AUTOINFLAMMATORY DISEASES IN HEMATOLOGY FROM DIAGNOSIS TO TREATMENT

AUTOINFLAMMATORY_DISEASES_IN_HEMATOLOGY_FROM_DIAGNOSIS_TO_TREATMENTkapak

CUTANEOUS AND SYSTEMIC PLASMACYTOSIS

Serkan Ünal

Kastamonu University, Faculty of Medicine, Department of Hematology, Kastamonu, Türkiye

Ünal S. Cutaneous and Systemic Plasmacytosis. In: Kurt Yüksel M, editor. Autoinflammatory Diseases in Hematology from Diagnosis to Treatment. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.185-190.

ABSTRACT

Cutaneous plasmacytosis (CSP), first described by Yashiro in 1976 and later redefined by Kitamura and others, is a rare condition characterized by red-brown macules, papules, and plaques, with dermal infiltration of mature plasma cells. Serum electrophoresis typically reveals polyclonal hypergammaglobulinemia. The exact cause of CSP remains unclear, though other conditions such as myeloproliferative neoplasms or inflammatory skin disorders must be excluded. CSP predominantly affects individuals of Asian descent, especially in middle-aged men, with cases most commonly reported in Japan. The disease often presents with cutaneous lesions, but systemic involvement such as lymphadenopathy and hypergammaglobulinemia is also common. Diagnosis is based on histopathological findings, clinical presentation, and laboratory tests. Treatment options include topical immunomodulators and systemic agents, although no standard approach exists. The disease has a chronic course, and although spontaneous regression is rare, some patients may experience partial resolution with treatment. Prognosis varies, with factors such as the extent of disease, response to therapy, and systemic involvement influencing outcomes. While CSP is generally not life-threatening, it can lead to significant morbidity, requiring ongoing management and monitoring.

Keywords: Autoimmune diseases; Rare diseases

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