PilomatrixomaD23.L

Author:Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 07.05.2022

Dieser Artikel auf Deutsch

Synonym(s)

calcified epithelioma; calcifying epithelioma of malherb; epithelioma calcificans; Epithelioma calcificans Malherbe; Epithelioma calcified; Malherb's epithelioma; Malignant tumour; Pilomatricoma; Pilomatrikom; Pilomatrixoma; Pilomatrixoma proliferating; Trichomatricoma

Requires free registration (medical professionals only)

Please login to access all articles, images, and functions.

Our content is available exclusively to medical professionals. If you have already registered, please login. If you haven't, you can register for free (medical professionals only).


Requires free registration (medical professionals only)

Please complete your registration to access all articles and images.

To gain access, you must complete your registration. You either haven't confirmed your e-mail address or we still need proof that you are a member of the medical profession.

Finish your registration now

HistoryThis section has been translated automatically.

A. Painted sherbet 1880

DefinitionThis section has been translated automatically.

Frequent, mostly solitary, rarely multiple, benign adnexal tumor originating from the hair matrix cells and calcifying early with hair follicle differentiation.

Occurrence/EpidemiologyThis section has been translated automatically.

Familial clusters have been described in the context of Gardner and Rubinstein-Taybi syndrome and in association with myotonic dystrophy.

EtiopathogenesisThis section has been translated automatically.

Controversial. Association with bcl-2 expression as well as mutations in the CTNNB1 gene and dysregulations in beta-catenin/LEF expression are discussed.

ManifestationThis section has been translated automatically.

At initial manifestation > 50% of patients are < 18 years of age. Manifestation peak: 2nd-15th LJ and 45th-65th LJ.

LocalizationThis section has been translated automatically.

Face, neck, back, preauricular.

Clinical featuresThis section has been translated automatically.

Solitary, rarely multiple, painless, hard to bony, sharply defined, 0.5-3 cm in diameter (rarely larger - giant pilomatricoma), subcutaneous, skin-colored, reddened or reddish-brown nodule. Frequently calcifications, more rarely ossifications. Very slowly progressive, usually growing over several years.

Rare clinical variants are.

  • anetodermic variant (skin over the tumor appears atrophic)
  • pigmented pilomatrixoma
  • ulcerated pilomatrixoma
  • giant forms (up to 20 cm in size)
  • multiple pilomatrixomas possibly in association with Turner syndrome, trisomy 9, Gardner syndrome
  • multiple familial pilomatrixomas

HistologyThis section has been translated automatically.

Lobular tumor in the middle to deep dermis, in some places also extending into the subcutis. The appearance depends on age (regressive changes). Basophilic cells with hyperchromatic nuclei and some (sometimes numerous) mitoses are localized in the outer layer. Centrally, there are usually eosinophilic cells (shadow cells). Older tumors show extensive zones of necrosis and metaplastic calcifications. Regressive tumors are surrounded by dense granulation tissue.

DiagnosisThis section has been translated automatically.

Clinic is characteristic, histology is conclusive.

Differential diagnosisThis section has been translated automatically.

Complication(s)This section has been translated automatically.

In very rare cases malignant degeneration is possible (not in childhood). S.u. Pilomatrix carcinoma.

TherapyThis section has been translated automatically.

Excision in toto.

LiteratureThis section has been translated automatically.

  1. Fernandes R et al (2003) Giant pilomatricoma (Epithelioma of Melherbe): Report of a case and review of literature. J Oral Maxillofac Surgery 61: 634-636
  2. Fetil E et al (2002) Multiple pilomatricoma with perforation. Int J Dermatol 41: 892-893
  3. Grabczynska SA et al (2002) Case 3: Multiple familial pilomatrixoma. Clin Exp Dermatol 27: 343-344
  4. Gromiko N (1927) On the knowledge of the malignant transformation of the calcified skin epithelioma. Arch Catholic Anat 265: 103-116
  5. Haferkamp B et al (1999) Pilomatrix carcinoma in an unusual localization. dermatologist 50: 355-359
  6. Hardisson D et al (2001) Pilomatrix carcinoma: a clinicopathologic study of six cases and review of the literature. At J Dermatopathol 23: 394-401
  7. King IC et al (2015) Multiple familial pilomatrixomas in three generations: an unusual clinical picture. Pediatric dermatol 32: 97-101
  8. Malherbe A, Chenantais J (1880) Note sur l'épitheliome calcifié des glandes sébacées. Prog Med 8: 826-828
  9. Robinson AJ et al (2016) Multiple familial pilomatrixomas in the absence of other clinical features: a case of familial benign pilomatrixoma. Australas J Dermatol 57:75-76
  10. Simon RS, Sanchez-Yus E (2002) Multinodular pilomatrixoma. Dermatology 204: 80-81
  11. Wang J et al (2002) Pilomatrixoma: clinicopathologic study of 51 cases with emphasis on cytologic features. Diagn Cytopathol 27: 167-172
  12. Zamecnik M (2000) Cell death in pilomatricoma. J Cutan Catholic 27: 100

Authors

Last updated on: 07.05.2022