systemic exanthematous histiocytosis
Introduction
Introduction to systemic rash histiocytosis General Eruptive Histiocytosis (GEH) is a papular, non-lipid, self-healing histiocytosis that occurs primarily in adults. In 1963, Winkelmann and Muller first reported an adult. In 1987, Paputo et al. reported a series of children's cases. The disease is extremely rare. There are about 30 cases reported in the literature, of which 7 are children. basic knowledge The proportion of illness: 0.001% Susceptible people: no specific population Mode of infection: non-infectious Complications: swelling
Cause
Systemic eruptive histiocytosis
(1) Causes of the disease
The cause is unknown.
(two) pathogenesis
The pathogenesis is still unclear. The clinical manifestations and histopathological features of this disease are closely related to benign head histiocytosis, disseminated xanthomas and yellow granuloma.
Prevention
Systemic rash histiocytosis prevention
The etiology of this disease is not clear, and may have certain correlation with environmental factors, genetic factors, dietary factors, and mood and nutrition during pregnancy. Therefore, it is impossible to directly prevent the disease against the cause. Early detection, early diagnosis, and early treatment are important for indirect prevention of this disease, and can also reduce the incidence of infection complications. For patients with existing infections, antibiotics should be used as soon as possible.
Complication
Systemic rash histiocytosis complications Complications swelling
The disease is extremely rare, mainly skin damage. Complications are common in patients with skin ulceration and low constitution or long-term use of immunosuppressive agents. Due to skin mucosal damage, skin integrity is destroyed, so patients may be scratched. Inducing skin bacterial infections or fungal infections, usually secondary to low body constitution, or long-term use of immunosuppressive agents and fungal infections such as onychomycosis, such as concurrent bacterial infections may have fever, skin swelling, ulceration and purulent secretion logistics Out of performance. Severe cases can lead to sepsis, which should be brought to the attention of clinicians.
Symptom
Systemic rash histiocytosis symptoms common symptoms histiocytosis papule nodules
May occur at any age, the lesions are composed of asymptomatic round or oval rash or nodules, yellow or dark blue, solid nature, size 3 ~ 10mm, lesions appear in groups, the number can be up to Hundreds, adult skin lesions, symmetrically distributed in the trunk and limbs, can even invade the mucosa, children's skin lesions are irregularly distributed throughout the body, mucous membranes are not invaded, adults and children do not involve the internal organs, skin lesions can continue for several years, can gradually Regressed.
Examine
Systemic rash histiocytosis
Histopathology: monocular tissue cells and a few lymphocytes infiltrated in the dermal papilla and dermis, no multinucleated giant cells, nuclear chromatin is rare, cytoplasm is abundant, lightly stained, and the cytoplasm is unclear. These cells are often in blood vessels. Surrounded by nesting, immunohistochemical staining of infiltrating cells S-100 protein and CD1a (OKT-6) negative, CD-11b and CD-4 positive, ultramicroscopic examination showed that tumor cells contain a large number of clusters of dense and regular layers Small body, even visible small body, no Birbeck particles, tissue cell lipid staining negative.
Diagnosis
Diagnosis and identification of systemic eruptive histiocytosis
diagnosis
Diagnosis based on clinical manifestations and medical history.
Differential diagnosis
1. Juvenile yellow granuloma can be identified according to the color and histopathology of the lesion and the foam cells and Touton cells.
2. Papular xanthoma with juvenile yellow granuloma.
3. Multi-center reticular cell hyperplasia This disease is associated with arthritis, and the tissue is characterized by a large number of multinucleated giant cells.
4. Benign head histiocytosis This disease is confined to the head and face, only in children, can be distinguished from GEH.
5. Langerhans cell histiocytosis can be identified according to the clinical features and typical tissue images of the disease, immunohistochemistry and electron microscopy.
