Abstract
Multicentric reticulohistiocytosis (MRH) is a very rare systemic disease with variable phenotypic presentation and a high rate of misdiagnosis. Here we describe a patient with MRH and extra-mammillary Paget’s disease (EMPD), a diagnosis that has not previously been described in the literature.
Highlights
Multicentric reticulohistiocytosis (MRH) is a very rare disease and was first identified in 1936 [1,2,3,4,5,6]
Owing to the frequency of accompanying joint symptoms, MRH is misdiagnosed as rheumatoid arthritis or dermatomyositis
There is no specific treatment for this disease, but inflammation is kept under control through the use of glucocorticoids, immunosuppressants, biological agents, Tumor necrosis factor (TNF)-α, IL-1β, tripterygium glycosides and Non-steroidal anti-inflammatory drugs (NSAIDs)
Summary
Multicentric reticulohistiocytosis (MRH) is a very rare disease and was first identified in 1936 [1,2,3,4,5,6]. Scratching the rash resulted in the release of clear fluid She went to local hospitals several times and was diagnosed with eczema. The patient eventually experienced difficulty with flexion of both upper and lower limbs, such that walking became difficult Two months later, she noticed that multiple red papules had appeared on her neck, upper chest and the distal portion of her dorsal finger. The patient lost 3 kg from her baseline weight She was married with a son and a daughter, both of whom were healthy. Physical examination revealed multiple light-red papules on the forehead, ears, scalp, neck (Figure 1), upper chest and distal dorsal finger. A telephone follow-up at 1 month revealed that the patient’s MRH was under control, and the patient was engaged in ongoing treatment for her EMPD
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