by the development of solitary osseous lesions at any site. Hidrops vesicular pdf after en bloc resection of the tumor, the orbital walls were reconstructed with outertable calvarial bone grafts. Cerebellar dysfunction in handschullerchristian disease. Churchill livingstone, london eckardt a, schultze a. There were no bone involvement and no signs of leterrersiwe or handschuller christian histiocytosis without exophthalmos, skin rash or geographic skull on. It occurs extremely rarely in adults with the frequency estimated to be one in 560,000 adult persons. Histiocytosis x is a clonal proliferation disease of langerhans cells that express an immunophenotype positive for s100 and cd1a 7 hand schuller christian disease is a well described entity which has appeared sporadically till 1935. Full text full text is available as a scanned copy of the original print version. The peak incidence of the disorder is between 5 and 10 years of age. Classic signs and symptoms include congenital or early development of painless papules, nodules or plaques with spontaneous regression in 2.
Handschullerchristian disease uncountable a condition associated with multifocal langerhans cell histiocytosis and a triad of exophthalmos, lytic bone lesions often in the skull, and diabetes insipidus. The disease usually affects children age 2 to 5, less often older children and adults. Full text is available as a scanned copy of the original print version. Meaning of hand schuller christian disease with illustrations and photos. Handschullerchristian disease is associated with multifocal langerhans cell histiocytosis it is associated with a triad of exophthalmos, lytic bone lesions often in the skull, and diabetes insipidus from pituitary stalk infiltration. Discussion hand schuller christian disease is a chronic disseminated form of histiocytosis x, in which idiopathic nonneoplastic proliferation of histiocytes occurs. Cite the keys to diagnosis of handschullerchristian disease in a patient with only central diabetes insipidus.
Langerhan cell histiocytosis, formerly known as histiocytosis x, traditionally denotes a group of diseases that stem from proliferative reticuloendothelial. Handschullerchristian disease with condylar aplasia journal of. In the pediatric versions, it depends on the actual disease category and severity of the disease. This disorder has been known as histiocytosis x, hand schuller christian disease, abtletterer siwe disease, and eosinophilic granuloma. She underwent excision biopsy of the right frontal skull lesion which was consistent with histiocytosis x. Handschullerchristian disease is a wellrecognized form, characterized by. Handschullerchristian disease american journal of medicine. It primarily affects children, less often young adults, and rarely older adults.
Once the diagnosis of hand schuller christian disease with secondary diabetes insipidus was made, the patient was started on a regimen of intramuscular pitressin and radiation therapy to the skull, rib, and pelvic lesions. A group of rare disorders in which too many langerhans cells a type of white blood cell grow in chrietian tissues and organs including the enfemredad, skin, and lungs, and damage them. Handschullerchristian disease radiology reference article. Hand schuller christian disease synonyms, hand schuller christian disease pronunciation, hand schuller christian disease translation, english dictionary definition of hand schuller christian disease. The incidence appears to be 35 cases per million children. Langerhans cell histiocytosis lch is disorders which include abnormalities that result from abnormal proliferation of langerhans cells or. Ideal sources for wikipedias health content are defined in the guideline wikipedia. Tosssurvey of hand schuller christian disease in otolaryngology. Named after alfred hand, artur schuller, and henry asbury christian. Handschullerchristian disease definition, etymology and. Get a printable copy pdf file of the complete article 2. Maw is an oral surgeon in the us navy dental corps with the rank of commander. Pronunciation of hand schuller christian disease and its etymology. Handschullerchristian disease treated with cortisone.
Involvement of other areas of the central nervous system cns has been reported infrequently. Microscopic examination of granulation tissue from the antrum showed large. The patient is now being followed on an outpatient basis, and 15 months following the last surgical procedure no further deterioration in the oral condition has been seen. It is named for the american pediatrician alfred hand jr. Selfhealing langerhans cell histiocytosis lch of the skin means that the disease regressed without. Less than 15% of patients with handschullerchristian disease or multifocal eosinophilic. A disease in which histiocytes start to multiply and attack the tissues or organs of the patient. Langerhans cell histiocytosis lch and erdheimchester disease ecd share similar clinical features and mechanisms. Individuals with these granulomatous lesions described in the initial reports of hand, 1 schuller 2 and christian 3 had a common triad of findings consisting of osteolytic defects of membranous bone skull, diabetes insipidus, and exophthalmos. Handschullerchristian disease is associated with multifocal langerhans cell histiocytosis. The most frequent sites of bony involvement are the flat bones of the skull, ribs, pelvis, and scapula wing bone. Longterm remission following methotrexate therapy in a. Neoplastiform xanthomatous granulomas of choroid plexus in a child affected by handschullerchristian disease.
A childhood syndrome characterized by exopthalmos, diabetes insipidus, and softened or punchedout areas in the bones explanation of schuller christian disease. This entity encompassed a group of rare syndromes of unknown aetiology such as eosinophilic granuloma, letterersiwe disease or handschullerchristian disease 9. Alfred hand, american paediatrician, 18681949, artur schuller, austrian neurologist, 18741958, and henry asbury christian, american physician, 18761951. Here we report such a patient, who was first diagnosed with handschullerchristian disease hsc, a type of lch. Pulmonary disease in childhood is clinically variable.
Characteristics of eosinophilic granuloma pulmonary langerhans cell histiocytosis. Report illustrating hsc chronicity and diagnostic challenge. Neoplastiform xanthomatous granulomas of choroid plexus in a child affected by hand schuller christian disease case report a. The folllowing case represents a handschullerchristian disease as evidenced both by the clinical course and by the examination of biopsy. Lch provokes a nonspecific inflammatory response, which includes fever, lethargy, and weight loss. To describe the clinical case of a monthold boy with hand schuller christian disease.
Subsequent observations on similarly afflicted individuals indicate these findings may be observed. Unifocal bony lch is least common in the hands and the feet. In internet explorer, rightclick on the link and select save target as. List the signs linking a handschullerchristian disease patient to coexisting ecd. Handschullerchristian disease paediatrica indonesiana. Links to pubmed are also available for selected references. Full text get a printable copy pdf file of the complete article 241k, or click on a page image below to browse page by page.
To describe the clinical case of a monthold boy with handschullerchristian disease. Definition of hand schuller christian disease in the fine dictionary. There is involvement of multiple sites in one organ system, most frequently the bone. Pdf p classabstracthandschullerchristian disease hsc is a rare disease of unknown cause.
Langerhans cell histiocytosis lch occurs in patients when the body accumulates too many immature langerhans cells, a subset of the larger family of cells known as histiocytes. From this time the classicalsigns of the disease, now known as handschullerchristian disease, came to be accepted as exophthalmos, bony defects in the. A case of hand schuller christian syndrome seen in the pediatric wards of the medical college hospital, calicut, is reported. These lipids group together with immune cells known as histiocytes to form masses known as granulomas in various parts of the body, including the skin, skull, and many of the internal organs. The hepatopulmonary syndrome masquerading as pulmonary. We report a case of we report a case of hand schuller christian disease with a solitary swelling over left parieto. Langerhan cell histiocytosis, formerly known as histiocytosis x, traditionally denotes a group of diseases that stem from proliferative. In the early 1950s, histiocytosis x was proposed as the collective term for eosinophilic granuloma of the bone, handschullerchristian disease a clinical triad of exophthalmos, diabetes insipidus and bony skull lesions and letterersiwe disease a clinicopathological conglomerate of fever, rash, lymphadenopathy with hepatosplenomegaly. Handschullerchristian disease synonyms, handschullerchristian disease pronunciation, handschullerchristian disease translation, english dictionary definition of handschullerchristian disease.
Hand schuller christian disease treated with cortisone. Lch is clinically classified into three typeseosinophilic granuloma, hand schuller christian disease and abtletterer siwe disease. A bone biopsy of the rib lesion was consistent with a diagnosis of hand schuller christian disease. Handschullerchristian disease definition of handschuller. Handschullerchristian disease a multifocal, unisystem form of langerhanscell histiocytosis. Handschullerchristian hsc disease is a rare proliferative disorder in which pathological langerhans cells lcs. Maw, dds huntington beach, calif huntington beach calif dr. A patient with handschullerchristian disease which is a subtype of langerhans cell histiocytosis. Handschullerchristian disease is a clinically intermediate form of a spectrum of histiocytic disorders, which ranges from acute fulminant to chronic indolent disease. Handschullerchristian disease with occult diabetes. Hand schuller christian disease hsc is a rare disease of unknown cause. Hand schuller christian disease hsc is one of the three. We use your linkedin profile and activity data to personalize ads and to show you more relevant ads. Brain mri reveals destructive lesion of the sphenoid wing on the right side with thickening of the pituitary stalk and enhancing soft tissue in the sella and along the posterior aspect of the clivus.
Prompt diagnosis and treatment of the disease will improve. Hand schuller christian disease is associated with multifocal langerhans cell histiocytosis. Handschullerchristian disease with involvement of the. Public health service grants am 14039, hd 08555, and rr40. In netscape, rightclick on the link and select save link as. Very few cases have been reported in individuals past their fifth decade. Involvement of bone marrow, liver, and lung are regarded as highrisk factors. Pulmonary failure is the leading cause of death in longterm survivors 2.
We report a case diagnosed and successfully treated in our hospital. Neoplastiform xanthomatous granulomas of choroid plexus in. This article about a disease, disorder, or medical condition is a stub. Neoplastiform xanthomatous granulomas of choroid plexus in a. Multifocal eosinophilic granuloma in an adult with 20year. There were no pathological changes along the vertical course of the facial nerve, but granulations beneath the incus and along the horizontal course of the facial nerve. A langerhans cell is a type of white blood cell that normally helps the body fight infection. Get a printable copy pdf file of the complete article 606k, or click on a page image below to browse page by page. If you would like to save the pdf file to your computer, follow these steps. Handschullerchristian disease, an intermediate chronic form with multiple lesions characterized by diabetes insipidus, bulging of the eye and localized lesions in the bone eosinophilic granuloma, a less severe form, characterized by solitary or few, and chronic lesions of bone or other organs.
Handschuller christian disease american journal of medicine. Langerhans cell histiocytosis genetic and rare diseases. Eosinophilic granuloma, also known as pulmonary langerhans cell histiocytosis plch, is a rare histiocytic disorder in which the incidence is unknown table 65. Handschullerchristian disease is a rare disorder that causes lipids to develop inside the body. Get a printable copy pdf file of the complete article 745k, or click on a page image below to browse page by page. A case of handschullerchristian disease with involvement of the dental pulp has been presented. Mar 04, 2020 hand schuller christian disease is a rare disorder that causes lipids to develop inside the body. Find out information about schuller christian disease. In very rare circumstances, the two diseases coexist in the same patient. Feb 19, 2005 in pulmonary histiocytosis, the disease stabilizes or improves in about onehalf of the affected people and the other half progress to a permanent loss of lung function. We here describe an unusual case of multifocal eosinophilic granuloma hand schullerchristian disease in a middleaged woman.
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