Successful Treatment of Generalized Eruptive Keratoacanthoma of Grzybowski with Acitretin

Abstract

Introduction Keratoacanthomas (KA) are common cutaneous skin tumors originating from the hair follicles. Unlike squamous cell carcinoma, KA can regress spontaneously and have a benign evolution. Solitary KA is the most common form but familial multiple KA (Ferguson–Smith type), genetically predisposed KA (such as in xeroderma pigmentosum, or Muir–Torre syndrome), or sporadic multiple eruptive KA (Grzybowski type) have been described. Generalized eruptive KA of Grzybowski (GEKA) is a rare condition (around 40 reported cases). The pathophysiology is still unclear. Human papillomavirus (HPV) has been detected in sporadic KA but the presence of HPV39 has never been reported, to our knowledge, in GEKA. Case Report GEKA in an 80-year-old woman was successfully treated with acitretin (0.5 mg/kg/day) combined with surgical removal of the largest lesions. Treatment was well tolerated and led to decreased pruritus and tumor regression within 6 months. The presence of HPV39 was detected in a lesion by polymerase chain reaction and Sanger sequencing. No genetic alteration was found, in particular in the genes usually altered in squamous cell carcinoma (including NOTCH1 , NOTCH2 , CDKN2A , TP53 ). Conclusion We report a case of GEKA associated with the presence of HPV39 and the successful use of acitretin combined with surgical removal of the larger lesions.

Grzybowski’s Generalized Eruptive Keratoacanthomas in a Patient with Terminal Kidney Disease—An Unmet Medical Need Equally Ameliorated by Topical Imiquimod Cream and Lapacho Tea Wraps: A Case Report

“…Widespread appearance of skin lesions on the body may also occur in connection with infectious agents such as HPV. The absence of viral material in a representative lesion in our patient is in line with findings of some authors [ 14 ], while others identified certain subtypes such as HPV39 in KA [ 10 ]. It should be mentioned that even sophisticated molecular laboratory tests may fail to detect HPV, as positivity is often lost in regressing warts.…”

“…GEKA is a rare condition with high disease burden, typically being more pruritic than singular lesions of KA. The papules, nodules, and tumors are widespread and may arise on all parts of the body, including mucosa [ 10 ]. The etiology of GEKA remains elusive; however, well-described risk factors include a variety of autoimmune diseases (e.g., rheumatoid arthritis, Sjögren’s syndrome), immunomodulating agents, and hematologic malignant diseases (e.g., multiple myeloma, myelodysplastic syndrome).…”

Case for diagnosis. A pruritic eruption of keratotic papules over the face and neck

“…GEKA is a rare variant of multiple keratoacanthomas, with only around 40 cases reported. 1 Its onset most commonly occurs between the fifth and seventh decades, without sex predilection. 2 Unlike other types of multiple keratoacanthomas, such as Ferguson-Smith or Witten and Zak, all cases are sporadic.…”

“…Nonetheless, several factors have been proposed to play a role in its pathogenesis, including trauma, immunological abnormalities, ultraviolet radiation, chemical carcinogens, and viruses, particularly human papillomavirus. 1 , 3 , 6 …”

The third‐generation retinoid adapalene triggered DNA damage to induce S‐phase arrest in HaCat cells

“…In addition, retinoids were also widely used for the experiments of diverse human cancers due to their influences on the cell proliferation and apoptosis of multiple tumor cells . Currently, the commonly used retinoids were all‐trans‐retinoic acid (ATRA), acitretin, isotretinoin, and tazarotene . However, repeated and long‐term use of these drugs often caused a lot of adverse reactions like allergic reaction, dry skin, piercing, photosensitivity, and skin infection.…”