Blastic Plasmacytoid Dendritic Cell Neoplasm

Blastic plasmacytoid dendritic cell neoplasm presenting as violaceous forehead plaque

“…Plasmacytoid dendritic cells comprise less than 0.5% of circulating mononuclear cells and are classically found in lymph nodes and tonsils [6]. They are absent in healthy skin, but in certain dermatological conditions such as contact dermatitis and lupus erythematosus, they are recruited to the skin where they secrete massive amounts of IFN1, IFNalpha, and IFNbeta [2].…”

“…The presentation of this disorder is highly heterogenous and has a high frequency of cutaneous and bone marrow involvement [1]. Skin manifestations are often the first symptoms that cause a patient to seek medical care and these can precede leukemic spread [1,2]. Dermatological presentations vary between bruise-like macules to isolated violaceous nodules and may be limited and focal or diffuse [3].…”

“…Dermatological presentations vary between bruise-like macules to isolated violaceous nodules and may be limited and focal or diffuse [3]. This range of clinical presentations sometimes creates a diagnostic pitfall, with BPDCN being mistaken for traumatic purpura, inflammatory conditions such as contact dermatitis, or leukemia cutis [2]. The prognosis for BPDCN is poor, and diagnostic delay can prevent patients from receiving early and aggressive treatment, which is the only approach shown to improve outcomes [2,3].…”

“…This range of clinical presentations sometimes creates a diagnostic pitfall, with BPDCN being mistaken for traumatic purpura, inflammatory conditions such as contact dermatitis, or leukemia cutis [2]. The prognosis for BPDCN is poor, and diagnostic delay can prevent patients from receiving early and aggressive treatment, which is the only approach shown to improve outcomes [2,3]. We present a case of BPDCN presenting as a violaceous plaque on the forehead in an otherwise asymptomatic man.…”

Clinical Insights into the Management of Blastic Plasmacytoid Dendritic Cell Neoplasm

“…The bruise-like infiltrates lesions, most characteristic for BPDCN, could be an early manifestation of neoplastic cell infiltration with red blood cell extravasation in the skin. 14 The Modified Skin Weighted Assessment Tool (mSWAT) can be used to estimate and monitor the extent and severity of cutaneous disease. 15 …”