Unusual "skin disease"

foreword

PrefacePreface

In life, when it comes to skin diseases, I believe many people are no strangers
.

Most of them are caused by allergies.

In life, when it comes to skin diseases, I believe many people are no strangers

case by case by case

The patient, male, 73 years old, was admitted to the hospital with the main complaint of " repeated red patches on the trunk accompanied by itching for half a year " .
Half a year ago, there was no obvious reason for the appearance of large red nail patches the size of soybeans on the trunk, which caused itching and discomfort.
The above rashes gradually increased.
He went to the local clinic for treatment, the diagnosis was unknown, and topical drugs were given (specifically unknown), but the rash did not subside .
Today, in order to seek further diagnosis and treatment, I came to our hospital for treatment.
The outpatient diagnosis is " acute febrile neutrophilic dermatosis? Mycosis fungoides? " .

The patient, male, 73 years old, was admitted to the hospital with the main complaint of " repeated red patches on the trunk accompanied by itching for half a year " .

Half a year ago, there was no obvious reason for the appearance of large red nail patches the size of soybeans on the trunk, which caused itching and discomfort.
The above rashes gradually increased.
He went to the local clinic for treatment, the diagnosis was unknown, and topical drugs were given (specifically unknown), but the rash did not subside .

Blood routine ( specimen number: 86 in our hospital on April 20, 2020 ) showed : white blood cells 7.
75×10^9/L , red blood cells 5.
35×10^12/L , neutrophil ratio 63% , lymphocyte ratio 29.
4% , platelets 205×10 ^9/L .

According to the medical history and clinical signs, cutaneous lymphoma cannot be ruled out at present, and the skin pathology is further confirmed

Lymph node color Doppler ultrasound suggested: multiple lymph nodes (hilar structure is unclear), consider: lymphoma

Figure 1  Bone Marrow Report

Figure 1  Bone Marrow Report

Figure 2   Streaming report

Figure 2   Streaming report

Figure 3  Blood GPS report 

Figure 3  Blood GPS report 

Submitted for blood pathology, bone marrow report: abnormal lymphocytes accounted for 46% ; flow cytometry: showed that abnormal cells accounted for about 56.

4% of nuclear cells, and their immunophenotypes were CD33+ very few , HLADR+, CD5+ few , CD4+, CD56+, CD2+, a small amount of CD7+ , a small amount of CD303+ , a small amount of CD304+ , CD123++, the current immunophenotype information is considered to be blastic plasmatic dendritic cell tumor ;

Submitted for blood pathology, bone marrow report: abnormal lymphocytes accounted for 46% ; flow cytometry: showed that abnormal cells accounted for about 56.
4% of nuclear cells, and their immunophenotypes were CD33+ very few , HLADR+, CD5+ few , CD4+, CD56+, CD2+, a small amount of CD7+ , a small amount of CD303+ , a small amount of CD304+ , CD123++, the current immunophenotype information is considered to be blastic plasmatic dendritic cell tumor ;

Immunohistochemistry: CD56 extensive ( + ) CD43 scattered ( + ), CD45RA occasionally ( + ), CD123 extensive ( + ), Ki-67 (about 80% ), tumor cells accounted for about 80% , consistent with blast cytoplasm Cytoid dendritic cell tumors involve the bone marrow
.

 

Immunohistochemistry: CD56 extensive ( + ) CD43 scattered ( + ), CD45RA occasionally ( + ), CD123 extensive ( + ), Ki-67 (about 80% ), tumor cells accounted for about 80% , consistent with blast cytoplasm Cytoid dendritic cell tumors involve the bone marrow

The disease has a high degree of malignancy, difficult treatment, poor prognosis, and high cost.

The current treatment is to relieve symptoms and improve the quality of life

case analysis

The blood routine changes of the patients since hospitalization are summarized in Table 1 :

The blood routine changes of the patients since hospitalization are summarized in Table 1 :

Table 1   Changes of blood routine in patients on different days

Table 1   Changes of blood routine in patients on different days

Figure 4 Changes in blood routine at different time points

Figure 4 Changes in blood routine at different time points

Figure 5 Changes in red blood cells at different time points

Figure 5 Changes in red blood cells at different time points

We can see from Figures 4 and 5 that platelets, erythrocytes and hemoglobin decreased significantly when the patient did not receive chemotherapy and symptomatic treatment; the increase in the second hospitalization was due to the transfusion of erythrocytes and platelets, and there was a brief recovery; White blood cells, CRP , and monocytes increased linearly in the later stage
.

 

We can see from Figures 4 and 5 that platelets, erythrocytes and hemoglobin decreased significantly when the patient did not receive chemotherapy and symptomatic treatment; the increase in the second hospitalization was due to the transfusion of erythrocytes and platelets, and there was a brief recovery; White blood cells, CRP , and monocytes increased linearly in the later stage

The disease develops rapidly, the disease is highly malignant, until the failure of multiple organs, sometimes seemingly ordinary skin disease, the final result also has an unusual side

The importance of a physical examination can sometimes give us clues to diagnose a disease
.
Neither pathology nor bone marrow can make a correct diagnosis.
Diagnosis requires a combination of comprehensive analysis and multidisciplinary analysis.
Flow cytometry results and immunophenotypes give a definitive diagnosis
.
BPDCN was clearly classified as an AML -related precursor tumor in the 2008 edition of the World Health Organization Classification of Hematopoietic and Lymphoid Tissue Tumors, and by 2016 , the disease was classified as a separate category hematological tumors [1] .

Blast plasmacytoid dendritic cell tumor is a rare hematological tumor in which tumor cells resemble blast lymphocytes or myeloblasts [2] , which requires us to be careful when looking at cells
.
Comprehensive analysis of the hematological pathology GPS report for the diagnosis of the disease is the key
.
It can occur at any age, and is prone to occur in the elderly.
Most of them are skin diseases.
The clinical course is highly invasive and the prognosis is extremely poor.
The median survival time is about 1 year [3]
.
If repeated treatment of skin diseases with similar symptoms fails, you need to be vigilant
.

Blast plasmacytoid dendritic cell tumor is a rare hematological tumor in which tumor cells resemble blast lymphocytes or myeloblasts [2] , which requires us to be careful when looking at cells
.
Comprehensive analysis of the hematological pathology GPS report for the diagnosis of the disease is the key
.
It can occur at any age, and is prone to occur in the elderly.
Most of them are skin diseases.
The clinical course is highly invasive and the prognosis is extremely poor.
The median survival time is about 1 year [3]
.
If repeated treatment of skin diseases with similar symptoms fails, you need to be vigilant
.

experience

experience

Hematological neoplasms are proliferative manifestations of malignant cells of the larger hematological system, including various leukemias, multiple myeloma, and malignant lymphomas
.
There are also many rare hematological tumors, which are easily deceived by the skin appearance in our work, but can be identified based on the unique morphology, flow cytometry and immunophenotype of the disease
.
There is no end to learning, we still need to continue to learn knowledge and sum up experience to help more patients
.

Hematological neoplasms are proliferative manifestations of malignant cells of the larger hematological system, including various leukemias, multiple myeloma, and malignant lymphomas
.
There are also many rare hematological tumors, which are easily deceived by the skin appearance in our work, but can be identified based on the unique morphology, flow cytometry and immunophenotype of the disease
.
There is no end to learning, we still need to continue to learn knowledge and sum up experience to help more patients
.

references

references

[1] Chen Xiaojun , Liu Yanquan , Huang Surong , Zhao Liwei , Shen Jianzhen .
Research progress of blastic plasmacytoid dendritic cell tumor [J].
PLA Medical Journal , 2021, 46(10): 1040-1044.

[1] Chen Xiaojun , Liu Yanquan , Huang Surong , Zhao Liwei , Shen Jianzhen .
Research progress of blastic plasmacytoid dendritic cell tumor [J].
PLA Medical Journal , 2021, 46(10): 1040-1044.

[2] Zhang Yingnan , Zhang Maogong .
Advances in research and diagnosis and treatment of blastic plasmacytoid dendritic cell tumors [J].
Journal of Clinical Dermatology , 2020, 49(7): 442-445.

[2] Zhang Yingnan , Zhang Maogong .
Advances in research and diagnosis and treatment of blastic plasmacytoid dendritic cell tumors [J].
Journal of Clinical Dermatology , 2020, 49(7): 442-445.

[3] Zhang Jing , Li Yanqiu , Huang Changzheng .
Clinical and pathological analysis of three cases of blastic plasmacytoid dendritic cell tumor [J].
China Journal of Leprosy and Dermatology , 2019, 35(11): 659-663.

[3] Zhang Jing , Li Yanqiu , Huang Changzheng .
Clinical and pathological analysis of three cases of blastic plasmacytoid dendritic cell tumor [J].
China Journal of Leprosy and Dermatology , 2019, 35(11): 659-663.

 

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