Targeting T Cells to Hit B Cells: Successful Treatment of Cutaneous Plasmacytosis with Topical Pimecrolimus

Abstract

Cutaneous plasmacytosis is a rare disease predominantly found in Japanese patients. We describe the case of a 75-year-old white female with cutaneous plasmacytosis of the face and involvement of the bone marrow. In contrast to other cases of cutaneous plasmacytosis, the patient revealed hypogammaglobulinemia and elevated levels of free light chains in the urine. Treatment with topical pimecrolimus 1%, which primarily targets T cells, led to almost complete clinical and histological remission of the skin lesions. Our report indicates that the therapeutically induced disappearance of the plasma cells was due to indirect T-cell-mediated effects.

Cutaneous and systemic plasmocytosis

“…Both tacrolimus as well as pimecrolimus have repeatedly been employed in the treatment of CSP, usually also with only slight benefit [6,12,36]. In one case pimecrolimus did produce almost complete clearing of both the clinical and histopathologic findings [37]. In other patients the pruritus stopped, a phenomenon that we have also observed with pimecrolimus, which is much appreciated by patients.…”

Pimecrolimus 1% Cream in Non-Specific Inflammatory Recurrent Balanitis

“…Although inflammatory reactions in non-specific balanitis have not been extensively investigated, the fact that a significant percentage of these patients present atopic diathesis might suggest a cytokine involvement analogous to atopic dermatitis inflammation. Pimecrolimus could also exhibit modulatory properties even in cases with plasma cell involvement, as suggested by a recent report [10]. In addition, patients applying pimecrolimus cream when symptoms initialized during the 3-month follow-up period reported significantly fewer days with symptoms than those applying the emollient, suggesting that use of the agent might result in early inhibition of the inflammatory process.…”

“…Pimecrolimus, a derivative of the macrolactam ascomycin, binds to the cytosolic receptor macrophilin-12 and the resulting drug-protein complex and inhibits the phosphatase calcineurin, resulting in the blockage of signal transduction in target cells. It selectively inhibits the release of both Th-1 (IL-2 and interferon-γ) and Th-2 (IL-4 and IL-10) cytokines and of T helper clone, and presents considerable anti-inflammatory properties [4,5,6,7]. …”

Kutane und systemische Plasmozytose

“…Sowohl Tacrolimus als auch Pimecrolimus sind wiederholt zur Therapie der KSP eingesetzt worden, meist ebenfalls nur mit geringer klinischer Wirkung [6,12,36]. Nur in einem Fall kam es unter Behandlung mit Pimecrolimus zu einer fast vollständigen Rückbildung der klinischen und histopathologischen Befunde [37]. Bei anderen Patienten sistierte der Pruritus, ein Phänomen, das auch von uns bei Pimecrolimus beobachtet werden konnte und von der betroffenen Patientin als sehr hilfreich empfunden wurde.…”