Orphanet: Pilomatrix carcinoma

Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.


Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

Pilomatrix carcinoma

Disease definition

A rare skin tumor characterized by an asymptomatic, solitary, often ulcerated nodule most commonly located in the face, involving the deep dermis, subcutaneous tissue, and skeletal muscle and fascia. Histopathologically, the lesion is composed of aggregates of atypical basaloid cells with numerous mitoses. Typical features include shadow cells, keratin cysts, and trichohyalin and keratohyalin granules. The tumor is locally aggressive and shows a tendency to recur after incomplete excision. Regional lymph node or visceral metastasis has been reported.


Classification level: Disorder
  • Synonym(s):
    • Calcified epithelial carcinoma of Malherbe
    • Calcifying epitheliocarcinoma
    • Malignant pilomatricoma
    • Trichomatrical carcinoma
  • Prevalence: -
  • Inheritance: -
  • Age of onset: Adolescent, Adult, Elderly
  • ICD-10: C44.3  C44.4
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.