Multicentric Reticulohistiocytosis (MRH / मल्टीसेंट्रिक रेटिकुलोहिस्टियोसाइटोसिस) एक rare systemic inflammatory disorder है।
- यह condition मुख्य रूप से skin (त्वचा) और joints (जोड़ों) को प्रभावित करती है।
- MRH में papulonodular skin lesions और destructive polyarthritis देखा जाता है।
- Disease usually adults (40–60 years) में common है और महिला में पुरुष की तुलना में अधिक होती है।
- Early diagnosis महत्वपूर्ण है क्योंकि joint destruction irreversible हो सकता है।
Multicentric Reticulohistiocytosis क्या है (What is MRH)
- Systemic histiocytic disorder
- Characterized by:
- Papulonodular skin lesions (face, hands, arms)
- Symmetrical polyarthritis (fingers, wrists, elbows, knees)
- Etiology: idiopathic, sometimes associated with malignancies (paraneoplastic syndrome)
Multicentric Reticulohistiocytosis कारण (Causes of Multicentric Reticulohistiocytosis)
1. Idiopathic / Unknown
- Exact cause unknown, immune system dysregulation suspected
2. Immune-mediated Mechanism
- Overactivation of macrophages and histiocytes
- Cytokine-mediated joint and skin involvement
3. Paraneoplastic Association
- Rare cases associated with breast, lung, gastrointestinal cancers
- MRH may precede diagnosis of underlying malignancy
4. Genetic Factors
- No clear inheritance pattern, sporadic cases reported
Multicentric Reticulohistiocytosis लक्षण (Symptoms of Multicentric Reticulohistiocytosis)
Skin Symptoms
- Multiple papules and nodules, reddish-brown or flesh-colored
- Usually over dorsal hands, face, arms, ears
- Lesions may coalesce into plaques
Joint Symptoms
- Symmetrical polyarthritis
- Swelling, pain, stiffness in small and large joints
- Progressive joint destruction (arthritis mutilans in severe cases)
Systemic Symptoms
- Fatigue
- Weight loss in some cases
- Rarely, internal organ involvement
Skin manifestations often precede joint symptoms।
Multicentric Reticulohistiocytosis कैसे पहचाने (Diagnosis of MRH)
- Clinical Examination – characteristic skin lesions and symmetrical polyarthritis
- Skin Biopsy / Histopathology – infiltration of histiocytes and multinucleated giant cells with eosinophilic cytoplasm
- Blood Tests – mild inflammatory markers (ESR, CRP) elevation
- Imaging (X-ray / MRI) – joint erosions, destruction
- Rule out underlying malignancy – age-appropriate cancer screening
Histopathology is gold standard for diagnosis।
Multicentric Reticulohistiocytosis इलाज (Treatment of Multicentric Reticulohistiocytosis)
1. Medications
- Disease-modifying antirheumatic drugs (DMARDs): Methotrexate, Leflunomide
- Corticosteroids: reduce inflammation
- Biologics: TNF-alpha inhibitors, IL-6 inhibitors in refractory cases
2. Symptomatic Treatment
- NSAIDs for pain and swelling
- Physical therapy to maintain joint function
3. Surgical Intervention
- Rarely needed for severe joint destruction or joint replacement
4. Monitoring
- Regular follow-up for joint integrity and skin lesion progression
- Periodic cancer screening in adults
Early treatment slows joint destruction and improves quality of life।
रोकथाम (Prevention)
- No guaranteed prevention due to idiopathic nature
- Early detection of skin lesions and joint symptoms
- Monitor for systemic symptoms and malignancies
- Maintain regular follow-ups with rheumatologist and dermatologist
सावधानियाँ (Precautions)
- Prompt reporting of new nodules, swelling, or pain
- Avoid delay in treatment to prevent irreversible joint damage
- Careful use of immunosuppressants under supervision
- Physical therapy to maintain mobility
FAQs (अक्सर पूछे जाने वाले प्रश्न)
Q1. MRH क्या contagious है?
नहीं, यह non-infectious, autoimmune / idiopathic disorder है।
Q2. क्या MRH cure हो सकती है?
Complete cure rare है, लेकिन early DMARDs और biologics से symptom control और joint preservation possible है।
Q3. कौन high risk में है?
Adults aged 40–60, especially women, और rare malignancy-associated cases।
Q4. Skin lesions हमेशा joint symptoms से पहले आते हैं?
अक्सर हाँ, skin lesions पहले दिखाई देते हैं, पर कभी-कभी joint involvement simultaneous होता है।
निष्कर्ष (Conclusion)
Multicentric Reticulohistiocytosis (मल्टीसेंट्रिक रेटिकुलोहिस्टियोसाइटोसिस) एक rare inflammatory disorder है जो skin और joints को प्रभावित करती है।
Early diagnosis, immunosuppressive therapy, physical therapy और regular follow-up से joint destruction रोकना और quality of life बढ़ाना संभव है।
अगर किसी को papulonodular skin lesions, symmetrical joint pain या swelling दिखे, तो तुरंत rheumatologist या dermatologist से consultation करना चाहिए।