Epidermal Nevi, Neoplasms, and Cysts

Epidermal Nevi, Neoplasms, and Cysts

Syringoma
* 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
* Dilated cystic sweat ducts
* Treatment
o Electrodessication
o Laser ablation
o cryotherapy

Variants of Syringoma
* Clear cell syringoma
o Associated with diabetes mellitus
o Identical lesions, histological difference
* Other clinical variants
o Limited to the scalp causing alopecia
o Unilateral linear or nevoid distribution
o Limited to vulva and penis
o Limited to distal extremities

Eruptive syringoma
* Numerous lesions on the neck, chest, axilla, upper arms and periumbilically
* Young persons
* Histologically identical
* Reported in Down’s syndrome
* Clinically may be confused with reticulated papillomatosis of Gougerot-Carteaud

Eccrine hidrocystomas
* 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
* 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

Malignant eccrine poroma (porocarcinoma)
* 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
* Legs 30%, feet 20%, face 12%, thighs 8%
* If metastatic, 70% mortality
* Mohs MS TOC

Chondroid Syringoma and Malignant Chondroid Syringoma
* 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
* 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

Clear cell hidradenoma (nodular hidradenoma)
* 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
* 20% c/o pain on pressure
* Multiple lesions reported
* Women 2X men
* Extirpation is TOC

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

Eccrine spiradenoma
* Solitary, 1cm, deep-seated nodule
* Most frequently seen on the ventral surface
* Especially upper half of the body
* Skin-colored, blue or pink with normal overlying skin
* Multiple lesions, linear pattern may be seen
* Paroxysmal pain
* Benign clinical course
* Simple excision
* DDX may include
* A - angiolipoma
* N - neuroma
* G - glomus tumor
* E
* L – leiomyoma

Malignant eccrine spiradenoma
* 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

syringoacanthoma
* Extremely rare (21 cases)
* Seborrheic keratosis-like neoplasm
* Significant tissue destruction if left untreated
* Classification remains controversial

Eccrine syringofibroadenoma
* Most presentations are a solitary, hyperkeratotic nodule or plaque involving the extremities
* Characteristic marker of Schopf syndrome
o Hydrocystomas of the eyelids, hypotrichosis, hypodontia, and nail abnormalities

cylindroma
* 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
* Women chiefly affected
* Grow slowly
* Rarely undergo malignant degeneration
* May be mistaken for epidermoid cyst
* excision
* 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
* Eccrine carcinoma
o No characteristic clinical appearance
o High incidence of metastatic spread
* Mucinous eccrine carcinoma
o Commonly a round, elevated, reddish, and sometimes ulcerated mass
o Usually head and neck (75%)
o Slow growth and asymptomatic
o 11% incidence of metastasis
o 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

Primary cutaneous adenoid cystic carcinoma
* Rare
* Presents usually on the chest or scalp
* Mohs MS TOC

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

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

Hidradenoma papilliferum
* Benign solitary tumor
* Almost exclusively on the vulva
* Bleeding, ulceration, discharge, itching and pain
* Firm nodule few mm
* excision

Syringadenoma papilliferum (syringocystadenoma papilliferum)
* 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
* Transition to carcinoma is rare
* Excision is advised

Apocrine hidrocystoma/cystadenoma (apocrine retention cyst)
* Benign tumor
* Occurs chiefly on the face. solitary
* Penile shaft- median raphe cyst
* Dome-shaped, smooth-surfaced translucent nodule
* Bluish or brownish
* Simple excision

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

HAIR FOLLICLE NEVI AND TUMORS
Pilomatricoma (calcifying epithelioma of Malherbe)
* 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”
* 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

Trichofolliculoma
* 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)
* 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
* benign
* Solitary trichoepithelioma
o Nonhereditary
o Mostly on face
* Giant solitary trichoepithelioma
o May reach several cm
o Mostly on thigh and perianal
* Desmoplastic trichoepithelioma
o Difficult to differentiate from morphea-like BCC
o Solitary or multiple on the face

trichoblastoma
* Benign neoplasms of follicular germinative cells
* Asymptomatic
* Scalp and face
* Surgical excision

Trichilemmoma and Cowden’s disease (multiple hamartoma syndrome)
* Benign neoplasm of the hair follicle
* Small solitary papule on the face
* Nose and cheeks
* Multiple
o Marker for Cowden,s syndrome
* Generally limited to the head and neck
* 87% of patients with Cowden’s
* 38% develop malignancies
o Breast 25-36%
o Thyroid 7%
o Colon adenocarcinoma
* Tumor suppressor gene

Trichilemmal carcinoma
* Sun exposed areas
* Face and ears
* Slow growing epidermal papule, indurated plaque or nodule with tendency to ulcerate
* Surgical excision

Trichodiscoma and fibrofolliculoma
* Hundreds of flat or dome-shaped, skin-colored asymptomatic papules
* Face, trunk and extremities
* Autosomal dominant trait
* Controversial entity
* 2-4 mm skin-colored to white papules
* Solitary, more commonly multiple
* Scattered over the face, trunk and extremities

Proliferating trichilemmal cyst
* 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
* 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
* 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
* Noninheritable counterpart to the more familiar steatocystoma multiplex
* Face limbs or chest
* Simple excision

Steatocystoma multiplex
* 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

Eruptive vellus hair cysts
* 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

milia
* 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
* Variants include MEM (multiple eruptive milia)
* MEP (milia en plaque)
* Tx- incision and expression
* Tretinoin and minocycline for MEP

Pseudocyst of the auricle
* Fluctuant, tense, noninflammatory swelling of the upper ear
* Believed to be ass with trauma
* Tx – drainage
* ILI steroid

Cutaneous columnar cysts
* Four types of cyst that occur in the skin are lined by columnar epithelium
* Branchiogenic cyst
o Small solitary lesions just above the sternal notch
* Thyroglossal duct cysts
o Anterior aspect of the neck
o Malignancies reported 1%
* Cutaneous ciliated cysts
o Usually located on the legs of females
o Perineum vulva and foot regions
* Median raphe cyst
o Developmental defects lying in the ventral midline of the penis, usually on the glans
o Surgical intervention is standard therapy


Epidermal Nevi, Neoplasms, and Cysts.ppt