Multicentric reticulohistiocytosis

Abstract

Multicentric reticulohistiocytosis is a rare systemic granulomatous disease of an unknown cause, characterized by distinct histopathology. The skin, mucosa, synovial, bone, and internal organs may be involved. Cutaneous nodules and distinctive arthritis are the most prominent clinical features. A 55-year-old female was referred from Orthopedic Outpatient Department, with multiple, painful and tender nodules on the dorsum of her hands, forearms, elbows, back, and neck. The lesions were present predominantly around the joints with associated arthropathies. Smaller nodules were seen on the ear helices. There was no other clinically evident or investigative abnormality. A histopathological study confirmed the diagnosis of multicentric reticulohistiocytosis.

Multicentric reticulohistiocytosis: A case report with review

“…The finding of dermal infiltration of mononuclear and multinucleated giant cells with eosinophilic "ground glass" PAS positive cytoplasm is typical of MRH [15]. These cells are positive for TRAP, CD68, lysozyme and human alveolar macrophage-56 (HAM-56) whereas negative for S100protein, CD1a and factor XIIIa [3]. However it is noteworthy to mention that Bialy Nicki-B R et al [7] had observed S100 positivity.…”

“…While inflammatory joint disease is a frequent presenting symptom, and is sole symptom in 45% of patients, skin disease is the presenting manifestation in 30% of patients [10]. Arthropathy usually precedes skin lesions (50%); skin lesions may precede disease (25%) or both can occur concurrently (25%) [3]. It manifests with diffuse, symmetric, progressive and destructive polyarthritis affecting interphalangeal joints mainly the distal interphalangeal joints (75%) and other joints like knees, wrists, shoulders and elbows.…”

“…The appearance of papules in the periungual region is described as "coral beads" [5]. Involvement of oral and nasal mucosa is seen in roughly one-third of the cases [3]. Constitutional symptoms like fever, malaise, weakness, weight loss, anorexia may be present and the disease can involve multiple organ systems including cardiac and skeletal muscle, the pleura and gastrointestinal tract [4,11].…”

“…Multicentric reticulohistiocytosis is a rare, idiopathic, multisystem disorder characterised by the association of papulonodular lesions on skin and mucous membranes along with destructive arthritis [1,2]. Also known as lipoid dermatoarthritis, the disease has the potential to involve almost any organ in the body apart from skin, bone, tendons and joints [3]. It is reported to be associated with malignancy, autoimmune diseases, hyperlipidaemia and tuberculosis [4].…”

Multicentric Reticulohistiocytosis

“…Skin involvement generally establishes with translucent reddish-brown to flesh-colored papulonodules varying from 1-2 mm to 1 cm in diameter or larger. In MRH, papulonodular skin lesions are often associated with arthritis [2].…”