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Plasma cell orificial mucositis is a relatively rare condition which may occur on mucous membranes. Plasmoacanthoma is a verrucous tumor with plasmacytic infiltration and has been described as a separate disease entity. Such a tumor is supposed to occur without lesions which could be compatible with plasma cell orificial mucositis. The present case report documents the coexistence of both conditions in one single patient. This observation suggests that both conditions are part of one single disease entity.

A patient with Sweet's syndrome and leukopenia is reported. Hematological evaluation revealed hairy cell leukemia (HCL). The clinical picture was dominated by persistent fever, which is a common feature of both Sweet's syndrome and HCL. Since fever frequently reflects concomitant infection in HCL, a thorough search for infectious disease was performed. Blood cultures grew Mycobacterium kansasii. The patient recovered after treatment with recombinant interferon-alpha (r-IFN-alpha) and tuberculostatic drugs. Remarkably, the skin lesions completely regressed within 1 week after the start of r-IFN-alpha. In the literature, Sweet's syndrome is rarely mentioned as a feature of HCL. Mycobacterial disease, especially atypical mycobacteria, is relatively often seen in HCL.

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