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5.
Pediatr Dermatol ; 41(1): 96-99, 2024.
Article in English | MEDLINE | ID: mdl-37515487

ABSTRACT

Serine/threonine kinase 4 deficiency (STK4 or MST1, OMIM:614868) is an autosomal recessive (AR) combined immunodeficiency that can present with skin lesions such as epidermodysplasia verruciformis-like lesions (EVLL). Herein, we describe a 17-year-old male patient born from consanguineous parents presenting with recurrent respiratory infections, verruciform plaques, poikiloderma, chronic benign lymphoproliferation, and Sjögren syndrome with suspected interstitial lymphocytic pneumonia.


Subject(s)
Epidermodysplasia Verruciformis , Primary Immunodeficiency Diseases , Skin Diseases , Male , Humans , Adolescent , Epidermodysplasia Verruciformis/diagnosis , Epidermodysplasia Verruciformis/genetics , Epidermodysplasia Verruciformis/pathology , Papillomaviridae , Primary Immunodeficiency Diseases/diagnosis , Protein Serine-Threonine Kinases , Intracellular Signaling Peptides and Proteins
11.
Int J Dermatol ; 61(11): 1325-1335, 2022 Nov.
Article in English | MEDLINE | ID: mdl-34403500

ABSTRACT

Acquired epidermodysplasia verruciformis (AEV) is a form of epidermodysplasia verruciformis (EV) that is most commonly found in immunocompromised or immunosuppressed patients. EV is commonly associated with human papillomavirus (HPV), which is often found in EV and AEV lesions. Clinical presentation of AEV in patients with organ transplantation, HIV+, congenital HIV+, hematological diseases, and other iatrogenic immunosuppression are reviewed. Treatment options include topical cidofovir, topical retinoids, topical imiquimod, topical glycolic acid, HPV 9-valent vaccine, acitretin, improving cellular immunity, and changing transplant medication to mycophenolate mofetil.


Subject(s)
Epidermodysplasia Verruciformis , HIV Infections , Papillomavirus Infections , Vaccines , Acitretin/therapeutic use , Cidofovir/therapeutic use , Epidermodysplasia Verruciformis/diagnosis , Epidermodysplasia Verruciformis/drug therapy , HIV Infections/complications , Humans , Imiquimod/therapeutic use , Mycophenolic Acid/therapeutic use , Papillomaviridae , Papillomavirus Infections/complications , Vaccines/therapeutic use
13.
J Dermatol ; 48(9): 1414-1418, 2021 Sep.
Article in English | MEDLINE | ID: mdl-34212429

ABSTRACT

Few articles have described the difference between epidermodysplasia verruciformis (EV) and generalized verrucosis (GV). This study aimed to analyze the clinical findings and virology of the two diseases. The study enrolled patients diagnosed with EV and GV by clinical and histopathological findings. The demographic information, clinical manifestation, treatment, and therapeutic outcome were analyzed. All of the biopsy specimens of enrolled patients were sent for ß-human papillomavirus (HPV) DNA detection. A total of 13 cases were included in the present study, including five EV cases and eight GV cases. Legs and feet were significantly affected in GV cases (p = 0.035). All acquired EV cases had immunocompromised conditions, while only three GV cases could be identified as having an immunocompromised history. All EV cases were detected with ß-HPV infection, especially HPV5, but none of the GV cases were found to be infected with ß-HPV. The therapeutic response was refractory and often relapsed after discontinuation of treatment in both groups.


Subject(s)
Epidermodysplasia Verruciformis , Papillomavirus Infections , Epidermodysplasia Verruciformis/diagnosis , Epidermodysplasia Verruciformis/therapy , Humans , Immunocompromised Host , Papillomaviridae , Papillomavirus Infections/complications , Papillomavirus Infections/diagnosis
14.
Am J Dermatopathol ; 43(1): 71-74, 2021 Jan 01.
Article in English | MEDLINE | ID: mdl-32568838

ABSTRACT

ABSTRACT: Epidermodysplasia verruciformis (EV) is a rare dermatologic condition that is clinically characterized by flat, cutaneous, verrucous papules, pityriasis versicolor-like lesions, and similar lichenoid papules. There are 2 forms of EV: a classic inherited genodermatosis and a secondary acquired form. EV predisposes individuals to infections with certain types of human papillomavirus virus and subsequently increases the risk of cutaneous squamous cell carcinoma. The acquired form occurs in immunosuppressed patients, particularly in patients infected with HIV; however, it has also been described in patients who have undergone stem cell and solid organ transplantation. We report an additional case of renal transplantation and immunosuppressive therapy-associated acquired EV (AEV) in a 78-year-old man with multiple flesh-colored to violaceous, flat-topped papules distributed on the face and trunk clinically mimicking lichen planus. Biopsy was typical for that of EV, demonstrating enlarged keratinocytes with a blue-gray cytoplasm, a thickened granular layer, acanthosis, and hyperkeratosis. Herein, we discuss an unusual presentation of an AEV-mimicking lichen planus with review of the literature.


Subject(s)
Epidermodysplasia Verruciformis/virology , Immunocompromised Host , Immunosuppressive Agents/adverse effects , Kidney Transplantation/adverse effects , Acitretin/therapeutic use , Adult , Aged , Biopsy , Diagnosis, Differential , Epidermodysplasia Verruciformis/diagnosis , Epidermodysplasia Verruciformis/drug therapy , Epidermodysplasia Verruciformis/immunology , Female , Humans , Lichen Planus/diagnosis , Male , Middle Aged , Treatment Outcome , Young Adult
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