Scleroderma with Acro-Osteolysis and Papular Mucinosis Resembling Multicentric Reticulohistiocytosis.

OBJECTIVES

We describe a case of systemic sclerosis (SS) with acro-osteolysis associated with cutaneous mucinosis, usually characterized by mucin deposition in the skin. The main differential diagnosis was multicentric reticulohistiocytosis due to the presentation of papulonodular skin lesions.

MATERIALS AND

METHODS

A physical examination, imaging studies and laboratory tests were performed.

RESULTS

Distal bone resorption was evident on plain radiographs, and skin biopsy confirmed mucinosis. The SS diagnosis was based on the clinical features, high levels of antinucleolar antibodies and typical nailfold capillaroscopy findings.

CONCLUSION

To the best of our knowledge, this is the first description of cutaneous mucinosis accompanying SS with acro-osteolysis.

LEARNING POINTS: Cutaneous mucinosis is rarely associated with systemic sclerosis.Systemic sclerosis with cutaneous mucinosis may resemble multicentric reticulohistiocytosis.