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Andrew’s Diseases of the Skin Epidermal Nevi, Neoplasms, and Cysts Part 3 Chapter 29

Andrew’s Diseases of the Skin Epidermal Nevi, Neoplasms, and Cysts Part 3 Chapter 29. 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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Andrew’s Diseases of the Skin Epidermal Nevi, Neoplasms, and Cysts Part 3 Chapter 29

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  1. Andrew’s Diseases of the SkinEpidermal Nevi, Neoplasms, and Cysts Part 3 Chapter 29

  2. 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

  3. Dilated cystic sweat ducts Treatment Electrodessication Laser ablation cryotherapy syringoma

  4. 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

  5. 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 Eruptive syringoma

  6. 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 hidrocystomas

  7. 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

  8. Eccrine poroma

  9. 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 Malignant eccrine poroma(porocarcinoma)

  10. 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 Chondroid Syringoma and Malignant Chondroid Syringoma

  11. 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 Clear cell hidradenoma(nodular hidradenoma)

  12. 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

  13. 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 Eccrine spiradenoma

  14. Benign clinical course Simple excision DDX may include A - angiolipoma N - neuroma G - glomus tumor E L – leiomyoma Eccrine spiradenoma

  15. In long standing lesions malignant degeneration may occur and my be lethal. Malignant Eccrine Spiradenoma Malignant eccrine spiradenoma

  16. Papillary eccrine adenoma • Uncommon benign lesion • Dermal nodules • Extremities of black patients • Tendency to recur • Complete surgical excision

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

  18. 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 Eccrine syringofibroadenoma

  19. 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 cylindroma

  20. Dominantly inherited form Numerous rounded masses of various sizes on the scalp Appears soon after puberty Resembles bunches of grapes or small tomatoes cylindroma

  21. Eccrine carcinoma No characteristic clinical appearance High incidence of metastatic spread 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 Sweat gland carcinoma

  22. 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

  23. Primary cutaneousadenoid cystic carcinoma • Rare • Presents usually on the chest or scalp • Mohs MS TOC

  24. 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

  25. APOCRINE GLANDS

  26. 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

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

  28. 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 Syringadenoma papilliferum(syringocystadenoma papilliferum)

  29. Syringoma papilliferum

  30. 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 hidrocystoma/cystadenoma(apocrine retention cyst)

  31. 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

  32. HAIR FOLLICLE NEVI AND TUMORS

  33. 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(calcifying epithelioma of Malherbe)

  34. pilomatricoma

  35. Malignant pilomatricoma • Extremely rare • Do not behave aggressively

  36. 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 Trichofolliculoma

  37. 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 Trichoepithelioma(epithelioma adenoides cysticum, multiple familial trichoepitheliomas)

  38. 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

  39. trichoblastoma • Benign neoplasms of follicular germinative cells • Asymptomatic • Scalp and face • Surgical excision

  40. 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 87% of patients with Cowden’s 38% develop malignancies Breast 25-36% Thyroid 7% Colon adenocarcinoma Tumor suppressor gene Trichilemmoma andCowden’s disease (multiple hamartoma syndrome)

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

  42. 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 Trichodiscoma and fibrofolliculoma

  43. 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 Proliferating trichilemmal cyst

  44. 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 Dermoid cyst

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