Cutaneous sarcoidosis: a new subset in the spectrum of paraneoplastic dermatoses

Sarcoidosis is a well-described disease that can be associated with various malignancies; however, this correlation is still not fully clarified.

To determine the clinical and histological features, demographic characteristics, and prognosis of patients diagnosed with paraneoplastic sarcoidosis (PS).

This observational cohort prospective study included all patients diagnosed as cutaneous sarcoidosis. All patients were monitored for therapeutic response, prognosis, and any associated diseases or malignancies.

Out of 94 patients with cutaneous sarcoidosis, we identified 12 patients (12.8%) with PS. There was a male (n = 8) preponderance (ratio 2 : 1),and the mean ± SD age of patients was 64.6 ± 13.21 years. The spectrum of paraneoplastic malignancies included six cases of Hodgkin lymphoma, five of multiple myeloma and one of non-Hodgkin lymphoma (NHL; diffuse large B-cell lymphoma). The underlying malignancy was usually diagnosed within 4-25 months (mean ± SD 12.7 ± 6.85) from diagnosis of cutaneous sarcoidosis. The response to systemic steroid was relatively poor and patients on combination therapy showed frequent relapses. However, after treatment of the associated malignancy, seven patients (58.3%) showed complete resolution of the lesions and five (41.7%) showed moderate improvement. During follow-up, there was no relapse reported for either PS or malignancy.

Our results suggest that sarcoidosis is a new subset in the spectrum of paraneoplastic syndrome. We propose that lymphoproliferative disorders should be considered in patients with cutaneous sarcoidosis who have a poor response to conventional therapy or whose skin biopsy shows a significant number of mitoses.