Paget's disease-like reticulocytosis
Introduction
Introduction to Paget's disease-like reticulosis Paget's disease-like reticulocyte hyperplasia, also known as localized epidermotropic reticulosis or Woringer-Kolopp disease. In 1931, Ketren and Goodman described a clinically similar MF, apparently of multiple epithelial origin of multiple skin lesions. In 1939, Woringer and Kolopp reported a case of a 6-year-old boy with a single plaque-like lesion in the arm. One of Paget's disease-like reticular cell hyperplasia was proposed by Braum-Falco et al. based on clinical and histological appearance. basic knowledge The proportion of sickness: 0.00012% Susceptible people: more common in men Mode of infection: non-infectious Complications: lymphoma
Cause
The etiology of Paget's disease-like reticulosis
Causes:
The cause is not clear.
Pathogenesis:
The pathogenesis is still unclear, the disease is rare, 20 cases reported abroad, more common in men, domestic Liu Jihe and other reports, 1 case, elderly women, rash involving the vulva and ankle, and later see a case of middle-aged male limited to the lower limbs patient.
Prevention
Paget's disease-like reticulosis prevention
The cause of the disease is still unclear, and there are no special effective preventive measures. Diet should pay attention to taking more light and nutritious food, eat more food to improve immunity, in order to improve the body's disease resistance.
Complication
Paget's disease-like reticulosis complications Complications lymphoma
The disease is characterized by a long duration of damage and a rare progression to systemic lymphoma.
The damage develops rapidly, often invading the lymph nodes, and its biological behavior is invasive.
Symptom
Paget's disease-like reticulosis symptoms common symptoms nodules
Can be divided into 2 types, the two are the same in histology, but the clinical process is completely different.
1.Woringer-Kolopp type
For the limited subtype, it is a pale red to pale brown single plaque, mainly located in the limbs. If more than one damage, the damage has a tendency to invade the palmar, and in a few months, the damage gradually increases. The size can be more than 10cm. In some cases, the damage is good for many years, and 20% of the patients are less than 15 years old. The disease is characterized by long duration of damage and rare progression to systemic lymphoma.
2.Ketron-Goodman type
It is a generalized subtype, which is characterized by most reddish plaques similar to MF. The damage develops rapidly and often invades the lymph nodes, and its biological behavior is invasive.
Examine
Inspection of Paget's disease-like reticulosis
Histopathology: Both types of large atypical T cells were highly epithelial invasive, single or nested, similar to Paget's disease, and most of the atypical fibrosis was seen in the intraepithelial lymphocytes.
Immunohistochemistry: typical damage is similar to typical MF, which is expressed as CD4+, CD3+, CD5+ and CD8-.
Diagnosis
Diagnosis and identification of Paget's disease-like reticulocyte hyperplasia
Localized subtypes should be differentiated from hypertrophic lichen planus or Bowen's tumor, plaques of disseminated Paget-like reticulocyte hyperplasia, similar to plaques and nodules of other lymphomas or leukemias, due to clinical lack of eczema damage Can exclude MF.
