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A Metadata Extraction Approach for Clinical Case Reports to Enable Advanced Understanding of Biomedical Concepts
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[Multicentric reticulohistiocytosis: A case report].
1Department of Rheumatology and Immunology, The 960th Hospital of the PLA Joint Logistics Support Force, Tai'an 271000, Shandong, China.
Summary
Multicentric reticulohistiocytosis (MRH) is a rare multisystem disease presenting with skin nodules and destructive arthritis. Diagnosis relies on clinical, radiographic, and skin biopsy findings, with treatment involving glucocorticoids and immunosuppressants.
Area of Science:
- Rheumatology
- Dermatology
- Pathology
Background:
- Multicentric reticulohistiocytosis (MRH) is a rare multisystemic non-Langerhans cell histiocytosis.
- It is characterized by papulonodular mucocutaneous lesions and destructive arthritis.
- The exact pathogenesis of MRH remains unclear.
Observation:
- A 65-year-old woman presented with generalized erythema, papules, nodules (including a "coral bead" appearance on auricles and hands), and symmetrical polyarthritis with morning stiffness and deformity.
- Laboratory tests revealed elevated inflammatory markers (ESR, CRP), positive rheumatoid factor and antinuclear antibody.
- Radiography showed joint destruction, while skin biopsy demonstrated dermal histiocytic infiltrate with ground-glass cytoplasm, positive for CD68 and negative for S100 and CD1a.
Findings:
- The clinical presentation, radiographic evidence, and histopathological findings confirmed the diagnosis of multicentric reticulohistiocytosis (MRH).
- Skin biopsy is crucial for diagnosis, revealing characteristic histiocytes with ground-glass cytoplasm and CD68 positivity.
- MRH can be mistaken for other autoimmune or rheumatic diseases due to overlapping symptoms.
Implications:
- Early diagnosis and treatment of MRH are essential to prevent progressive joint destruction and manage mucocutaneous lesions.
- Combination therapy with glucocorticoids and immunosuppressants showed satisfactory efficacy in the presented case.
- Increased clinician awareness of MRH is needed to avoid diagnostic delays and ensure appropriate management of this rare condition.
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