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Papules, especially on the head, are covered with yellowish-gray, greasy to the touch scale-crusts. Patients are usually misdiagnosed with seborrheic eczema.

In the future, necrosis appears in the center of the papules, followed by the formation of scars.

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In addition to the scalp, back, chest, papular and petechial rash can also be localized on the lower abdomen and thighs. In combination with petechiae, lichenoid papules and punctate scars, infiltrative tumor-like formations can be observed. Hemorrhagic erosions asacol pills and on the genitals are often noted.

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Sometimes changes on the skin are accompanied by weeping and severe itching, serous and hemorrhagic crusts.

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Paronychia and onychia, onycholysis, subungual hyperkeratosis, subungual purpura of some or all of the nails of the hands and feet may occur. Nail involvement is one of the most unfavorable prognostic signs in histiocytosis X. Children always have ear lesions in the form of asacol pills otitis media, mastoiditis. Peripheral lymph nodes range in size from a pea to a bean and are larger.

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Enlarged liver and spleen. The blood revealed hypochromic anemia with aniso- and poikilocytosis, hypochromia, leukopenia, neutrophilia with a stab shift to the left, increased ESR, monocytosis, thrombocytopenia, decreased clotting time and bleeding. The myelogram shows reticulohistiocytic cells, an increase in young (blast) forms. An mesalamine 400 mg examination reveals numerous foci of destruction of the bones of the skull, limbs, flat bones of the pelvis, ribs, and sometimes shoulder blades. Decalcification zones and osteolysis are noted.

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The existence of transitional forms of mesalamine online is possible, when signs of Hand-Schüller-Christian disease (thirst, polyuria, exophthalmos) appear against the background of Abt-Letterer-Siwe disease. If it is difficult to diagnose Abt-Letterer-Siwe disease, it is necessary to conduct histological studies of the affected skin foci and lymph nodes. In fresh foci of the affected skin, a proliferative reaction is detected in the form of the presence in the dermal infiltrate of a large number of large histiocytes with bubble-like nuclei, lymphocytes. Some histiocytes have a foamy appearance, the reaction to lipids in them is positive. Last update of the page: 20.09.2011 Feedback Sitemap.

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The information presented on the site should not be used for self-diagnosis and treatment and cannot serve as a substitute for an in-person consultation with a dermatologist. Abt-Letterer-Siwe disease is called acute systemic progressive histiocytosis, which has a hereditary cause. Most often, the disease begins to develop in early childhood, but occasionally its first symptoms appear only during puberty. It is believed that Abt-Letterer-Siwe disease is more common in boys than girls.

Why Abt-Letterer-Siwe disease occurs The disease is primarily due to a genetic mutational factor, however, the onset of the disease is usually associated with the presence of a viral or bacterial infection (tonsillitis, influenza, otitis media, etc.). An unusual course of the disease indicates its neoplastic nature.

With the development of Abt-Letterer-Siwe disease, the general condition of the baby deteriorates sharply.

How Abt-Letterer-Siwa disease proceeds Most often, Abt-Letterer-Siwe disease begins in the first year of a child's life, sometimes it happens even during the neonatal period. Suddenly, the baby's body temperature rises sharply (up to 40 degrees), after which the temperature can either rise or fall. Sometimes for a long time the temperature remains subfebrile. Hemorrhagic papules can be seen on the scalp and skin of the shoulder blades, abdomen and chest. Necrosis develops in their center,turning into the formation of small dotted scars. Often, small erosions and hemorrhages are noted on the mucous membranes of the mouth and genital organs.

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In patients with Abt-Letterer-Siwe disease, changes are also observed in the respiratory organs: emphysematous, small-focal, infiltrative.

Patients often develop severe polyadenopathy and hepatosplenomegaly. Quite often, otitis media and mastoiditis are diagnosed, their distinguishing feature is resistance to antibiotic treatment. Many children suffering from Abt-Letterer-Siwe disease have damage to the bone tissue of the skull, ribs, collarbones, shoulder blades, and pelvis. Often this is accompanied by pain, swelling in the surrounding tissues. A study using fluoroscopy can reveal foci of osteoporosis, osteolysis, periostitis, decalcification.

Low weight gain, pale skin, weakness are especially characteristic of this disease. A blood test also shows a pathological picture: a combination of hypochromic anemia, leukopenia or leukocytosis, monocytosis. The myelogram shows polymorphism of bone marrow cells.

How Abt-Letterer-Siva disease is treated One of the main methods of treatment is the appointment of glucocorticoids, which are designed to inhibit the function of the reticulohistiocytic system. Such treatment stimulates hematopoiesis and normalizes metabolism.

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AMY KIRSZTAJN

Usually, courses of treatment with glucocorticoids are quite long, the dose of the drug must be gradually reduced before the end of treatment, sometimes it takes several months, but it can often be years. It is important that drug withdrawal be completed when the body enters remission, not earlier.


AMBER FRETWELL

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Children suffering from Abt-Letterer-Siwe disease are prescribed increased doses of glucocoricoids, and antibiotics are also prescribed in case of viral and bacterial diseases, such as measles, scarlet fever, chicken pox, SARS. This feature of treatment is dictated by the fact that Abt-Letterer-Siwe disease tends to worsen during such periods.

DAWN HENSON

JESSICA DONOHUE

Sometimes the doctor may prescribe mesalamine drugs, anabolic drugs, gamma globulin, vitamin therapy. According to the indications, X-ray therapy is prescribed for the affected bone tissue. You can see the comments or write your own. Abt-Letterer-Siwe disease, reticulohistiocytosis in children.

 

 

KATE WALKER

Abt-Letterer-Sive disease (true reticulohistiocytosis) was first described by Letterer in 1924. In 1933, Sive made a detailed report on the clinical picture, and in 1936 Abt summarized the results of his own research and literature data regarding acute reticulohistiocytosis in children. Therefore, the disease is called Abt-Letterer-Siwe disease. A number of asacol for this disease are used in the literature: diffuse reticuloendotheliosis, aleukemic reticulosis, histiomonocytic acute malignant reticulosis, non-lipid reticulosis, acute reticuloendotheliosis, histiocytosis X, etc.

 

LILY CIANCUTTI

The etiology and pathogenesis of reticulohistiocytosis are not completely known. The possibility of a viral or bacterial infection is assumed (in the anamnesis of children, infections are often noted - tonsillitis, otitis media, sepsis, etc.). The steadily progressive course of the disease may indicate the neoplastic nature of the process. A. M. Abezgauz believed that the genetic mutation factor is of great importance in the pathogenesis of the disease.

LAUREN CRUTSINGER

Symptoms of asacol pills. The disease usually begins in infancy, sometimes even in the neonatal period, with a sudden rise in temperature to 40 ° C. Then the temperature curve acquires a hectic character, but sometimes it can be subfebrile. On the scalp, in the area of ​​the shoulder blades, abdomen and chest, a lot of hemorrhagic scaly lichenoid papules and petechial elements appear. In the center of the papules, necrosis occurs, followed by the formation of dotted scars; sometimes infiltrative-tumor formations are observed, leaving larger scars. Some patients with Abt-Letterer-Siwe disease may have onychia and paronychia. Erosions and hemorrhages are often noted on the mucous membranes of the mouth and vulva.

MELISSA LAWTON

Characteristic features are pronounced polyadenopathy and hepatosplenomegaly. Otitis and mastoiditis are often noted, which are resistant to antibiotic therapy and surgical intervention. In most patients, the bones of the skull, ribs, collarbones, shoulder blades, limbs, and pelvis are affected, which is accompanied by pain and swelling of the soft tissues. On radiographs, foci of asacol, osteoporosis, osteolysis, decalcification zones of a cystic nature, periostitis are determined. There may be changes in the lungs of an infiltrative, small-focal and emphysematous nature, which are less often detected physically, but mainly radiologically (increased basal pattern, formation of cystic cavities, thickening of the walls of the bronchi, the presence of emphysema vesicles).

 

SHELBY HOWARD

The general condition of the child deteriorates rather quickly, appetite disappears, the skin becomes pale, poor weight gain is noted,weakness. In the peripheral blood: hypochromic anemia with aniso- and poikilocytosis, leukopenia or leukocytosis with neutrophilia and a left shift in the leukocyte formula to myelocytes; some patients have monocytosis. The myelogram is characterized by polymorphism of bone marrow cells, sometimes the content of reticulohistiocytic elements is increased.

 

SAMA HROMNIK

Histopathology: in the skin foci, clusters of histiocytes are found, among which there are lymphocytes and eosinophils. The protoplasm of some histiocytes is foamy, stained with fat. The epidermis can be destroyed due to the pressure of the underlying skin layers. In the visceral organs, normal structures are replaced by a proliferate of large pale reticulohistiocytic cells.

 

TOSHIA COOPER

In cytological studies made using a puncture biopsy, 4 types of cells were found in smears among erythrocytes, leukocyte and reticulohistiocytic elements. K. A. Moskacheva refers to the first type as small cells, often of irregular shape, with mesh or homogeneous protoplasm and usually loose nuclei. The second type is multinucleated (from 4 to 7 nuclei) cells, the nuclei are predominantly round and loose. The third type includes cells containing large, medium and small vacuoles. Cells of the fourth type are represented by macrophages, among which there are multinucleated giants.


Greg Ridout

 


ROBIN DETTERMAN


NATHANIEL FOSTER


MICHAEL ALONSO

JANET BRIGGS


KEN BERRICK


LETICIA GALYEAN