1.Autoimmune polyglandular syndrome type Ⅳ: thymoma, vitiligo, myasthenia gravis, systemic lupus erythematosus, pemphigus vulgaris and alopecia universalis successively occurred in a patient
Yuetong QIAN ; Jiawei LIU ; Wei LIU ; Donglai MA
Chinese Journal of Dermatology 2018;51(10):733-736
On November 30th,2017,a 62-year-old female patient was admitted to the hospital because of multiple depigmented patches all over the body for 16 years,as well as erythema and blisters on the scalp,trunk and upper extremities for more than 8 years,which had been aggravated for 1 year.The patient underwent surgical resection of thymoma in 1996,and developed myasthenia gravis in 2004.Skin examination showed scattered erythemas of various sizes on the scalp,face,chest and upper extremities,with crusts on the surface of some erythemas and positive Nikolsky's sign.Hypopigmented macules mingled with depigmentated macules of various sizes were scattered all over the body.A large area of depigmentation was observed on both hands,and the nail plates were atrophic.Hairs,eyebrows,eyelashes,axillary and pubic hairs were abscent.Laboratory examination showed the presence of homogeneous IgG antinuclear antibody with a titer of 1:640,anti-double stranded DNA antibody (276 IU/ml),lupus anticoagulant (1.62),anti-Dsg1 antibody (> 150 U/ml) and anti-Dsg3 antibody (91 U/ml).Histopathological examination of dorsal blister tissues and hematoxylin-eosin staining showed fissured blisters and acantholytic cells in the epidermis,monolayer basal cells on the basilar membrane,and perivascular infiltration of a few lymphocytes in the superficial dermis.The patient successively developed thymoma,vitiligo,myasthenia gravis,systemic lupus erythematosus,pemphigus vulgaris and alopecia universalis,so a diagnosis of autoimmune polyglandular syndrome type Ⅳ was made.Since autoimmune-mediated endocrinopathies are prone to occur successively,changes of related hormones and antibodies in the patient should be monitored for early diagnosis and treatment of newly emerging autoimmune diseases.
2.Autoinflammatory diseases accompanied by vasculitis
Wei LIU ; Jiawei LIU ; Yuetong QIAN ; Donglai MA
Chinese Journal of Dermatology 2019;52(5):340-342
Autoinflammatory diseases are a group of inherited disorders characterized by dysregulation of the immune system,periodic fever and reccurrence of inflammation.The common rashes of autoinflammatory diseases include acne,pyoderma gangrenosum,erysipelas,urticaria,ichthyosis,pustulosis,while vasculitis is rare.This review summarizes autoinflammatory diseases accompanied by vasculitis.Familial Mediterranean fever mainly manifests as small-and medium-vessel vasculitis such as Henoch-Sch(o)nlein purpura and polyarteritis nodosa,while large-vessel vasculitis is common in Blau syndrome.Small-and medium-artery vasculitis can occur in patients with deficiency of adenosine deaminase 2 as well,so it should be differentiated from polyarteritis nodosa in clinical practice.Stimulator of interferon gene-associated vasculopathy with onset in infancy mainly presents as cutaneous small-vessel vasculitis and vasculopathy.
3.Radiotherapy-related skin diseases
Yuetong QIAN ; Jiawei LIU ; Wei LIU ; Donglai MA
Chinese Journal of Dermatology 2019;52(2):145-148
As an effective treatment method of tumors and other diseases,radiotherapy has been widely applied to clinical practices.After radiotherapy,about 95% of patients suffer from skin injuries to different extents,including acute and/or chronic radiodermatitis,or radiotherapy-related skin diseases,including cutaneous fibrosis,morphea,sclerosing panniculitis,bullous skin diseases,eosinophilic polymorphic and pruritic eruption,lichen sclerosus et atrophicus,angiosarcoma,atypical vascular lesions and radiation recall dermatitis.These skin injuries greatly affect the quality of life and treatment compliance of patients.This review briefly summarizes common and rare skin diseases induced by radiotherapy,so as to improve the understanding of radiotherapy-related skin diseases in clinicians.
4.A case of pemphigoid nodularis
Yuetong QIAN ; Hongzhong JIN ; Donglai MA ; Tao ZHANG
Chinese Journal of Dermatology 2020;53(3):230-231
5.Role of inflammasomes in skin tumors
Wei LIU ; Jiawei LIU ; Yuetong QIAN ; Donglai MA
Chinese Journal of Dermatology 2020;53(10):838-840
Inflammasomes are a kind of intracellular multiprotein complex that comprises a part of the innate immune response. Inflammasome function abnormalities can lead to immune imbalance and have been linked to an increasing number of diseases. Researches have shown that inflammasomes are associated with some skin tumors including basal cell carcinoma, squamous cell carcinoma, Kaposi′s sarcoma and malignant melanoma, implicating that persistent inflammatory response and immune imbalance caused by abnormal activation of inflammasomes play a crucial role in tumor occurrence, development, invasion and metastasis. This review summarizes research advances in the role of inflammasomes in the pathogenesis of skin tumors.
6.Gardner-Diamond syndrome
Yan TAN ; Yuetong QIAN ; Donglai MA
Chinese Journal of Dermatology 2023;56(8):787-788
Gardner-Diamond syndrome is a rare skin disease characterized by recrudescent, spontaneous and tender ecchymoses, and mostly affects psychologically disturbed adult women. The positive autoerythrocyte sensitization test has noticeable diagnostic significance. Currently, there is no specific therapy for Gardner-Diamond syndrome. This review summarizes advances in the pathogenesis, clinical features, diagnosis and treatment of this disease.