Pemphigus vegetans with coexistent herpes simplex infection and deep venous thrombosis of the lower extremities

Abstract

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.

Hallopeau type of pemphigus vegetans, an unusual clinical presentation of an infrequent disease

“…The histopathology of the vegetative lesions shows suprabasal intraepidermal clefts, acantholytic cells, pseudoepitheliomatous hyperplasia, and intraepi- The exact pathophysiology is unknown but it has been associated with HIV infection, herpes simplex, deep vein thrombosis, use of intranasal cocaine, solid organ neoplasms, and medications [1,[3][4][5].…”

“…It is more common in women and the age at diagnosis is 40 to 60 years [2]. A triggering factor is identified in several cases, such as medications, infections, or malignancies [3]. We report the case of a woman in the fourth decade of life with a history of hypothyroidism, who presented with vegetative plaques of the axillae and recent appearance of painful erosions in the oral cavity.…”

Venous thromboembolism in viral diseases: A comprehensive literature review

“…It was seen that Pemphigus is linked with a greater incidence of thromboembolism and PV is linked to HSV. 8 …”

“…Treatment was with anticoagulation drugs, high‐dose prednisone, broad‐spectrum antibiotics, anti‐fungal drugs, acyclovir, and aggressive fluid resuscitation. It was seen that Pemphigus is linked with a greater incidence of thromboembolism and PV is linked to HSV 8 …”

“…6,7 Some supplementary studies have reported DVT and cerebral venous thrombosis associated with Herpes Simplex Virus (HSV). 8,9 Hepatitis C virus (HCV) infection accompanies VTE with a prevalence of 0.8%. 10 The most frequent site of vein thrombosis is the portal vein in cirrhotic patients.…”