Rationale:Blastic plasmacytoid dendritic cell neoplasm (BPDCN), derived from precursors of plasmacytoid dendritic cells, is a rare and aggressive malignancy with frequent cutaneous involvement. Although cutaneous lesions are often chemosensitive, BPDCN portends a poor prognosis as most patients relapse after developing drug resistance.Patient concerns:We report a case of a 65-year-old man who presented with a rapidly enlarging hyperpigmented plaque on his shoulder with subsequent similarly appearing macules and plaques on his chest, back, and neck.Diagnosis:Skin biopsy revealed a dense adnexocentric dermal infiltrate of immature blastoid cells without epidermal involvement. The infiltrate was immunoreactive for CD4, CD56, CD123, and Bcl-2, but negative for CD3, CD8, CD30, MPO, EBER, and ISH. The patient was diagnosed with BPDCN based on these cell markers.Intervention:Bone marrow biopsy and radiologic work-up showed no evidence of extracutaneous involvement. The patient attained partial remission after undergoing 2 rounds of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP regimen) before autologous stem cell transplantation, however, he quickly relapsed and developed new cutaneous lesions.Outcomes:The patient was treated with venetoclax, a Bcl-2 inhibitor, and exhibits complete resolution of prior skin findings and continues to remain free of new cutaneous lesions 10 months posttreatment initiation with venetoclax.Lessons:Herein, we present a case that supports the use of venetoclax, a Bcl-2 inhibitor, in the off-label treatment of BPDCN with Bcl-2 overexpression. Only 1 prior case has reported the off-label use of venetoclax for the treatment of BPDCN. This case highlights a novel therapeutic option for BPDCN patients unresponsive to traditional treatment.
While re-epithelialization of subepidermal blisters is a commonly accepted phenomenon, there are no published data demonstrating its incidence. Because only half of the biopsies showed the classic subepidermal blister, it is important to be aware of the spectrum of histopathologic findings that occur in this disease. Specifically, the presence of an intraepidermal blister and/or epidermal necrosis on routine H&E-stained specimens does not preclude the diagnosis of pemphigoid.
An elderly patient presented with a 4-month history of eroded hypertrophic condylomatous plaques with areas of vesiculation on the groin and lower extremities bilaterally, associated with swelling. Biopsy and immunofluorescence studies confirmed the diagnosis of pemphigus vegetans (PVeg). Further clinical evaluation revealed deep venous thrombosis of the lower extremities and septic shock from secondarily infected pemphigus lesions. Fluid obtained from vesicles was positive for herpes simplex virus (HSV) via PCR-based testing. The patient was therapeutically anticoagulated, treated with high-dose corticosteroid therapy, broad-spectrum intravenous antibiotics and acyclovir. This case represents a constellation of diagnoses not previously described. Although pemphigus vulgaris has been linked to thromboembolic events and has been associated with HSV, these associations have not been previously reported in PVeg. The coexistence of these diseases should encourage vigilance in the clinical work up of a patient with PVeg.
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