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Pilomatricoma - Calcifying Epithelioma of
Malherbe: Case Report and Review of the
Literature
I
Atiya Mahboob, Farrukh Iqbal, Zafar Iqbal, Sabia Riaz
Department of Medicine, Shaikh Zayed Medical Complex, Lahore.
SUMMARY
A case ofpilomatricoma is described in a 13-year old girl who presented with a small stony
hard mass on her right thigh. Malignant change can occur, therefore such lesion should be
removed completely.
INTRODUCTION
C
utaneous calcification and ossification occurs
in association with a wide variety of
conditions, including inflammatory processes,
neoplasms, scarring and metabolic conditions. The
most common cutaneous neoplasm with ossification
is the pilomatricoma l .
CASE REPORT
A 13-year old girl presented to Skin Outpatient
Department, Shaikh Zayed Hospital in February
1999 with a small mass on middle of lateral aspect
of her right thigh for the last 6 years. It was slowly
growing and painful on pressure. There was no
history of trauma at the site of the lesion, neither
there was any history of bleeding or discharge from
the mass. Systemic review was unremarkable.
Cutaneous examination revealed a mobile, tender,
subcutaneous nodule measuring 1.5 cm x 0.5 cm in
size, with stony hard consistency, in the middle of
the lateral aspect of her right thigh. The overlying
skin was normal. Regional lymph nodes were not
palpable. Provisional diagnosis of calcinosis cutis
was made. Excision biopsy of nodule was done and
submitted for histopathological examination.
Macroscopic examination of the serial sections
of 1.5x0.5 cm nodule showed multiple greyish
white solid areas.
Microscopic examination of the lesion revealed
the keratinized, stratified squamous skin with
features of hyper and parakeratosis. There was
dense fibrosis with mild chronic inflammatory cells
infiltrate in the upper dermis. The deeper dermis
and subcutaneous tissue revealed circumscribed
nodule (Fig. 1) composed of extensive areas of
calcification within cystic spaces (Fig. 2)
surrounded by nests of ghost cells (Figs. 2, 3)
foamy macrophages, few plasma cells, lymphocytes
and multinucleated foreign body type of giant cells
(Figs. 1, 3). Keratin flakes were present. Areas of
fibrosis and hyalinization were also seen. There was
no evidence of granuloma formation or malignant
change. These features were consistent with a
diagnosis of pilomatricoma.
DISCUSSION
Calcified lesions in the skin have been noted
since ancient times. Galen, before 200 A.D,
described stones in some tumors, as did Ambrosie
Pare in 1585. A more precise description was given
by Wilcken's in 1856. However, the first complete
work, based on a series of patients, was published
by Malherbe and Chenantais in 1880. They
described calcifying epitheliomas, initially thought
to be tumors of sebaceous glands. Malherbe himself
in 1905 corrected this view. The term
pilomatrixoma, to denote origin from hair matrix
cells, was suggested by Forbis and Helwig in 1961.
This was later corrected to pilomatricoma. These
tumors have a wide variety of signs, which often
cause misdiagnosis. These are rare but still the
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Mahboob et al.
Fig. l
Fig. 2
Circumscribed nodules in dermis. Chronic
inflammatory infiltrate and multinucleated foreign
body type of giant cells.
Areas of calcification within cystic
surrounded by nests of ghost cells.
spaces,
most common benign tumors of hair matrix2 . These
accounted for one in 500 histologic specimen taken
from 209 cases collected during 20 year period3 .
Pilomatricoma occurs at any age from infancy
and is frequently seen in children. The majority of
the patients are under 20 years of age and females
Fig. 3
Chronic inflammatory infiltrate, multinucleated
foreign body type of giant cells and ghost cells.
are affected more often than males4 , 5 . It is not
hereditary but a number of familial cases are
recorded2 . It usually presents as a solitary lesion but
on occasions multiple tumors are evident as part of
an autosomal dominant inherited disorder6. Rarely
it may represent a dermatological marker of
systemic disease e.g. dystrophia myotonica7 or
Gardener's syndrome8 . The tumor presents as a
slowly growing, firm to hard nodule on head, upper
limbs, neck, trunk or lower limbs4. The skin over
the tumor is normal and tumor has a lobular shape
on palpation2 . In adults there may be quite short
history and there is usually no evidence of a
preceding cyst9 . It may be subjected to periodic
inflammation and on occasions presents as a
granulomatous ·swelling. Calcification is seen in
80% of lesions10 , t 1. Chalky deposits are sometimes
evident clinically or may be revealed by radiology.
Malignant change is extremely rare. It arises chiefly
in large lesions that have been present for many
years 12 . It occurs more often in middle aged men in
the regions of head and neck, rarely on the eye
lid !3. Pilometric carcinoma can metastasize to the
lungs, bone and viscera with a subsequent poor
outcome 14-16.
The anagen, catagen and telogen cycles of
normal hair growth are regulated by programmed
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Pilomatricoma
cell death (apoptosis). bcl-2 is a proto- oncogene
that helps to suppress apoptosis in both benign and
malignant tumors. Both apoptosis and bcl-2 are
critical factors in normal hair follicle development.
In one series of 10 histologically proven cases of
pilomatricomas, bcl-2 expression was studied by
immunohistochemical methods 17. All of the cases
were strongly decorated by bcl-2 immunostaining
and faulty suppression of apoptosis was attributed to
the pathogenesis of pilomatricomas.
As regard its pathological aspect, it is situated
in the dermis and is composed of well­
circumscribed, rounded islands giving a lobulated
contour. The outer cells are small and their rounded
nuclei crowded together make this region deep
basophilic. Mitotic figures can usually be seen. The
cytoplasm is scanty and the cell margins indistinct.
Towards the center of the mass, the cytoplasm
becomes more abundant and eosinophilic. The
nuclear outline persists, but the chromatin is sparse
and clumped in dark granules, then, all the
basophilic material disappears, leaving a "Ghost
cell". Melanin may be present. The stroma that
encapsulates the masses usually contains
inflammatory and foreign body cells and
occasionally may ossify. Rarely malignant change
can occur12 , 16 .
In one
study
after
histopathological
examination of 118 lesions of 116 patients with
pilomatricoma, four distinct chronological stages
were categorized: early, fully developed, early
regressive and late regressive 18 . Early lesions were
small cystic structures lined by squamoid and
basaloid epithelium containing keratin filaments and
faulty hair matrix material composed of shadow
cells. Fully developed lesions were large neoplasms
lined by basaloid epithelium at their periphery and
within, composed of regularly shaped, densely
packed zones of comified masse5 containing shadow
cells. Early regressive lesions had no apparent
epithelial lining but did have basaloid cell foci at the
;>eriphery; within, they were composed of pink hair
:natrix material with shadow cells surrounded by
granulation tissue with inflammatory infiltrates and
ultinucleated histiocytic giant cells. Late
-egressive lesions had no epithelial component and
ere composed of irregularly shaped, partially
- nfluent masses of faulty hair material and
.:..tlcified or ossified shadow cells embedded in a
...csmoplastic stroma with little or no inflammatory
filtrate. Thus the lesion begins as an infundibular
matrix cyst and ends up as a calcified and ossified
nodule with no visible epithelial component.
In one study two patients with subcutaneous
nodules (later histologically proved to be
pilomatricoma) on forearms underwent imaging
tests 19. Plain films showed non-specific well
circumscribed lesions. Ultrasonography revealed
nodular, well circumscribed hyperechoic lesion in
one patient. In both cases spin-echo (SE) Ti­
weighted images (TiWl) showed homogeneous,
intermediate signal intensity (SI). On gadolinium­
enhanced TiWI (1 patient), no enhancement was
observed. Both lesions showed predominant low to
intermediate SI on T2WI.
Treatment
Local excision is required for benign lesions.
Wider excision will be needed if malignancy is
suspected. Role of radiotherapy and chemotherapy
in the treatment of disseminated disease is
unclea1'5, 16 . Our case highlights the fact that any
cutaneous lesion with similar clinical features
should be suspected as pilomatricoma and diagnosed
histopathologically after complete removal to avoid
subsequent serious consequences.
REFERENCES
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Mackie RM. Tumors of skin appendages. In: Textbook
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Julian CG, Bowers PW. A clinical review of 209
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Roth SI, et al. Cutaneous ossification. Report of 120
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15. O' Donovan DG, Freemont AJ, Adams JE et al.
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carcinoma with multiple visceral metastases. Cancer
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17. Farrier S, Morgan M. bcl-2 expression in pilomatricoma.
Am J Dermatopathol 1997; 19: 254-7.
18. Kaddu S, Soyer HP, Hod!, S et al. Morphological stages
of pilomatricoma. Am J Dermatopathol 1996; 18: 333-8.
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The Authors:
Atiya Mahboob,
Assistant Professor
Department of Dermatology
Division of Medicine,
Shaikh Zayed Medical Complex,
Lahore.
Farrukh Iqbal,
Associate Professor
Department of Medicine,
Shaikh Zayed Medical Complex,
Lahore.
Zafar Iqbal
Professor and Head of
Department of Medicine
Shaikh Zayed Medical Complex.
Lahore.
Sabia Riaz
Department of Histopathology
Shaikh Zayed Medical Complex,
Lahore.
Address for Correspondence:
Atiya Mahboob,
Assistant Professor
Department of Dermatology
Division of Medicine,
Shaikh Zayed Medical Complex,
Lahore.
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