Congenital self-healing Langerhans histiocytosis

Introduction to congenital self-healing Langerhans cell histiocytosis Congenital Self-Healing Langerhans Histiocytosis (CSLH), also known as congenital self-healing reticulocyte hyperplasia. This disease was reported by Hashimoto and Pritzker in 1973, hence the name Hashimoto-Pritzker disease. Self-limiting, benign, only skin lesions without systemic damage; its skin lesion characteristics and histopathology, in fact, similar to Langerhans cell histiocytosis, immunohistochemical examination of S-100 protein CDIa (OKT6) was also positive, and Birbeck granules were also seen in ultramicroscopic examination; thus, the disease was included in the most benign form of the Langerhans cell histiocytosis spectrum. basic knowledge The proportion of illness: 0.003%--0.005% Susceptible people: good for infants and young children Mode of infection: non-infectious Complications: sepsis

Congenital self-healing Langerhans histiocytosis

(1) Causes of the disease

The cause is unclear and may be related to viral immunology and new organisms.

(two) pathogenesis

The pathogenesis is still unclear and may be similar to the Langerhans cell tissue test.

Congenital self-healing Langerhans cell histiocytosis prevention

The disease belongs to chromosomal diseases, and the cause of chromosomal abnormalities is unclear. It may have certain correlation with environmental factors, genetic factors, dietary factors, and mood and nutrition during pregnancy, so this disease cannot be directly prevented. Regular examination should be done during pregnancy. If the child has a tendency to develop abnormalities, chromosome screening should be done in time, and abortion should be performed in time to avoid the birth of the diseased child.

Congenital self-healing Langerhans cell histiocytosis complications Complications sepsis

The disease is mainly caused by skin damage, accompanied by itching. Because the skin integrity is destroyed, it may cause skin bacterial infection or fungal infection due to scratching, usually secondary to low body constitution, or long-term use of immunosuppressants and ash. Patients with fungal infections such as nails, such as concurrent bacterial infections, may have symptoms such as fever, swelling of the skin, ulceration, and purulent secretion. Severe cases can lead to sepsis.

Congenital self-healing Langerhans histiocytosis symptoms common symptoms nodular mucosal damage tissue cell proliferation

The disease occurs in infants and newborns. The typical damage is multiple disseminated, high-rise, solid red-brown nodules. In the weeks after birth, these damages can increase and increase, some can be quite large, and later Brownish sputum; after the ecdysis, there is a pale white atrophic scar, and in a few cases, isolated lesions develop rapidly and develop congenital spontaneous ulcerative tumors, which occur in the face, trunk or limbs. The disease is characterized by no mucosal damage. In addition to occasional liver enlargement, there is usually no systemic symptoms, no obvious hematopoietic system abnormalities, skin lesions can quickly resolve spontaneously, and physical and mental development is normal.

Congenital self-healing Langerhans cell histiocytosis examination

Histopathology: The tissue is the same as Langerhans cell histiocytosis; however, in some cases, there are more foamy macrophages mixed, Langerhans cells are located in the dermal papilla and reticular layers, sometimes To epidermotropism, a considerable amount of eosinophils are present in the infiltration.

Immunohistochemical examination: S-100 protein and CDIa (OKT6) were positive, and ultra-microscopic examination of 10% to 25% of cells showed Birbeck particles, large tissue cells with dense cells, regular lamellar bodies and Unique phagocytic lysosomes.

Diagnosis and identification of congenital self-healing Langerhans cell histiocytosis

The disease occurs in infants and newborns. The damage caused by multiple disseminated red-brown nodules can be increased and the number of these lesions can increase in the weeks after birth. After a few cases of pale white atrophic scars, a few cases of isolated lesions develop rapidly congenital spontaneous ulcerative tumors occur in the face of the trunk or limbs. The disease is characterized by no mucosal damage, except for occasional liver disease, usually no systemic symptoms or Abnormal hematopoietic system abnormal skin lesions can quickly resolve the body and mental development.

The material in this site is intended to be of general informational use and is not intended to constitute medical advice, probable diagnosis, or recommended treatments.