Disfiguring Generalized Verrucosis in an Indonesian Man With Idiopathic CD4 Lymphopenia

Abstract

Background: Human papillomavirus (HPV) infections cause a spectrum of clinical disease states, depending on the causative HPV and the characteristics of the infected host, especially the status of cell-mediated immunity. Generalized verrucosis is an unusual clinical presentation of a disseminated HPV infection associated with severe immunodeficiency status. Observations: We present a case of extreme disfigurement associated with an HPV-2 (common wart virus) infection. Virologic studies, immune status of the patient, and treatment(s) are summarized. Conclusions: The severe disfigurement of this patient was a result of an underlying severe immunodeficiency, permissive for a disseminated HPV-2 infection that was allowed to progress for many years before the initiation of therapy. Such a rare case illustrates the natural history of generalized verrucosis in the setting of severe immunodeficiency in the absence of sustained medical interventions. Medical and surgical treatments resulted in marked improvement in the general health of this patient, as well as improvement of the disfigurement that resulted from the generalized verrucosis.

Idiopathic CD4 Lymphocytopenia: Spectrum of opportunistic infections, malignancies, and autoimmune diseases

“…Only English language papers were reviewed. We found 258 cases diagnosed with ICL in 143 published papers[134567891011121314151617181920212223242526272829303132333435363738394041424344454647484950515253545556575859606162636465666768697071727374757677787980818283848586878889909192939495969798…”

Bilateral thick crusts on the legs

“…1 Generalized verrucosis is associated with immunodeficiency syndromes and demonstrates koilocytes on histopathology. 10 The initiating factor for our patient's behavior was likely his underlying psoriasis and stasis dermatitis and subsequent reported pain and immobility. However, given his history of not bathing, hermit-like existence, and severity of the lesions, a contributing psychiatric diagnosis is highly likely.…”

Generalized verrucosis: A review of the associated diseases, evaluation, and treatments

“…73,[76][77][78][79] In addition to HPV infection causing generalized verrucosis, other presentations of HPV associated with this disease include juvenile laryngeal papillomatosis and recurrent vulvar intraepithelial neoplasia. 80 HPV-2 is believed to be a more benign strain of HPV as compared with EV strains, which frequently lead to malignancy. This observation is supported by the case of a 39-year-old Indonesian man who had clinical signs of the disease for over 25 years (Fig 6).…”

“…The extensive cutaneous horns caused much functional impairment and physical disfigurement, but there have been no skin cancers to date. 80 Another interesting case with CD4 1 lymphocytopenia involved a patient with intestinal lymphangiectasia, a protein-losing enteropathy marked by hypogammaglobulinemia, hypoalbuminemia, lymphopenia, and generalized verrucosis. 37 In this disease, na€ ıve CD4 1 lymphocytes are shed through the gastrointestinal tract through the protein-rich lymphatic fluid.…”

“…The presence of HPV-2 in generalized verrucosis combined with cutaneous horn formation is a typical example in which HPV detection may help guide therapeutic interventions. 80 HPV typing systems will help us in the future to identify new cutaneous HPVs. Real-time polymerase chain reaction protocols for the determination of viral copy number help to verify the dominant HPV type in infections with numerous HPV types.…”