Page 32 - The Netter Collection of Medical Illustrations - Integumentary System_ Volume 4 ( PDFDrive )

Plate 2-5 Integumentary System

Cylindromas

Variable-sized nodules commonly found
on the scalp. Can be an isolated finding
or found in association with Brooke-
Spiegler syndrome

ECCRINE SPIRADENOMA

Eccrine spiradenomas are uncommon benign tumors of
the skin. Most often they are solitary, but they can occur
in conjunction with cylindromas in the Brooke-Spiegler
syndrome. They can occur in any location on the
human body but are most commonly found on the head
and neck. The next most common region is the ventral
trunk. These tumors are uncommon on the extremities. Brooke-Spiegler syndrome.
Spiradenomas tend to appear between the ages of 15 Multiple eccrine spiradenomas
and 40 years, although they have been reported to occur and cylindromas. Inherited in
an autosomal dominant manner
at any age. Malignant degeneration is extremely rare,
but if it does occur, it is often fatal.
Clinical Findings: A spiradenoma usually manifests
as a solitary dermal nodule or papule ranging from 5 to
20 mm in diameter. The average size is approximately
10 mm. They are typically seated deeply in the dermis
and can be very painful to light touch. The tumors grow
very slowly, and except for the pain can go unnoticed
for some time. The pain tends to have a waxing and
waning course, and it is more often than not the reason
the patient seeks medical advice. The overlying epider-
mis is almost always normal. The dermal nodule some-
times takes on a purple or bluish coloration. Although
they are most commonly solitary, multiple spiradeno-
mas may be seen in association with multiple cylindro-
mas in Brooke-Spiegler syndrome.
Brooke-Spiegler syndrome is an autosomal dominant
inherited skin condition caused by a genetic defect in
the CYLD gene. This syndrome is characterized by
multiple cylindromas, spiradenomas, and trichoepithe-
liomas. The tumors usually begin in the third decade
of life and increase in number and size throughout the
patient’s life. The CYLD gene encodes a tumor suppres-
sor protein and is an important downregulator of the
nuclear factor NF-κB pathway. The clinical phenotype
varies depending on the type of mutation in this gene.
Patients with familial cylindromatosis also have defects
in this gene. The gene has been localized to the long
arm of chromosome 16.
The eccrine spiradenoma is considered to be one of
the group of unique tumors that can cause painful
dermal nodules. This group also includes angiolipomas, Low power. Well-circumscribed basophilic dermal High power. Two cell populations are present,
neuromas, glomus tumors, and leiomyomas. This group nodule within the dermis a larger pale cell type and a peripheral smaller
of tumors makes up the differential diagnosis when basophilic cell type.
evaluating these painful nodules. If the nodule is asymp-
tomatic, lipoma and other adnexal tumors would also
be considered in the differential diagnosis.
The exact cell type from which the spiradenomas
are derived is still undetermined. They were originally changes, and the multilobulated tumors do not connect Treatment: Surgical excision is curative. Surgical
believed to arise from eccrine tissue, but increasing with the epidermis. This gives rise to the term “blue removal with carbon dioxide laser ablation has also been
evidence is pointing to a derivation from apocrine balls in the dermis.” The tumor is composed of two found to be highly successful. Because of the number
tissue. unique cell types. Large, pale cells predominate, with and size of the tumors in patients with the Brooke-
Histology: The histological hallmark of an eccrine surrounding aggregates of smaller basophilic cells that Spiegler syndrome, a multidisciplinary approach is
spiradenoma is the appearance of large nests of baso- contain hyperchromatic nuclei. The tumor is well cir- often taken. Plastic surgeons are often the primary
philic cells in the dermis. There are no epidermal cumscribed and is surrounded by a fibrous capsule. physicians removing these tumors.

18 THE NETTER COLLECTION OF MEDICAL ILLUSTRATIONS