Rare presentation of multifocal pilomatrixoma in the head
and neck region
Muhammad Ali Hussain
J. R. Srinivasan
Received: 4 December 2010 / Accepted: 17 May 2011 / Published online: 4 June 2011
Pilomatrixoma is a benign harmless appendageal tumor
with differentiation toward hair cells. It is usually man-
ifested as a solitary, asymptomatic, firm nodule. It may be
dermal or subcutaneous. Frequently, there is a clearly
delimiting capsule. This has long been considered a rare
tumor, but it may be more common than previously
realized. Though more common in children, the occurrence
in adults is being increasingly recognized. The recommen-
ded treatment is surgical excision.
This lesion was originally called a “calcifying epitheli-
oma” by Malherbe and Chenantais in their initial descrip-
tion in 1880. In 1961, Forbis and Helwig proposed the
current name to more accurately signify the origin of the
tumor. Other rare clinical types of calcifying epithelioma of
Malherbe are the bullous form and the perforating type.
Histologically, the lesion matures by basophilic cells
moving from the periphery towards the center of the lesion.
En route, 'shadow cells' form as they lose their nuclei and
gain keratin. Immature hairs and other pilosebaceous
structures may be seen. Calcification occurs in the shadow
cells and the stroma.
Very large tumors can cause considerable discomfort but
are uncommon. Malignant change is uncommon, but where
it occurs, it is locally invasive and can cause visceral
metastases and death. Most reported cases have occurred in
the Caucasian population. Whether this represents publica-
tion bias or a true racial predisposition is unclear.
Most studies report a slight preponderance in females. In
one retrospective study of 209 cases published in 1998, the
female-to-male ratio was 1.5:1 . Most reported cases
have occurred in children. Lesions are often discovered in
the first 2 years of life; however, in the same study,
investigators found the age of presentation showed a
bimodal pattern, with the first peak being 5–15 years and
the second being 50–65 years .
We are reporting an 11-year-old fit and healthy Caucasian
male child with the rare presentation of multiple piloma-
trixoma in the head and neck region. In 2005 when he was
6 years of age, he presented with a history of a slowly
growing painless lump on the left nasal bridge area having
had duration of a few months. The size of the nodule was
8×8 mm. It was firm to hard with sharp-defined margins
(Fig. 1). A clinical diagnosis of pilomatrixoma was made
and was treated with excision biopsy for diagnostic
purposes. Histology confirmed the diagnosis of pilomatrix-
oma. He was later discharged from the clinic.
The patient again attended the clinic after 5 years; he had
two more lesions, one on each lower eyelid (Fig. 2). The
lesion on the left lower eye lid was of the size of 5×3×2 mm
and the lesion on the right lower eyelid was of the size of 5×
2×3 mm. Clinical diagnosis of multiple pilomatrixoma was
made and they were again removed under GA and histology
again confirmed both of these lesions as pilomatrixoma.
To our understanding, this type of multiple presentation
of pilomatrixoma in the head and neck region is very rare.
There is only one case reported in the Turkish literature in
The objective of this article is to describe a rare presentation of
multiple pilomatrixoma in a child 11 years of age and to review the
literature concerning this entity.
M. A. Hussain (*)
J. R. Srinivasan
Plastic and Reconstructive Surgery Department,
Royal Preston Hospital,
PR2 9HT, Preston, UK
Eur J Plast Surg (2012) 35:697–699