Transcript Andrew’s Diseases of the Skin Epidermal Nevi, Neoplasms

Andrew’s Diseases of the Skin
Epidermal Nevi, Neoplasms, and
Cysts Part 3
Chapter 29
Syringoma
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Small translucent
papule
Commonly on eyelids
or upper cheeks
Axilla, abdomen,
forehead, penis, vulva
Develop slowly and
persist indefinitely
Asymptomatic
18% of adults with
Down’s syndrome
syringoma
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Dilated cystic sweat
ducts
Treatment
 Electrodessication
 Laser ablation
 cryotherapy
Variants of Syringoma
Clear cell syringoma
 Associated with diabetes mellitus
 Identical lesions, histological difference
 Other clinical variants
 Limited to the scalp causing alopecia
 Unilateral linear or nevoid distribution
 Limited to vulva and penis
 Limited to distal extremities
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Eruptive syringoma
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Numerous lesions on
the neck, chest, axilla,
upper arms and
periumbilically
Young persons
Histologically
identical
Reported in Down’s
syndrome
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Clinically may be
confused with
reticulated
papillomatosis of
Gougerot-Carteaud
Eccrine hidrocystomas
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Translucent papules
1-3mm
May have bluish tint
Usually solitary,
however, multiple
lesion may be seen
Occur on the face
May become more
prominent in hot
weather
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Treatment – excision
Topical atropine or
scopolamine
Eccrine poroma
Benign, slow-growing, slightly protruding,
sessile, soft, reddish tumor
 Most commonly occur on the sole or the
side of the foot. May occur anywhere
 Bleeds with slight trauma
 Frequent cup-shaped shallow depression
from which the tumor grows
 Benign – simple excision
 Eccrine poromatosis
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Eccrine poroma
Eccrine poroma, scan power view. Note numerous epidermal
connections by the tumor and the degree of acanthosis.
Low power view of above showing the vascular stroma and
relatively uniform cell population.
High power view of the small kertinocyte population
having distinct cytoplasmic borders.
Malignant eccrine poroma
(porocarcinoma)
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Most arise from
longstanding eccrine
poromas (50%)
Clinically similar
May also manifest as a
blue or black nodule,
plaque or ulcerated
tumor
M=F, avg 70 yrs
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Legs 30%, feet 20%,
face 12%, thighs 8%
If metastatic, 70%
mortality
Mohs MS TOC
There are sharply
demarcated nests
of tumor within
the epidermis.
There is sparing
of a rim of normal
epidermal
keratinocytes in
most areas
There is much more atypia than would be expected in a clonal
seborrheic keratosis or in hidroacanthoma simplex. There are no
transitional atypical cells blending with the peripheral normal
keratinocytes. An abnormal mitosis is seen in this picture.
Chondroid Syringoma and
Malignant Chondroid Syringoma
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Firm intradermal or
subcutaneous nodule
Most commonly
located on the nose or
cheeks
80 % involving the
head and neck
Symptomatic 5-30mm
Felt to be of eccrine
origin
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Malignant mixed tumor
of the skin
Most occur on
extremities. Reported on
face, scalp, back, buttocks
Grow rapidly. Metastasis
more the 50%
Aggressive surgical
excision, Adjuvant
radiation therapy w/wo
chemotherapy
Scanning power view of a shaply demarcated subcutaneous
tumor. There are tubular foci, a cystic area, and a solid
component.
A high power view of the epithelium lining the cystic area.
A medium power view
showing tubular
differentiation.
A high power view showing cells of the type shown
above mixed with other cells lining a tubular structure as
well as being present in solid clusters peripheral to the
tubule..
Clear cell hidradenoma
(nodular hidradenoma)
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Classified as an
eccrine sweat gland
tumor
Single nodular, solid
or cystic, occasionally
protruding mass
Flesh colored or
reddish
Anywhere. Most
common site is the
head
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20% c/o pain on pressure
Multiple lesions reported
Women 2X men
Extirpation is TOC
Scan power view. Note the epidermal connection/s.
A high power view. Cuboidal and low columar cells form lumina in
this example. There are two delicate blood vessels in this picture.
Clear cell differentiation is seen, and the cells are larger than those
found in the other acrospiromas.
Another high power view. The luminal cells have some apocrine
features. A few delicate blood vessels are in this picture. Some of
the epithelial cells have clear cytoplasm and some do not.
Arrows point to some of the mucus producing cells. A characteristic
unit composed of a vessel surrounded by cells having stainable
cytoplasm which are surrounded by clear cells is seen near the top of
the image.
Malignant clear cell hidradenoma
(hidradenocarcinoma)
Extremely rare
 Presents as a solitary nodule
 Lower extremity 32.9 %, upper extremity
27.6 %, trunk 11.9 %, head 26.3 %
 Metastasis occurs 60%
 Tx wide local excision, radiation and
chemotherapy
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Eccrine spiradenoma
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Solitary, 1cm, deepseated nodule
Most frequently seen
on the ventral surface
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Especially upper half
of the body
Skin-colored, blue or
pink with normal
overlying skin
Multiple lesions, linear
pattern may be seen
Paroxysmal pain
Eccrine spiradenoma
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Benign clinical course
Simple excision
DDX may include
A - angiolipoma
N - neuroma
G - glomus tumor
E
L – leiomyoma
Spiradenoma composed of light and dark cells having
no visible cytoplasmic junctions.
Malignant eccrine spiradenoma
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In long standing
lesions malignant
degeneration may
occur and my be
lethal. Malignant
Eccrine Spiradenoma
Papillary eccrine adenoma
Uncommon benign lesion
 Dermal nodules
 Extremities of black patients
 Tendency to recur
 Complete surgical excision
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Note the papillary intraluminal projections of epithelial cells.
Such projections, and the fact that long tubules are present, help
to differentiate this from a syringoma..
syringoacanthoma
Extremely rare (21 cases)
 Seborrheic keratosis-like neoplasm
 Significant tissue destruction if left
untreated
 Classification remains controversial
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Eccrine syringofibroadenoma
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Most presentations are
a solitary,
hyperkeratotic nodule
or plaque involving
the extremities
Characteristic marker
of Schopf syndrome
 Hydrocystomas of
the eyelids,
hypotrichosis,
hypodontia, and
nail abnormalities
cylindroma
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Dermal eccrine
cylindroma, Spiegler’s
tumor, turban tumor,
and tomato tumor
benign
Predominately on
scalp and face
Solitary, firm but
rubber-like nodule
Pinkish to blue
Few mm to several cm
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Women chiefly
affected
Grow slowly
Rarely undergo
malignant
degeneration
May be mistaken for
epidermoid cyst
excision
cylindroma
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Dominantly inherited
form
Numerous rounded
masses of various
sizes on the scalp
Appears soon after
puberty
Resembles bunches of
grapes or small
tomatoes
Sweat gland carcinoma
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Eccrine carcinoma
 No characteristic
clinical appearance
 High incidence of
metastatic spread
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Mucinous eccrine carcinoma
 Commonly a round,
elevated, reddish, and
sometimes ulcerated mass
 Usually head and neck
(75%)
 Slow growth and
asymptomatic
 11% incidence of metastasis
 Local excision
Aggressive digital
papillary adenocarcinoma
Aggressive malignancy involving the digit
between the nail bed and the distal
interphalangeal joint spaces in most cases
 Presents as a solitary nodule
 50% recurrence rate
 Just under 50% develop metastasis
 All patients should have CXR
 Complete excision TOC
 Amputation may be required
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Primary cutaneous
adenoid cystic carcinoma
Rare
 Presents usually on the chest or scalp
 Mohs MS TOC
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Microcystic adnexal carcinoma
(sclerosing sweat duct carcinoma)
Generally a very slow-growing plaque or
nodule
 Occurs most commonly on the upper lip of
women
 Perineural infiltration is common and may
be extensive
 TOC Mohs
 No reports of metastases
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APOCRINE GLANDS
ceruminoma
Rare apoeccrine tumor that rarely becomes
malignant
 Firm nodular mass in the EAC
 Ulceration and crusting may occur
 Obstruction
 Questionable true entity
 Treatment - excision
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Hidradenoma papilliferum
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Benign solitary tumor
Almost exclusively on
the vulva
Bleeding, ulceration,
discharge, itching and
pain
Firm nodule few mm
excision
Syringadenoma papilliferum
(syringocystadenoma papilliferum)
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Most commonly
develops in a nevus
sebaceous of
Jadassohn
Scalp or face
Firm rose red papules
Groups
Vesicle-like inclusions
are seen
May simulate MC
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Transition to
carcinoma is rare
Excision is advised
Syringoma papilliferum
Apocrine hidrocystoma/cystadenoma
(apocrine retention cyst)
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Benign tumor
Occurs chiefly on the
face. solitary
Penile shaft- median
raphe cyst
Dome-shaped,
smooth-surfaced
translucent nodule
Bluish or brownish
Simple excision
A high power view of the linings of two of the cysts. A few
brown, lipofuchsin pigment granules (PG) are in the basilar part
of the epithelium. There is a suggestion of pigmented material in
the apices of other cells. Apocrine snouts are prominent.
Apocrine gland carcinoma
Rare
 Axilla is the most common site
 May be seen in the nipple, vulva and EAC
 May originate from aberrant mammary
glands
 Widespread metastases may occur
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HAIR FOLLICLE NEVI AND
TUMORS
Pilomatricoma
(calcifying epithelioma of Malherbe)
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Usually a single tumor
Most commonly on
the face, neck or arms
Deeply seated firm
nodule, covered with
normal or pink skin
Asymptomatic
Stretching may show
“tent sign”
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Derived from hair
matrix cells
Clinical DDX is
impossible
Simple excision
Familial patterns do
occur
Multiple in
Rubinstein-Taybi and
Gardner syndrome
pilomatricoma
Malignant pilomatricoma
Extremely rare
 Do not behave aggressively
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Trichofolliculoma
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Benign, highly
structured adenoma of
the pilosebaceous unit
Small dome-shaped
nodule on the face or
scalp
A small wisp of fine,
immature hairs
protrude from a
central pore
Simple excisional bx
Trichoepithelioma
(epithelioma adenoides cysticum,
multiple familial trichoepitheliomas)
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Occur as multiple cystic
and solid nodules
typically on the face
Small, rounded, smooth,
shiny,slightly translucent
and firm.
Flesh colored or slightly
reddish
Slightly depressed center
Often grouped and
symmetrical
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benign
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Solitary trichoepithelioma
 Nonhereditary
 Mostly on face
Giant solitary
trichoepithelioma
 May reach several cm
 Mostly on thigh and
perianal
Desmoplastic
trichoepithelioma
 Difficult to
differentiate from
morphea-like BCC
 Solitary or multiple on
the face
trichoblastoma
Benign neoplasms of follicular germinative
cells
 Asymptomatic
 Scalp and face
 Surgical excision
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Trichilemmoma and
Cowden’s disease
(multiple hamartoma syndrome)
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Benign neoplasm of the
hair follicle
Small solitary papule on
the face
Nose and cheeks
Multiple
 Marker for Cowden,s
syndrome
Generally limited to the
head and neck
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87% of patients with
Cowden’s
38% develop
malignancies
 Breast 25-36%
 Thyroid 7%
 Colon
adenocarcinoma
Tumor suppressor gene
Trichilemmal carcinoma
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Sun exposed areas
Face and ears
Slow growing
epidermal papule,
indurated plaque or
nodule with tendency
to ulcerate
Surgical excision
Trichodiscoma
and fibrofolliculoma
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Hundreds of flat or
dome-shaped, skincolored asymptomatic
papules
Face, trunk and
extremities
Autosomal dominant
trait
Controversial entity
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2-4 mm skin-colored
to white papules
Solitary, more
commonly multiple
Scattered over the
face, trunk and
extremities
Proliferating trichilemmal cyst
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Large exophytic
neoplasms
Almost exclusively
confined to scalp and
back of neck
May ulcerate
Ass with nevus
sebaceous
Metastasis may occur
Most respond to surgical
excision
Dermoid cyst
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Congenital in origin
Chiefly along lines of
cleavage
Result from improper
embryologic development
Potential for intracranial
communication
CT or MRI scan is required
to rule this out prior to BX
over cranial cleavage
planes
Freely mobile and not
attached to the skin
Pilonidal cyst
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Midline hairy patch or pit in the sacral region with
a sinus orifice in the bottom, or a cyst beneath it
Usually becomes symptomatic during adolescence
Opening cyst widely, debriding it, and packing it
with silver nitrate crystals
More advanced surgical intervention may be
required
SCC has been reported to arise from chronic
inflammatory pilonidal disease
Pilonidal sinus
Steatocystoma simplex
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Noninheritable
counterpart to the
more familiar
steatocystoma
multiplex
Face limbs or chest
Simple excision
Steatocystoma multiplex
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Multiple, small, yellowish, cystic nodules 2-6 mm
Principally on the upper anterior trunk, upper
arms, axillae and thighs
Lesions may be generalizes
High familial tendency
Contain a syruplike, yellowish, odorless oily
material
Likely autosomal dominant inheritance
Tx- excision of individual lesions
Incision and expression or aspiration
Steatocystoma multiplex
Eruptive vellus hair cysts
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Autosomal dominant
inheritance
Yellowish to reddish
brown, small papules of
the chest and proximal
extremities
Disseminated lesions
reported
Pigmented follicular cysts
Face or neck
 Suggested to be a variant of multiple
pilosebaceous cysts
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milia
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White keratinous cysts, 1-4 mm
Chiefly on the face esp under eyes
May occur in great numbers
Occur in up to 50 % of newborns
Primarily develop without a predisposing
condition
Can develop in inflammatory conditions and skin
diseases such as epidermolysis bullosa,
pemphigus, bullous pemphigoid, PCT, herpes
zoster, contact dermatitis, and after prolonged use
of NSAIDS
milia
Variants include MEM (multiple eruptive
milia)
 MEP (milia en plaque)
 Tx- incision and expression
 Tretinoin and minocycline for MEP
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milia
Pseudocyst of the auricle
Fluctuant, tense, noninflammatory swelling
of the upper ear
 Believed to be ass with trauma
 Tx – drainage
 ILI steroid
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Cutaneous columnar cysts
Four types of cyst that occur in the skin are
lined by columnar epithelium
 Branchiogenic cyst
 Small solitary lesions just above the
sternal notch
 Thyroglossal duct cysts
 Anterior aspect of the neck
 Malignancies reported 1%
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Cutaneous columnar cysts
Cutaneous ciliated cysts
 Usually located on the legs of females
 Perineum vulva and foot regions
 Median raphe cyst
 Developmental defects lying in the
ventral midline of the penis, usually on
the glans
 Surgical intervention is standard therapy
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The End