1060630小兒科病例討論會：erythema nodosum

台灣兒科醫學會 高雄地區兒科聯合病例討論會 病例報告 June 30th, 2017 聖功醫院兒科部羅文聰醫師

General data • Name: 黃O婕 • Gender/Age: Female/17-year-old • History No.: 19xxxx5

Chief complaint • Skin rashes over bilateral legs for 4 days. • Fever and pain on bilateral legs for 2 days.

Present illness 1 week ago

Travel to 慈湖及大溪農場.

Day 1

Small skin rashes on pretibia region were noted.

Day 3

Fever was noted, and skin rashes increased.

Day 4

Skin rashes increased combined with swelling and persisting fever. Due to the symptoms, she was sent to hospital A where the impression was cellulitis and amoxicillin/clavulanate was used.

Day 5

Due to persisting skin rashes and fever, she was admitted.

Past history • Denied history of major operation or trauma.

• Denied history of systemic diseases.

• ADHD with mild mental retardation.

Personal history • Vaccination was given after delivery as schedule until now.

• Preterm with NSD. • Allergy history: no known drug and food allergy.

• Occupation: student.

• Traveling history: 慈湖及大溪農場 (1 week ago).

• Denied of any contact history.

• Marriage status: not yet.

Family history HTN

Oral ulcer 不易癒合

Physical examination • Vital signs: BT: 38.5 ℃ PR: 139 /min RR: 20 /min BP:107/65 mmHg • Skin: target-like maculopapular rashes with local heat and tenderness over four limbs → tender firm erythematous deepseated nodules over four limbs.

• HEENT: no stiffness, no cervical lymphadenopathy, no oral ulcer, mild congestion of throat, no congestion of eardrum, bilateral.

• Chest: clear breathing sound, bilateral.

• Extremities: swelling with ROM limitation of bilateral knee and ankle joints.

Day 5

Lab. data

Impression • Erythema nodosum.

• Erythema multiforme, suspicious of Mycoplasma pneumoniae infection.

• Suspect scrub typhus.

Plan to do • • • •

Check liver function and U/R. Check M. pneumoniae IgM Ab. Consult infectious specialist for evaluation.

Consult rheumatologist to rule out the possibility of autoimmune disease.

• Consult dermatologist for differential diagnosis of skin lesions.

Hospital course

Lab. data 檢驗代碼

檢驗結果

檢驗結果單位

最小/最大安全值

AST

18

U/L

─ 32

ALT

9

U/L

─ 31

Medical Therapy • Doxycycline (Day 1): for suspicious of scrub typhus

• Azithromycin (Day 4): for suspicious of M. pneumoniae infection

• Methylprednisolone (Day 5): for anti-inflammation

Vital Signs Doxycycline

Azithromycin

Methylprednisolone

Lab. data • M. pneumoniae IgM Ab: equivocal.

• Blood culture: no growth for 7 days.

R/O thrombosis • *** 血液腫瘤科檢查 *** • 日期 Pro.C Pro.S • 2011/06/01 81.0 90.2 • *** 生化免疫檢查 *** • 日期 RF C3 C4 IGG IGM • U/ml mg/dl mg/dl IU/ml IU/ml • 2011/06/01 <11.3 128 21.2 865 210 • D-D diamer:↑935 • Fibrinogen: ↑ 814.7

PT/PTT:11.4/33.6 Albumin: 3.4

檢驗代碼

檢驗結果

單位

<11.3

IU/mL

Anti-MPO

0.2

IU/ml

neg.<3.5

Anti-PR3

0.4

IU/ml

neg.<2

RF-RIA

安全值 0.0 ─ 15.0

ANCA 定量

Sonography of knee joints â&#x20AC;˘ Report: 1. Arthritis of bilateral knees and right tibiotalar joints with minimal effusion.

2. Panniculitis of bilateral lower legs.

3. Please correlate with clinical condition.

Skin biopsy • The specimen submitted consisted of 1 small piece of skin tissue measuring 0.8 x 0.2 x 0.2 cm in size, fixed in formalin.

• Grossly, it was gray in color and soft in consistence.

Totally embedded for sections.

• Microscopically, the sections show a picture of focal periadnexal inflammatory infiltrate and mild panniculitis.

No evidence of malignancy.

• • • • •

The specific IgM response to Mycoplasma pneumoniae infection: interpretation and application to early diagnosis.

Moule JH, Caul EO, Wreghitt TG. Clinical Microbiology and Public Health Laboratory, Addenbrooke's Hospital, Cambridge.

Abstract Specific IgM antibody production in patients with serologically proven Mycoplasma pneumoniae infection by the complement fixation (CF) test was studied using a mu-capture ELISA. Sera from 79% of patients were found to be IgM positive. Patients could be divided into two groups relating to the amount of specific antibody produced. High levels of specific IgM (greater than or equal to 10 units) were more commonly found in younger patients. Seventy-six per cent of patients under the age of 20 produced relatively high levels of IgM compared to 35% of patients over the age of 20.

In contrast, the number of patients who produced low or undetectable levels of IgM (less than 10 units) was found to increase with age. This trend was found to be significant which suggests that low or undetectable levels of IgM may be due to reinfection with M. pneumoniae. Specific IgM was found to appear in the serum at approximately 7 days after the onset of symptoms, peaking at between 10 and 30 days, and then falling to undetectable levels at an estimated 12-26 weeks post onset of symptoms. Twenty-eight per cent of acute-phase sera (CF titres less than 256) from patients whose sera subsequently showed a fourfold or greater rise in M. pneumoniae CF antibody titre were IgM positive. Thus using mu-capture ELISA a diagnosis of M. pneumoniae infection may often be made more rapidly than by the complement fixation test.

OPD follow-up M. pneumoniae IgM antibody: positive.

Erythema Nodosum (EN)

Introduction • Red or violet subcutaneous nodules, usually develop in pretibial location.

• Delayed hypersensitivity reaction.

• The pathogenesis is largely unclear.

Epidemiology • Most common type of panniculitis.

• More common among females (F:M=6:1).

• The annual incidence is approximately 1 to 5/100,000 persons, most often in women ages 15 to 40 years. Clin Exp Rheumatol 2001; 19:365

Etiology

Fitzpatrickâ&#x20AC;&#x2122;s color atlas & synopsis of clinical dermatology 6th, 2009

Cause • The majority of patients with EN have evidence of recent streptococcal infection.

• In Europe and North America, sarcoidosis constitutes a dominant cause of EN. • Tuberculosis remains an important underlying condition in other parts of the world.

UpToDate, May 2011: erythema nodosum

Pathogenesis • A panniculitis involving inflammation of septa in subcutaneous fat tissue, usually without associated vasculitis.

• Many immune-mediated mechanisms were implicated: – Type IV hypersensitivity.

– Immunocomplex deposition?

Clinical Manifestations â&#x20AC;˘ Look like erythema but feel like nodules. â&#x20AC;˘ Tender nodules (3-20cm), not sharply marginated.

Clinical Manifestations • Painful, erythematous nodules →bruiselike lesions that resolve without scarring over a 2 to 8-week period. Arch Dermatol. 1977;113(7):909 • Mostly on the anterior lower legs.

• Lesions can also appear on the thighs, trunk, and upper extremities, but absence of nodules on the legs is atypical.

Diagnosis • Hematology: – Elevated ESR (initial), CRP, leukocytosis.

– Anti-streptolysin titer.

• Bacteria culture: – Throat culture for group A β-hemolytic streptococcus.

– Stool culture for Yersinia, Salmonella, and Campylobacter organisms.

Diagnosis • Image: – CXR and gallium scan to rule out sarcoidosis.

• Dermatopathology: – The diagnosis of EN often is clinical.

– Deep skin incisional biopsies are required to sample the subcutaneous tissue adequately.

J Invest Dermatol. 1975;65(5):441

Diagnosis • Dermatopathology: – Biopsy is required only in atypical cases.

– Indications of biopsy: • patients with no lesions on the legs.

• persistence beyond six to eight weeks.

• the development of ulceration.

Dermatopathology â&#x20AC;˘ Acute (polymorphonuclear) and chronic (granulomatous) inflammation in the panniculus and around blood vessels in the septum and adjacent fat. â&#x20AC;˘ Septal panniculitis: thickened septa of the subcutaneous tissue with inflammatory cell infiltration.

Treatment • EN is usually self-limited or resolves with treatment of underlying disorder.

• The treatment is symptomatic.

• Aspirin or NSAID – for anti-inflammatory, analgesic, and antipyretic properties.

Treatment • Antibiotics – not warranted in patients with asymptomatic pharyngitis though related to streptococcal infection.

• Systemic corticosteroid – seldom necessary.

– advantage: hasten symptomatic relief.

– disadvantage: the possibility of masking an underlying neoplastic, inflammatory, or infectious condition.

Prognosis â&#x20AC;˘ Regress spontaneously in 2-8 weeks, with new lesions erupting during that time. â&#x20AC;˘ Lesions never break down or ulcerate and heal without scarring.

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