Arthritis and interstitial granulomatous dermatitis (Ackerman syndrome) with pulmonary silicosis

To describe the case of a patient suffering from pulmonary silicosis associated with a rheumatoid factor negative, antinuclear antibody positive, symmetrical, nonerosive synovitis, and interstitial granulomatous dermatitis (IGD) and compare it with similar cases reported in the literature.

Literature search to identify published cases of IGD with arthritis and cases associated with silicosis.

Thiry-eight cases of IGD with arthritis were found. These cases were associated with various conditions such as drug reactions, autoimmune diseases, chronic infections, or paraneoplastic syndromes for which no specific underlying cause was identified. The patient had skin lesions corresponding to the rope sign, as noted in other reports. Histology showed a histiocytic, granulomatous dermatitis, which, in association with arthritis, was first described by Ackerman et al. Circulating immune complexes or altered apoptosis have been discussed as mechanisms, although there is no experimental evidence to support either hypothesis. As in other cases, treatment had limited success. Most relief was achieved with hydroxychloroquine, dapsone, and corticosteroids.

Arthritis and IGD associated with silicosis is a rare clinical entity that can be differentiated from other conditions. This condition should be considered when patients present with typical dermatologic features, such as the rope sign, and arthritis.