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

2. Syringoma Small translucent papule Small translucent papule Commonly on eyelids or upper cheeks Commonly on eyelids or upper cheeks Axilla, abdomen, forehead, penis, vulva Axilla, abdomen, forehead, penis, vulva Develop slowly and persist indefinitely Develop slowly and persist indefinitely Asymptomatic Asymptomatic 18% of adults with Down’s syndrome 18% of adults with Down’s syndrome

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

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

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

9. Eccrine hidrocystomas Translucent papules 1-3mm Translucent papules 1-3mm May have bluish tint May have bluish tint Usually solitary, however, multiple lesion may be seen Usually solitary, however, multiple lesion may be seen Occur on the face Occur on the face May become more prominent in hot weather May become more prominent in hot weather Treatment – excision Topical atropine or scopolamine

11. Eccrine poroma Benign, slow-growing, slightly protruding, sessile, soft, reddish tumor Benign, slow-growing, slightly protruding, sessile, soft, reddish tumor Most commonly occur on the sole or the side of the foot. May occur anywhere Most commonly occur on the sole or the side of the foot. May occur anywhere Bleeds with slight trauma Bleeds with slight trauma Frequent cup-shaped shallow depression from which the tumor grows Frequent cup-shaped shallow depression from which the tumor grows Benign – simple excision Benign – simple excision Eccrine poromatosis Eccrine poromatosis

12. Eccrine poroma

14. Eccrine poroma, scan power view. Note numerous epidermal connections by the tumor and the degree of acanthosis.

15. Low power view of above showing the vascular stroma and relatively uniform cell population.

16. High power view of the small kertinocyte population having distinct cytoplasmic borders.

17. Malignant eccrine poroma (porocarcinoma) Most arise from longstanding eccrine poromas (50%) Most arise from longstanding eccrine poromas (50%) Clinically similar Clinically similar May also manifest as a blue or black nodule, plaque or ulcerated tumor May also manifest as a blue or black nodule, plaque or ulcerated tumor M=F, avg 70 yrs M=F, avg 70 yrs Legs 30%, feet 20%, face 12%, thighs 8% If metastatic, 70% mortality Mohs MS TOC

19. There are sharply demarcated nests of tumor within the epidermis. There is sparing of a rim of normal epidermal keratinocytes in most areas

20. 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.

21. Chondroid Syringoma and Malignant Chondroid Syringoma Firm intradermal or subcutaneous nodule Firm intradermal or subcutaneous nodule Most commonly located on the nose or cheeks Most commonly located on the nose or cheeks 80 % involving the head and neck 80 % involving the head and neck Symptomatic 5-30mm Symptomatic 5-30mm Felt to be of eccrine origin 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

23. Scanning power view of a shaply demarcated subcutaneous tumor. There are tubular foci, a cystic area, and a solid component.

24. A high power view of the epithelium lining the cystic area.

25. A medium power view showing tubular differentiation.

26. 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..

27. Clear cell hidradenoma (nodular hidradenoma) Classified as an eccrine sweat gland tumor Classified as an eccrine sweat gland tumor Single nodular, solid or cystic, occasionally protruding mass Single nodular, solid or cystic, occasionally protruding mass Flesh colored or reddish Flesh colored or reddish Anywhere. Most common site is the head Anywhere. Most common site is the head 20% c/o pain on pressure Multiple lesions reported Women 2X men Extirpation is TOC

29. Scan power view. Note the epidermal connection/s.

30. 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.

31. 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.

32. 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.

33. Malignant clear cell hidradenoma (hidradenocarcinoma) Extremely rare Extremely rare Presents as a solitary nodule Presents as a solitary nodule Lower extremity 32.9 %, upper extremity 27.6 %, trunk 11.9 %, head 26.3 % Lower extremity 32.9 %, upper extremity 27.6 %, trunk 11.9 %, head 26.3 % Metastasis occurs 60% Metastasis occurs 60% Tx wide local excision, radiation and chemotherapy Tx wide local excision, radiation and chemotherapy

34. Eccrine spiradenoma Solitary, 1cm, deep- seated nodule Solitary, 1cm, deep- seated nodule Most frequently seen on the ventral surface 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

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

36. Spiradenoma composed of light and dark cells having no visible cytoplasmic junctions.

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

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

40. 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..

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

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

45. cylindroma Dermal eccrine cylindroma, Spiegler’s tumor, turban tumor, and tomato tumor Dermal eccrine cylindroma, Spiegler’s tumor, turban tumor, and tomato tumor benign benign Predominately on scalp and face Predominately on scalp and face Solitary, firm but rubber-like nodule Solitary, firm but rubber-like nodule Pinkish to blue Pinkish to blue Few mm to several cm Few mm to several cm Women chiefly affected Grow slowly Rarely undergo malignant degeneration May be mistaken for epidermoid cyst excision

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

49. Sweat gland carcinoma Eccrine carcinoma 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

50. Aggressive digital papillary adenocarcinoma Aggressive malignancy involving the digit between the nail bed and the distal interphalangeal joint spaces in most cases Aggressive malignancy involving the digit between the nail bed and the distal interphalangeal joint spaces in most cases Presents as a solitary nodule Presents as a solitary nodule 50% recurrence rate 50% recurrence rate Just under 50% develop metastasis Just under 50% develop metastasis All patients should have CXR All patients should have CXR Complete excision TOC Complete excision TOC Amputation may be required Amputation may be required

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

53. Microcystic adnexal carcinoma (sclerosing sweat duct carcinoma) Generally a very slow-growing plaque or nodule Generally a very slow-growing plaque or nodule Occurs most commonly on the upper lip of women Occurs most commonly on the upper lip of women Perineural infiltration is common and may be extensive Perineural infiltration is common and may be extensive TOC Mohs TOC Mohs No reports of metastases No reports of metastases

56. APOCRINE GLANDS

57. ceruminoma Rare apoeccrine tumor that rarely becomes malignant Rare apoeccrine tumor that rarely becomes malignant Firm nodular mass in the EAC Firm nodular mass in the EAC Ulceration and crusting may occur Ulceration and crusting may occur Obstruction Obstruction Questionable true entity Questionable true entity Treatment - excision Treatment - excision

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

61. Syringadenoma papilliferum (syringocystadenoma papilliferum) Most commonly develops in a nevus sebaceous of Jadassohn Most commonly develops in a nevus sebaceous of Jadassohn Scalp or face Scalp or face Firm rose red papules Firm rose red papules Groups Groups Vesicle-like inclusions are seen Vesicle-like inclusions are seen May simulate MC May simulate MC Transition to carcinoma is rare Excision is advised

62. Syringoma papilliferum

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

66. 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.

67. Apocrine gland carcinoma Rare Rare Axilla is the most common site Axilla is the most common site May be seen in the nipple, vulva and EAC May be seen in the nipple, vulva and EAC May originate from aberrant mammary glands May originate from aberrant mammary glands Widespread metastases may occur Widespread metastases may occur

68. HAIR FOLLICLE NEVI AND TUMORS

69. Pilomatricoma (calcifying epithelioma of Malherbe) Usually a single tumor Usually a single tumor Most commonly on the face, neck or arms Most commonly on the face, neck or arms Deeply seated firm nodule, covered with normal or pink skin Deeply seated firm nodule, covered with normal or pink skin Asymptomatic Asymptomatic Stretching may show “tent sign” 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

70. pilomatricoma

75. Malignant pilomatricoma Extremely rare Extremely rare Do not behave aggressively Do not behave aggressively

76. Trichofolliculoma Benign, highly structured adenoma of the pilosebaceous unit Benign, highly structured adenoma of the pilosebaceous unit Small dome-shaped nodule on the face or scalp Small dome-shaped nodule on the face or scalp A small wisp of fine, immature hairs protrude from a central pore A small wisp of fine, immature hairs protrude from a central pore Simple excisional bx Simple excisional bx

81. Trichoepithelioma (epithelioma adenoides cysticum, multiple familial trichoepitheliomas) Occur as multiple cystic and solid nodules typically on the face Occur as multiple cystic and solid nodules typically on the face Small, rounded, smooth, shiny,slightly translucent and firm. Small, rounded, smooth, shiny,slightly translucent and firm. Flesh colored or slightly reddish Flesh colored or slightly reddish Slightly depressed center Slightly depressed center Often grouped and symmetrical Often grouped and symmetrical benign

82. Solitary trichoepithelioma Solitary trichoepithelioma  Nonhereditary  Mostly on face Giant solitary trichoepithelioma Giant solitary trichoepithelioma  May reach several cm  Mostly on thigh and perianal Desmoplastic trichoepithelioma Desmoplastic trichoepithelioma  Difficult to differentiate from morphea-like BCC  Solitary or multiple on the face

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

93. Trichilemmoma and Cowden’s disease (multiple hamartoma syndrome) Benign neoplasm of the hair follicle Benign neoplasm of the hair follicle Small solitary papule on the face Small solitary papule on the face Nose and cheeks Nose and cheeks Multiple Multiple  Marker for Cowden,s syndrome Generally limited to the head and neck 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

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

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

109. Proliferating trichilemmal cyst Large exophytic neoplasms Large exophytic neoplasms Almost exclusively confined to scalp and back of neck Almost exclusively confined to scalp and back of neck May ulcerate May ulcerate Ass with nevus sebaceous Ass with nevus sebaceous Metastasis may occur Metastasis may occur Most respond to surgical excision Most respond to surgical excision

110. Dermoid cyst Congenital in origin Congenital in origin Chiefly along lines of cleavage Chiefly along lines of cleavage Result from improper embryologic development Result from improper embryologic development Potential for intracranial communication Potential for intracranial communication CT or MRI scan is required to rule this out prior to BX over cranial cleavage planes 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 Freely mobile and not attached to the skin

111. Pilonidal cyst Midline hairy patch or pit in the sacral region with a sinus orifice in the bottom, or a cyst beneath it 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 Usually becomes symptomatic during adolescence Opening cyst widely, debriding it, and packing it with silver nitrate crystals Opening cyst widely, debriding it, and packing it with silver nitrate crystals More advanced surgical intervention may be required More advanced surgical intervention may be required SCC has been reported to arise from chronic inflammatory pilonidal disease SCC has been reported to arise from chronic inflammatory pilonidal disease

112. Pilonidal sinus

113. Steatocystoma simplex Noninheritable counterpart to the more familiar steatocystoma multiplex Noninheritable counterpart to the more familiar steatocystoma multiplex Face limbs or chest Face limbs or chest Simple excision Simple excision

114. Steatocystoma multiplex Multiple, small, yellowish, cystic nodules 2-6 mm Multiple, small, yellowish, cystic nodules 2-6 mm Principally on the upper anterior trunk, upper arms, axillae and thighs Principally on the upper anterior trunk, upper arms, axillae and thighs Lesions may be generalizes Lesions may be generalizes High familial tendency High familial tendency Contain a syruplike, yellowish, odorless oily material Contain a syruplike, yellowish, odorless oily material Likely autosomal dominant inheritance Likely autosomal dominant inheritance Tx- excision of individual lesions Tx- excision of individual lesions Incision and expression or aspiration Incision and expression or aspiration

115. Steatocystoma multiplex

116. Eruptive vellus hair cysts Autosomal dominant inheritance Autosomal dominant inheritance Yellowish to reddish brown, small papules of the chest and proximal extremities Yellowish to reddish brown, small papules of the chest and proximal extremities Disseminated lesions reported Disseminated lesions reported

117. Pigmented follicular cysts Face or neck Face or neck Suggested to be a variant of multiple pilosebaceous cysts Suggested to be a variant of multiple pilosebaceous cysts

118. milia White keratinous cysts, 1-4 mm White keratinous cysts, 1-4 mm Chiefly on the face esp under eyes Chiefly on the face esp under eyes May occur in great numbers May occur in great numbers Occur in up to 50 % of newborns Occur in up to 50 % of newborns Primarily develop without a predisposing condition 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 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

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

120. milia

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

122. Cutaneous columnar cysts Four types of cyst that occur in the skin are lined by columnar epithelium Four types of cyst that occur in the skin are lined by columnar epithelium Branchiogenic cyst Branchiogenic cyst  Small solitary lesions just above the sternal notch Thyroglossal duct cysts Thyroglossal duct cysts  Anterior aspect of the neck  Malignancies reported 1%

123. Cutaneous columnar cysts Cutaneous ciliated cysts Cutaneous ciliated cysts  Usually located on the legs of females  Perineum vulva and foot regions Median raphe cyst Median raphe cyst  Developmental defects lying in the ventral midline of the penis, usually on the glans  Surgical intervention is standard therapy

124. The End