The disease relapses as soon as the hormone is reduced. This rheumatism patient is too difficult!

*Only for medical professionals' reference Preliminary summary: The patient went to the doctor with multiple skin rashes and itching all over the body.
At first, he considered "allergic dermatitis", and the rash was relieved after hormone treatment
.

After the hormone reduction, the patient began to experience bilateral eyelid edema, fatigue, and pharyngeal discomfort again.
After a comprehensive examination, the truth was discovered
.

Case introduction The patient in this case is a 53-year-old male who was admitted to the hospital with the main complaint of "multiple skin rashes with itching all over the body for 1 year and exacerbation for 1 month"
.

▌ First of all, let’s review the medical history: about 1 year ago, the patient first saw scalp erythema with itching.
At first, he didn’t care about it.
Afterwards, the rash gradually expanded and spread to the face, limbs and trunk with obvious symptoms.
Pruritus, the patient went to the dermatology clinic.
At the first visit, he suspected "allergic dermatitis?" and was treated with "prednisone 60mg/day, levocetirizine anti-allergic and calcium supplementation".
The rash gradually relieved and the hormones gradually decreased
.

After the patient's first visit, the condition was confusing, and the doctor recommended a comprehensive examination to confirm the diagnosis
.

Seven months ago, the patient began to develop bilateral eyelid edema without any obvious cause, and the rash had not completely subsided, accompanied by general weakness and pharyngeal discomfort.
He denied fever, joint swelling and pain, abdominal pain, diarrhea, abnormal urination and other symptoms.
Ming was diagnosed and admitted to the hospital
.

During the hospitalization, the patient underwent a skin biopsy of the right upper arm
.

Skin biopsy (see Figure 1) showed: mild hyperplasia of squamous epithelium, mild epidermal keratosis and dyskeratosis, formation of keratinous cysts, superficial dermal edema, infiltration of numerous lymphocytes and plasma cells, perivascular inflammation, HE section It is more consistent with the histological changes of skin inflammatory lesions
.

Figure 1 Skin biopsy muscle enzyme spectrum under light microscope shows: creatine kinase 323U/L, creatine kinase isoenzyme 36.
6 U/L, lactate dehydrogenase 303U/L
.

Other indicators such as blood routine, erythrocyte sedimentation rate, C-reactive protein, ferritin, etc.
are generally normal
.

Chest CT showed no obvious abnormalities (see Figure 2)
.

Tumor markers and other imaging examinations found no evidence of tumor
.

Electromyography suggests myogenic damage
.

Figure 2 Chest CT patients were negative for anti-nuclear antibodies, and anti-SSA, SSB, Ro-52, and Smith antibodies were all negative
.

Further improve the anti-myositis antibody spectrum, suggesting that the anti-transcription intermediary factor (TIF) 1-γ antibody is positive, and the anti-OJ, KS, Scl-70, Ku, PL-7 and other antibodies are all negative
.

The doctor and the patient worked together to investigate the truth, and the case finally came to light and the dust settled
.

Combined with the physical examination at the time, the patient had extensive skin rash, symmetrical proximal limb weakness, elevated serum muscle enzymes, electromyogram indicating muscle damage, positive anti-TIF1-γ antibody, etc.
, diagnosis should consider dermatomyositis, and treatment He was treated with methylprednisolone 60mg/day, hydroxychloroquine, methotrexate, etc.
, and was discharged from the hospital after his condition improved.
The hormone outside the hospital was gradually reduced to 20mg/day, and his condition was relatively stable for the first few months
.

However, unfortunately, one month ago, during the hormone reduction process, the rash and fatigue were significantly worse than before
.

The patient re-examined positive anti-nuclear antibodies, and perfect PET-CT showed: 1.
Diffuse increase in FDG metabolism of skin and muscle in multiple parts of the body
.

Combined with clinical, it is in line with the image manifestations of dermatomyositis; multiple subcutaneous edema
.

2.
Double lung fibrous foci; bilateral inferior posterior pleura benign hypertrophy
.

3.
Left kidney cyst
.

4.
Hemorrhoids
.

5.
F18-FDG PET-CT systemic examination (cranial to upper thigh) showed no other obvious abnormalities
.

After the patient received methylprednisolone 40mg/day×8d, gamma globulin 20g×5d and oral mycophenolate, the rash symptoms improved, the hormone was changed to oral administration and then discharged from the hospital, the hormone was reduced by himself outside the hospital, and 1 tablet was taken a week.
Gradually reduce to 20mg/day
.

One week ago, the patient's rash worsened again, and the rash gradually affected the skin of the head, face, neck, chest area, back and limbs, accompanied by double eyelid edema, dysphagia, and general fatigue
.

This time he was admitted to the hospital for continued treatment
.

Family history: Father died of "lymphoma"
.

Personal history is nothing special
.

Physical examination: body temperature 37.
2℃, pulse rate 94 beats/min, breathing 18 beats/min, blood pressure 127/93mmHg
.

Diffuse purple-red skin rashes were seen on the scalp, face, neck, front chest and limbs, partly fused into slices, irregular in shape, faded under pressure, Gottron sign, and purple-red spots symmetrically fused on the extension sides of the metacarpophalangeal joints of both hands (see picture) 3)
.

Examination of the heart, lungs and abdomen showed no obvious abnormalities
.

Difficulty in raising arms and squatting, and difficulty in raising head when lying down
.

Figure 3 Photo of the patient’s hands ▌ Laboratory testing after admission (see Table 1): Re-check the antinuclear antibody after admission (titer 1:100-1:320); anti-SSA, SSB, Ro-52, Smith antibodies, etc.
Is negative
.

Troponin T and lactate dehydrogenase increased slightly, ferritin levels increased, erythrocyte sedimentation rate, C-reactive protein and other indicators were roughly normal
.

Table 1 The main laboratory test indicators are as follows: ▌ ‍Admission diagnosis and treatment: The patient has a clear diagnosis of dermatomyositis.
He was given methylprednisolone 60mg/day and oral mycophenolate.
The rash, swallowing and choking, and the muscle strength of the limbs gradually alleviated.
, The condition is relatively stable, and the blood routine, biochemical, muscle enzyme spectrum and other indicators are regularly reviewed, and the treatment is still under follow-up
.

Case study ▌ Summarize the characteristics of this case: middle-aged men have extensive skin rashes with itching, muscle damage, anti-TIF1-γ antibody positive, no evidence of progressive lung interstitial lesions and tumors.
The above symptoms are effective with glucocorticoid therapy, but Symptoms get worse repeatedly
.

▌ In view of this case, combined with the current literature, we will analyze this case together~1.
From the appearance of rash, the early skin lesions of atypical dermatomyositis are highly characteristic symmetrical fusion of purple-red spots, which often occur in The face can also involve the back of the hands and fingers, the extended side of the arms, the deltoid area, the back of the shoulders and neck, the front of the neck and the upper chest V-zone, and the scalp
.

The trunk rash is diffuse or localized
.

Usually there are no symptoms such as itching, pain, and paresthesias
.

The rash of this patient initially manifested as scalp erythema, accompanied by obvious itching, and then gradually involved multiple skins on the face, limbs, and trunk
.

2.
The clinical significance of anti-TIF1-γ antibody Idiopathic inflammatory myopathy (IIM) is a group of acquired heterogeneous diseases with skeletal muscle involvement as the main manifestation, mainly including polymyositis, dermatomyositis and inclusion There are 3 types of body myositis (IBM)
.

Some autoantibodies are closely related to the clinical phenotype and prognosis of dermatomyositis
.

As early as 2006, Targoff et al.
reported that there was an autoantibody against a protein with a relative molecular mass of 155,000 in patients with IIM.
This antibody has not been found in patients with other autoimmune diseases and non-autoimmune diseases.
It seems to be specific to IIM.
Sex autoantibodies, and found that the antibody is associated with adult IIM with tumors
.

Later studies found that the target antigen recognized by the antibody was TIF1-γ protein
.

Recent studies have found that anti-TIF1-γ antibodies may be pathogenic antibodies, especially related to the pathogenesis of myositis and malignant tumors
.

Clinical studies have shown that patients with positive anti-TIF1-γ antibodies are more likely to have tumors, which can be used as a good predictor of myositis with tumors
.

Hida et al.
retrospectively analyzed 349 patients with idiopathic inflammatory myopathy and found that 75 patients had malignant tumors, of which 36 were positive for anti-TIF1-γ antibody, and 35 were found to have malignant tumors within 1 year of myositis diagnosis.
In 1 case, a malignant tumor was found one year before the diagnosis of myositis.
These patients all met the diagnostic criteria for dermatomyositis
.

Fiorentino et al
.
tested 134 cases of adult dermatomyositis with anti-TIF1-γ antibody .

The results showed that: 55 cases of anti-TIF1-γ antibody were positive, 46 of which only had anti-TIF1-γ antibody detected; the incidence of lung interstitial disease, Raynaud's phenomenon and arthritis/arthralgia in patients with anti-TIF1-γ antibody positive was significantly lower than negative Patients; itching is more common in patients with anti-TIF1-γ antibody positive; serum aldolase and creatine kinase levels in patients with anti-TIF1-γ antibody positive are significantly lower than those with anti-TIF1-γ antibody negative; patients with anti-TIF1-γ antibody positive Obvious skin damage, psoriasis-like damage, hypopigmented spots on the basis of mixed punctate telangiectasia or erythema and palm hyperkeratosis papules are significantly higher than that of negative patients, while the incidence of calcinosis is significantly lower than that of negative Patient
.

The conclusion of the case The patient’s initial presentation is not typical, and early diagnosis is difficult.
The anti-TIF1-γ antibody-positive patient has its special clinical phenotype and prognosis
.

Dermatomyositis associated with malignant tumors often occur in people over 50 years of age, with Heliotrope sign, skin necrosis, and myositis-specific antibody anti-TIF1-γ antibody, anti-nuclear matrix protein 2 antibody positive and other clinical characteristics, and need to be screened for malignant tumors Check or closely monitor follow-up
.

Reference materials: [1] Hida A, Yamashita T, Hosono Y, et al.
Anti-TIF1-γantibody and cancer-associated myositis: a clinicohistopathologic study［J］.
Neurology,2016,87(3):299-308.
[ 2]Fiorentino DF,Kuo K,Chung L,et al.
Distinctive cutaneous and systemic features associated with antitranscriptional intermediary factor-1γantibodies in adults with dermatomyositis［J］.
J Am Acad Dermatol,2015,72(3):449-455.
[3] Lv Ling, Lv Xiaoyan.
Research progress of dermatomyositis with malignant tumors [J].
Chinese Journal of Dermatology, 2021, 54(00): E038-E038.
[4] Huang Wan, Wei Hua.
Tumor-related Research progress in the pathogenesis of myositis[J].
Chinese Journal of Rheumatology,25(6):4.
Source of this article: Medical Rheumatology Channel Author of this article: Jindi Wang Yuwei Review of this article: Chen Xinpeng, deputy chief physician editor in charge: cassette copyright statement The original text of this article welcomes forwarding to the circle of friends-End-The medical community strives to make its published content accurate and reliable when approved, but does not make any comments on the timeliness of the published content, and the accuracy and completeness of the cited information (if any).
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