Trichoepithelioma Papulosum Multiplex

Abstract

Trichoepithelioma papulosum multiplex is an uncommon autosomal dominant disorder in which multiple trichoepitheliomas are seen. Its cause may be a defective tumor suppressor gene. Studies have mapped this gene to the 9p21 locus. However, there is a parallel or identical syndrome of multiple trichoepitheliomas and cylindromas. Within a given family, some members may have cyclindromas whereas others may have trichoepitheliomas or a combination of both. Although preliminary evidence suggests a different gene is responsible, it is possible that TPM may be caused by more than two independent genes, with some cases of TPM due to impairment of the gene for cylindromatosis. This entity, as well as other disorders with multiple appendageal tumors, may require clarification and distinction from TPM.

Multiple Non- Familial Trichoepitheliomas in a NineYear Child

“…Various synonymous terms e.g., trichoepitheliomapapulosum multiplex (TPM), epithelioma adenoids cysticum, acanthoma adenoids cysticum, multiple benign cystic epithelioma, Brooke's tumour or Brooke-Fordyce trichoepitheliomaare used for TE. 4 They are classified as solitary sporadic, multiple familial (MFT) and desmpoplastic. 5 insurrounding fibrous stroma.…”

Multiple Trichoepitheliome

“…Diskutiert wird der Defekt eines Tumor-Suppressor-Gens, das der Chromosomenregion 9p21 zugeordnet wird [22]. Der mit dem Gendefekt verbundene Verlust der Heterozygotie könnte dabei ursächlich für die Tumorentstehung verantwortlich sein [14,23]. Die histopathologischen Merkmale der Trichoepitheliome sind vielschichtig [1].…”

Brooke–Spiegler syndrome

“…It is characterized by trichoepithelioma papulosum multiplex (TPM), cylindromas and, occasionally, spiradenomas. Within a given family with TPM, some members may have cylindromas, whereas others may have trichoepitheliomas or a combination of both 8–12 . We describe the case of a 30‐year‐old man with multiple trichoepitheliomas and multiple cylindromas.…”

“…Within a given family with TPM, some members may have cylindromas, whereas others may have trichoepitheliomas or a combination of both. [8][9][10][11][12] We describe the case of a 30-year-old man with multiple trichoepitheliomas and multiple cylindromas. TPM was first reported separately by Brooke 13 and Fordyce 14 in 1892; Spiegler 15 is credited with reviewing extensive case material of cylindromatosis in 1899 more than 50 years after its original description by Ancell.…”