Multiple Keratoacanthoma-like Syndromes: Case Report and Literature Review

Medicina (Kaunas). 2024 Feb 22;60(3):371. doi: 10.3390/medicina60030371.


Keratoacanthoma (KA) is a fast-growing skin tumor subtype that can be observed as a solitary lesion or rarely as multiple lesions in the context of rare genetic syndromes. Syndromes with multiple keratoacanthoma-like lesions have been documented as multiple self-healing squamous epithelioma (Ferguson-Smith syndrome), eruptive keratoacanthoma of Grzybowski, multiple familial keratoacanthoma of Witten and Zak Muir-Torre syndrome, and incontinentia pigmenti. The treatment approach of those entities is challenging due to the numerous lesions, the lesions' undefined nature, and the co-existence of other malignant skin tumors. Herein, we report a case of a 40-year-old woman who developed multiple treatment-resistant Ferguson-Smith-like keratoacanthomas with a co-existing large and ulcerated invasive squamous cell carcinoma and microcystic adnexal carcinoma on the scalp. Multiple keratoacanthomas on her extremities were successfully treated with oral acitretin (0.5 mg/kg/day) in combination with topical Fluorouracil (5-FU) 5%, while excision and plastic surgery restoration were performed to treat the ulcerated cancer lesion on her scalp. Due to the interesting nature of this rare syndrome, we performed a literature review including case reports and case series on multiple-KA-like lesions syndromes and focusing on diagnosis and therapy approaches. We also conducted a comparison of patient reports, which included assessing the clinical appearance of the lesions and evaluating the success and progress or the failure of various treatment approaches that were implemented.

Keywords: Ferguson–Smith syndrome; Muir–Torre syndrome; eruptive keratoacanthoma of Grzybowski; incontinentia pigmenti; keratoacanthoma; multiple familial keratoacanthoma of Witten and Zak.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Acitretin / therapeutic use
  • Adult
  • Carcinoma, Squamous Cell* / diagnosis
  • Female
  • Fluorouracil / therapeutic use
  • Humans
  • Keratoacanthoma* / diagnosis
  • Keratoacanthoma* / drug therapy
  • Keratoacanthoma* / pathology
  • Skin Neoplasms* / diagnosis
  • Skin Neoplasms* / drug therapy


  • Acitretin
  • Fluorouracil

Grants and funding

The study received no external funding.