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DEVELOPMENT OF HAIR DURING EARLY GESTATION
CLASSIFICATION OF ADNEXAL NEOPLASMS
I. NEOPLASMS AND PROLIFERATIONS OF FOLLICULAR LINEAGE
II. NEOPLASMS AND PROLIFERATIONS WITH SEBACEOUS DIFFERENTIATION
III. NEOPLASMS AND PROLIFERATIONS WITH APOCRINE DIFFERENTIATION
IV. NEOPLASMS AND PROLIFERATIONS WITH ECCRINE DIFFERENTIATION
NEOPLASMS AND PROLIFERATIONS OF FOLLICULAR LINEAGE
NEOPLASMS AND PROLIFERATIONS OF FOLLICULAR LINEAGE
I. Follicular and Folliculosebaceous-apocrine Hamartomas
1. Trichofolliculoma
2. Fibrofolliculoma, perifollicular fibroma and
trichodiscoma
3. Nevus sebaceous
4. Mixed tumor (chondroid syringoma)
II. Neoplasms and Proliferations with Follicular
Germinative Differentiation• Trichoepithelioma/Trichoblastoma
III. Neoplasms and Proliferations with Matrical
Differentiation
1. Pilomatricoma2. Pilomatrical carcinoma
IV. Neoplasms and Proliferations with Follicular Sheath
(Trichilemmal) Differentiation
1. Trichilemmoma2. Trichilemmal carcinoma
V. Neoplasms and Proliferations with Superficial Follicular
(Infundibular and Isthmic) Differentiation
1. Tumor of follicular infundibulum (isthmicoma)2. Trichoadenoma (trichoadenoma of
Nikolowski)3. Proliferating pilar tumor (proliferating
follicular-cystic neoplasm)
TRICHOFOLLICULOMA
TRICHOFOLLICULOMA
It is an uncommon small benign tumor originating from the hair follicle usually in the second decade of life.
Trichofolliculoma does not represent a true neoplasm. Rather, the term indicates a group of follicularhamartomas in which fully formed follicular structuresemerge from a central dilated infundibular space.
It has an excellent prognosis.
TRICHOFOLLICULOMAWISPY VELLUS HAIRS EMERGE FROM A SKIN-COLORED
PAPULE WITH A DILATED CENTRAL PORE
TRICHOFOLLICULOMA
TRICHOFOLLICULOMA
C/P OF TRICHOFOLLICULOMA
A small, solitary flesh-colored or whitish papule that occurs most often on the face around the nose region, scalp or upper trunk.
Sometimes the lesion have a central pore (follicularostium or punctum from which it exudes sebum).
In some cases a characteristic small tuft of white hairmay be growing from the central pore.
Rarely, the clinical presentation will be as a largenodule or cyst.
HISTO. OF TRICHOFOLLICULOMA Central cystic space with infundibular cornification and
central orthokeratin with an opening onto the epidermalsurface.
Cross-sections of vellus hair shafts are identifiable within the cyst.
Relatively well-developed and occasionally oddly formed secondary and tertiary follicles protrude in radial fashion from the central structure.
The follicles usually display isthmic differentiation, bulb,papilla and exhibit inner and outer sheath.
The entire structure, including central cyst and its associated radiating secondary and tertiary follicles, is enveloped by a well circumscribed vascularized fibrous dense stroma.
HISTO. OF TRICHOFOLLICULOMATHERE IS A CENTRAL PATULOUS FOLLICULAR INFUNDIBULUM,
FROM WHICH FULLY FORMED OR NEARLY FULLY FORMED
FOLLICLES RADIATE
HISTO. OF TRICHOFOLLICULOMA
SEBACEOUS TRICHOFOLLICULOMA
IT IS A VARIANT OF
TRICHOFOLLICULOMA
DEMONSTRATING NUMEROUS WELL
DIFFERENTIATED SEBACEOUS LOBULES
EMPTYING INTO THE CENTRAL
PRIMARY FOLLICLE AND MAY BE LARGE
AND CYSTIC.
Rx OF TRICHOFOLLICULOMA
It is wholly benign and no treatment is needed.
If a trichofolliculoma is discovered by biopsy, no further intervention is required.
Occasionally they may be removed for cosmetic reasons or if they occur in functionally sensitive areas.
TREATMENT OPTIONS INCLUDE; 1. Curettage and electrodesiccation.2. Surgical excision.
NEVUS SEBACEOUS
NEVUS SEBACEOUS (OF JADASSOHN)
Originally known as ORGANOID NEVUS because it may include components of the entire skin.
It is an uncommon type of birthmark most often found on the scalp representing a classic nevus or congenital hamartoma.
It is due to postzygotic mutation and genetic mosaicism.
Nevus sebaceous is commonly thought of as a sebaceouslesion but it is a non-neoplastic malformation that includesepidermal, follicular, sebaceous, apocrine and connectivetissue elements.
C/P OF NEVUS SEBACEOUS
In an infant or young child, nevus sebaceous presents as a solitary, yellow-orange hairless patch, often oval or linear in shape with smooth or somewhat velvety surface.
It involves the scalp (the nevus will remain hairless, as the infant’s hair grows around it) or face commonly, the neckoccasionally, and the trunk rarely.
Lesions that are linear are distributed along the lines ofBlaschko, although this may be difficult to appreciate if small.
During childhood, the nevus thickens slightly.
C/P OF NEVUS SEBACEOUS
At adolescence, progressive thickening occurs and the surface becomes verrucous. It represents a fertile field for the development of
secondary adnexal neoplasms, commonly benign but occasionally malignant.
NEVUS SEBACEOUS
NEVUS SEBACEOUS
NEVUS SEBACEOUS
NEVUS SEBACEOUS OF FOREHEAD
NEVUS SEBACEOUS OF THE CHEEK
LINEAR NEVUS SEBACEOUS
NEVUS SEBACEOUS ON THE SCALP (A) AND CHIN (B) OF THE SAME PATIENT
TRICHOBLASTOMA ON TOP OF NEVUS SEBACEOUS
TRICHOBLASTOMA ON TOP OF NEVUS SEBACEOUS
SYRINGOCYSTADENOMA PAPILLIFERUM ON TOP OF NEVUS SEBACEOUS
BASAL CELL CARCINOMA ON TOP OF NEVUS SEBACEOUS
C/P OF NEVUS SEBACEOUS
TRICHOBLASTOMA is the most common secondary proliferation. Other common secondary neoplasms include syringocystadenoma papilliferum, trichilemmoma, desmoplastic trichilemmoma, sebaceous adenoma, apocrine adenoma, and poroma.
The actual incidence of secondary BCC is less than 1%. Only rarely does secondarysebaceous carcinoma or apocrine carcinoma arise in a nevus sebaceous. Malignancy probably develops only in longstanding or neglected lesions. Most tumors occur in adults older than 40 years.
NEVUS SEBACEOUS SYNDROME Nevus sebaceous syndrome refers to the rare association of a large nevus
sebaceous with disorders of the eye, brain and skeleton.
Sebaceous naevus syndrome may result in eye tumors and the skull may be
asymmetrical. Characteristic associated neurological features may include:
1. Developmental delay.
2. Epileptic seizures, especially infantile spasms.
3. Hemiparesis or cranial nerve palsies.
4. X-ray images and ultrasound evaluation may be quite normal.
5. Various structural abnormalities may be found within the brain.
HISTO. OF NEVUS SEBACEOUS
Infantile nevus sebaceous malformed follicular units are small and
deviate little from normal.
Childhood tiny deformed immature hair follicles provide a stark
contrast to normal terminal follicles at the periphery of the biopsy.
Small sebaceous and apocrine glands may be present. The epidermis
also thickens and becomes progressively more papillated, gradually
assuming a configuration akin to an epidermal nevus.
HISTO. OF NEVUS SEBACEOUS
late adolescence there is thickening of the epidermis with acanthosis
and papillomatosis. Clusters of follicular germinative cells positioned
along the dermal–epidermal junction. The underlying follicular units
remain smallish and appear distorted but their sebaceous lobules
increase in prominence. Dilated apocrine glandular structures with
inspissated secretions are not uncommonly identifiable in the reticular
dermis. This histopathology remains stable into adulthood.
NEVUS SEBACEUSMEDIUM MAGNIFICATION REVEALS EXPANSION OF THE EPIDERMIS IN A PAPILLATED FASHION, MUCH
LIKE THE PATTERN OF AN EPIDERMAL NEVUS.
THIS IS ACCOMPANIED BY ADNEXAL MALFORMATION, WITH ENLARGED SEBACEOUS
LOBULES AND CLUSTERS OF FOLLICULAR GERMINATIVE CELLS POSITIONED ALONG THE
DERMAL–EPIDERMAL JUNCTION.
NEVUS SEBACEUSPAPILLOMATOUS EPIDERMAL
HYPERPLASIA, WITH BASKET WEAVE
HYPERKERATOSIS AND HYPERTROPHIC
SEBACEOUS GLANDS.
NEVUS SEBACEUSSEBACEOUS GLAND OPENING INTO THE
EPIDERMIS.
HISTO. OF NEVUS SEBACEOUS Over time, some patients develop exaggerated verrucous epidermal
hyperplasia much like a verruca vulgaris.
Secondary neoplasms are common, and trichoblastoma,
trichilemmoma and syringocystadenoma are frequently observed. The
strong association between syringocystadenoma and nevus sebaceus
warrants careful inspection any time the diagnosis of
syringocystadenoma is made, to find out whether a nevus sebaceus can
be found in the same specimen.
Rx OF NEVUS SEBACEOUS Conservativecomplete excision in late childhood represents a commonlyutilized
approach due to; 1. Nevus sebaceous becomes increasingly verrucous and unsightly over time.2. The risk for the development of a secondary benign neoplasm, such as trichoblastoma or
syringocystadenoma, as it is relatively high.3. The risk of development of secondary carcinoma although it is low. 4. Lesions within the scalp may be difficult to follow clinically.
For facial lesions, consideration should be given to excision during childhood, beforethe development of secondary verrucous alteration, at a time when the risk ofscarring is reduced compared to adulthood.
The excisional margins can be minimal. Removal by shave or laser ablation is usually not successful.
TRICHOEPITHELIOMA
TRICHOEPITHELIOMA
Trichoepithelioma is a benign cutaneous neoplasms that originates from germinative cells of hair follicles.
It occurs as a sporadic non-familial form or as multipletrichoepitheliomas that is transmitted as an autosomaldominant trait. Lesions first appear in childhood and gradually increase in number with age.
For many, in the current dermatopathologic terms, trichoepithelioma represents a variant of trichoblastoma.
MULTIPLE TRICHOEPITHELIOMAS
ASYMPTOMATIC, MULTIPLE, FIRM, TRANSLUCENT
PAPULES INVOLVED THE MIDFACE ESPECIALLY THE
NASOLABIAL FOLDS.
MULTIPLE TRICHOEPITHELIOMAS
MULTIPLE FAMILIAL TRICHOEPITHELIOMAS
NUMEROUS SKIN-COLORED PAPULES AND NODULES ON
THE MIDFACE .
MULTIPLE TRICHOEPITHELIOMAS
MULTIPLE TRICHOEPITHELIOMAS
C/P OF TRICHOEPITHELIOMA
CLINICAL VARIANTS OF TRICHOEPITHELIOMA:
1. Solitary trichoepithelioma
2. Multiple Trichoepitheliomas either isolated or in association with
Brooke-Spiegler Syndrome.
3. Desmoplastic trichoepithelioma.
4. Trichoblastoma.
C/P OF TRICHOEPITHELIOMA
1. SOLITARY TRICHOEPITHELIOMA occurs more
commonly than multiple trichoepitheliomas usually
presents as a firm small (usually less than one
centimeter), skin-colored, yellow or pink dome-
shaped shiny papule or nodule on the face (lesions
have a predilection for the nose) or upper trunk.
C/P OF TRICHOEPITHELIOMA2. MULTIPLE TRICHOEPITHELIOMAS are yellowish-pink,
translucent dome-shaped papules distributed
symmetrically on the nasolabial areas, cheeks, eyelids,
forehead and upper lip occasionally occur on the neck and
the upper trunk.
The lesions may cause disfigurement because of
involvement of the face.
Often inherited as an autosomal dominant trait, the
papules first appear during childhood and grow slowly for
years and usually gradually increase in number with age.
C/P OF TRICHOEPITHELIOMA
BROOKE-SPIEGLER SYNDROME, is a rare genetic conditionresults in predisposition to three types of benign skinappendage tumor.
1. Trichoepitheliomas: with lesional density is always greatest in the central face is associated with a risk of secondary BCC.
2. Cylindromas: solitary or multiple tumors on the scalp may transform into cylindrocarcinoma.
3. Spiradenomas: painful nodules on head, neck and trunk may transform into spiradenocarcinoma.
C/P OF TRICHOEPITHELIOMA
3.DESMOPLASTIC TRICHOEPITHELIOMA represents a
variant of trichoepithelioma with exaggerated stromal
sclerosis (desmoplasia).
Commonly presents as a shiny firm skin colored to
erythematous annular lesion with a central dimple usually
solitary on the cheek of a woman. Mostly does not exceed
1 cm in diameter.
DESMOPLASTIC TRICHOEPITHELIOMA
PRESENT ON THE CHEEK OF A WOMAN
C/P OF TRICHOEPITHELIOMA4. TRICHOBLASTOMA: is very rare and tend to be larger and more
deeply located (in the deep dermis and subcutaneous tissue) thanclassical trichoepithelioma which is more superficial.
Presents as a small well circumscribed, solitary of skin-colored to brown or blue-black papules or nodules, usually 1-2 cm in diameter. They most commonly occur on the face and scalp of adults around 40-50 years of age.
Trichoblastoma is the most common neoplasm developed in pre-existing nevus sebaceus.
It may occasionally co-exist with BCC. Some trichoblastoma may also transform into the malignant counterpart TRICHOBLASTICCARCINOMA, which can be locally aggressive and have the potential to spread and metastasize.
TRICHOBLASTOMA ON TOP OF NEVUS SEBACEOUS
TRICHOBLASTOMA ON TOP OF NEVUS SEBACEOUS
HISTO. OF TRICHOEPITHELIOMA As they are benign neoplasms relative symmetry, circumscription
and a lack of cytologic atypicality. Histological features are multiple dermal nodules of basaloid cells
surrounded by fibrous stroma (similar to BCC). Prominence of follicular germinative basaloid cells that have scant
cytoplasm and dark staining nuclei and show striking peripheralpalisading with surrounding fibrocytic stroma that varies in degree.They are often arranged as small clusters or as reticulate and cribriform cords.
Small cornifying cystic spaces containing laminated keratin (horncysts) and a lining of pinkish squamous cells (keratinocytes), reflecting concurrent infundibular or isthmic differentiation, are also apparent.
HISTO. OF TRICHOEPITHELIOMA
In classic trichoepithelioma, the fibrocytic stroma is obvious and constitutes as much as half of the cellularity of the lesion or may be arrayed as either small or large nodules with only scantintervening sclerotic stroma.
A distinctive feature is the papillary mesenchymal bodies (foci of fibroblastic bulbar differentiation resembling abortive follicularpapillae).
There may be tiny collections of mature sebocytes, reflecting concurrent sebaceous differentiation, and (apocrine) ductaldifferentiation may also be noted.
TRICHOEPITHELIOMATHIS “CLASSIC” TRICHOEPITHELIOMA IS COMPOSED MOSTLY OF
CLUSTERS OF FOLLICULAR GERMINATIVE CELLS BUT ALSO
SHOWS SUPERFICIAL FOLLICULAR DIFFERENTIATION, WITH
SMALL KERATINIZING CYSTIC SPACES.
NOTE THAT CLEFTS WITHIN THE PROLIFERATION ARE
BETWEEN STROMAL ELEMENTS, IN CONTRAST TO THE CLEFTS
BETWEEN TUMOR AND STROMA THAT ARE CHARACTERISTIC OF
BASAL CELL CARCINOMA.
HISTOLOGY OF TRICHOEPITHELIOMA
WELL-DEMARCATED NODULES CONSIST OF SMALL
BASALOID CELLS THAT ARE ARRANGED IN A
PALISADE-LIKE PATTERN; MULTIPLE HORN CYSTS
WITH A FULLY KERATINIZED CENTER SURROUNDED
BY BASALOID CELLS LIE FREE IN THE FIBROUS
STROMA.
HISTOLOGY OF TRICHOEPITHELIOMA
THE CYSTIC SPACES CONTAIN KERATIN.
NOTICE THE LACK OF MITOTIC FIGURES OR
APOPTOTIC BODIES.
DESMOPLASTIC TRICHOEPITHELIOMA
IT IS COMPOSED OF THIN STRANDS OF
BASALOID (FOLLICULAR GERMINATIVE) CELLS,
ARRAYED IN SCLEROTIC STROMA.
MUCH LIKE CONVENTIONAL
TRICHOEPITHELIOMA, THERE ARE FOCI OF
SUPERFICIAL FOLLICULAR KERATINIZATION.
HIGH-POWERED VIEW OF DESMOPLASTIC
TRICHOEPITHELIOMANOTICE THE CLUSTER OF SQUAMOUS CELLS
SURROUNDING A SMALL CYSTIC AREA CONTAINING
KERATIN. INTERVENING STROMA IS MARKEDLY FIBROUS.
Rx OF TRICHOEPITHELIOMA Trichoblastoma is a benign condition so no treatment may be indicated but follow-up
for the possibility of secondary BCC.
Individual lesions may be removed surgically but scarring may occur.
Multiple facial trichoepitheliomas can be cosmetically disabling and because of the number of lesions, conventional excision is not indicated.
Other ablative approaches have been employed with some success but partialdestruction of the tumor is usually followed by regrowth. These include;
1. Electrodesiccation
2. Laser3. Dermabrasion
4. Cryotherapy
LASER SURGERY OF MULTIPLE TRICHOEPITHELIOMAS
IMMEDIATELY POST-PROCEDURE WITH
THE CO2 LASER AND
ELECTRODESICCATION OF LARGER
NODULES
AFTER TWO SESSIONS WITH CO2
LASER SURGERY
AFTER THREE YEAR FOLLOW-UPBEFORE TREATMENT
NEOPLASMS AND PROLIFERATIONS WITH SEBACEOUS DIFFERENTIATION
NEOPLASMS AND PROLIFERATIONS WITH SEBACEOUS DIFFERENTIATION
1. SEBACEOUS GLAND HYPERPLASIA
2. SEBACEOUS ADENOMA
3. SEBACEOUS EPITHELIOMA AND SEBACEOMA
4. SEBACEOUS CARCINOMA
SEBACEOUS GLAND HYPERPLASIA
SEBACEOUS GLAND HYPERPLASIA (SGH)
SGH is relatively common and does not
represent a true neoplasm. It represents
benign enlargement of sebaceous glands
usually seen on the forehead or cheeks of
the middle-aged and elderly.
SEBACEOUS HYPERPLASIA
C/P OF SEBACEOUS GLAND HYPERPLASIA
Presents with single or multiple, small, shiny, yellowish,up to 5 mm in diameter papules, usually on the central orupper face and sometimes on the upper trunk.
Commonly, the clinical lesions display a centralumbilication that corresponds to a central follicularinfundibular ostium.
There are often telangiectasia best seen using dermoscopy.
C/P OF SEBACEOUS GLAND HYPERPLASIA
Sebaceous hyperplasia may be more prevalent in immunosuppressed patients: for example, in a patient following organ transplantation.
Juxta-clavicular beaded lines - variant of sebaceous hyperplasia characterized by papulesarranged in parallel rows resembling 'strands ofbeads'.
SEBACEOUS HYPERPLASIASKIN COLORED TO YELLOWISH PAPULES WITH A CENTRAL
DELL
SEBACEOUS HYPERPLASIA
SEBACEOUS HYPERPLASIA
SEBACEOUS HYPERPLASIANUMEROUS LESIONS OF SEBACEOUS HYPERPLASIA IN A SOLID
ORGAN TRANSPLANT RECIPIENT RECEIVING LONG-TERM
CYCLOSPORINE
HISTO. OF SEBACEOUS GLAND HYPERPLASIA
The sebaceous gland in SGH shows normal
morphology.
The enlarged numerous sebaceous lobules usually
circumferentially surround a central infundibulum.
Sebaceous lobules composed of mature sebocytes
with a thin rim of seboblasts.
Rx OF SEBACEOUS GLAND HYPERPLASIA
MEDICAL:
• Topical retinoid for long-term application may be beneficial.
• Oral isotretinoin may be utilized for patients with extensive disfiguring lesions but these
may recur when treatment is stopped.
SURGICAL: If treatment is desired, lesions can be removed or diminished by;
1. Shave excision
2. Light electrosurgical destruction
3. Cryotherapy
4. Laser ablation
SEBACEOUS ADENOMA/SEBACEOMA
SEBACEOUS ADENOMA/SEBACEOMA Sebaceous adenoma is superficial benign
neoplasms in which there is a proliferation of
mature sebocytes and seboblasts.
Sebaceoma is deeply seated benign neoplasms
in which there is a proliferation of mature
sebocytes and seboblasts but seboblasts
predominates.
C/P OF SEBACEOUS ADENOMA/SEBACEOMA
It presents clinically as solitary or multiple yellowish papules or nodules, usually less than a centimeter in diameter, distributed on the head or neck and sometimes on the upper trunk.
Superficial sebaceous adenomas are usually relatively small and papular, while sebaceomas may present as a deep nodule.
Multiple sebaceous neoplasms, especially cystic ones, may serve as a presenting sign of the MUIR–TORRE SYNDROME.
SEBACEOUS ADENOMAS ON THE FACE IN A
PATIENT WITH MUIR-TORRE SYNDROME
A, EXTERNAL APPEARANCE OF THE LEFT EYE. NOTE A YELLOWISH-PINK WARTY
GROWTH ARISING FROM THE ANTERIOR LAMELLA OF THE LEFT UPPER EYELID. B, MULTIPLE YELLOW NODULAR LESIONS INVOLVING THE CENTRAL FOREHEAD,
NOSE, AND ADJACENT CHEEK AREA (ARROW).
MUIR-TORRE SYNDROME Is a rare inherited autosomal dominant condition in which there are
sebaceous skin neoplasms in association with internal cancer. The most common organ involved is the gastrointestinal tract, with
almost one half of patients having colonic adenocarcinoma. The second most common site is cancer of the genitourinary tract.
The sebaceous neoplasms include:
1. Sebaceous adenomas
2. Sebaceous epitheliomas
3. Sebaceous carcinomas Other skin lesions that may arise in affected family members
include:
1. Multiple Keratoacanthoma
2. Squamous cell carcinoma
3. Multiple follicular cysts
HISTO. OF SEBACEOUS ADENOMA/SEBACEOMA
SEBACEOUS ADENOMA is usually superficial containing mature sebocytic cells and basaloid seboblastic cells but sebocytes usually predominate. Seboblasts comprise a thin multilayer of cells at the periphery of each lobule that may show mitotic figures.
SEBACEOMA is usually more deeply situated, often involving the deep reticular dermis and sometimes the superficialhypodermis. The lesions are lobulated sharply circumscribedand seboblasts predominate and sebocytic differentiation may be only focal and remain the minor proportion.
SEBACEOUS ADENOMAIT SHOWS MATURE SEBOCYTIC CELLS
SURROUNDED BY A RIM OF BASALOID
SEBOBLASTIC CELLS.
SEBACEOMATHIS HIGH-MAGNIFICATION VIEW SHOWS A
BACKGROUND OF BASALOID SEBOBLASTIC CELLS
PUNCTUATED BY SCATTERED MATURE SEBOCYTES
WITH COARSELY VACUOLATED CYTOPLASM. THE
NEOPLASM WAS LARGE BUT SHARPLY
CIRCUMSCRIBED AT LOW MAGNIFICATION.
SEBACEOMAFREQUENT MITOSES CAN BE SEEN IN THIS
TUMOR, THOUGH CYTOLOGICAL ATYPIA IS
LACKING
Rx OF SEBACEOUS ADENOMA/SEBACEOMA
Complete surgical excision of the lesion is recommended to
exclude the possibility of BCC with sebaceous differentiation or
sebaceous carcinoma.
NEOPLASMS AND PROLIFERATIONS WITH APOCRINE AND ECCRINE DIFFERENTIATION
NEOPLASMS AND PROLIFERATIONS WITH APOCRINE AND ECCRINE DIFFERENTIATION It is difficult to determine whether sweat gland tumors are of eccrine or
apocrine differentiation. The precise diagnosis of sweat gland lesions is most likely to be made histologically rather than clinically.
Benign Neoplasms and Proliferations may be of both apocrine or eccrine Differentiation
1. Syringoma
2. Poroma
3. Hidradenoma
4. Adnexal adenocarcinoma
NEOPLASMS AND PROLIFERATIONS WITH APOCRINE DIFFERENTIATION
1. Apocrine adenoma
2. Spiradenoma
3. Cylindroma
4. Apocrine adenocarcinomas
NEOPLASMS AND PROLIFERATIONS WITH ECCRINE DIFFERENTIATION
1. Eccrine nevus (hamartoma)
2. Syringofibroadenoma
3. Papillary adenoma
4. Papillary adenocarcinoma
SYRINGOMA
SYRINGOMA
Syringoma represents a benign adnexal
neoplasm with mostly ductal (syringeal)
differentiation usually found on the upper
cheeks and lower eyelids of young adults.
SYRINGOMA
C/P OF SYRINGOMA Asymptomatic small, firm, skin-colored to yellowish papule
commonly multiple and symmetric may be eruptive. They first appear during adolescence and further increase
during adult life and remain indefinitely. Prone to occur in the periorbital area, especially the lower
eyelids sometimes, lesions involve the upper trunk, favoring the ventral surface, or genital skin.
The lesions are removed from women more commonly than men.
There are an association with Down syndrome. Clear cellsyringomas are associated with diabetes mellitus.
SYRINGOMA
COBBLESTONE APPEARANCE OF THE
SKIN UNDER THE EYES TYPICAL OF
MULTIPLE SYRINGOMAS.
SYRINGOMAS ON THE NECK MULTIPLE SKIN-COLORED, FIRM, SMALL
LESIONS. THESE BENIGN LESIONS TYPICALLY
APPEAR NEAR THE EYELIDS, BUT THEY CAN
OCCUR LOWER ON THE FACE.
SYRINGOMAS
C/P OF SYRINGOMA
ERUPTIVE SYRINGOMAS which is less common
appear as a crop of multiple lesions typically on the
chest, neck or lower abdomen but may involve the
extremities, including the palms and soles.
SYRINGOMA
SYRINGOMASMULTIPLE SKIN-COLORED TO PINK, SMOOTH PAPULES
ON THE NECK AND UPPER CHEST. THE LESIONS FAVOR
THE VENTRAL SURFACE OF THE TRUNK.
ERUPTIVE SYRINGOMA
ERUPTIVE SYRINGOMAS
SYRINGOMAS IN THE GENITAL AREAS
HISTO. OF SYRINGOMA The lesion is circumscribed and is usually confined to the
superficial dermis. Epithelial cells with dark stained nuclei and pale eosinophilic
cytoplasm forming nests and tubules with surrounding scleroticstroma.
Depending upon the exact plane of section, the nests of a syringoma vary in shape, and some nests may assume a characteristic morphology that resembles a comma or a tadpole.
Tubular areas exhibit ductular differentiation with central luminalined by a compact eosinophilic cuticle.
Usually syringoma lacks keratinization or follicular differentiation.
HISTOPATHOLOGY OF SYRINGOMA
DUCTULAR STRUCTURES (1) AND MORE
SOLID APPEARING NESTS AND STRANDS
(2) THAT ARE PRESENT WITHIN THE
DERMIS.
SYRINGOMATHIS SUPERFICIAL ADNEXAL NEOPLASM
CONSISTS OF NESTS OF CELLS WITH PALE
CYTOPLASM POSITIONED WITHIN SCLEROTIC
STROMA. MANY NESTS SHOW CENTRAL
DUCTAL DIFFERENTIATION WITH A COMPACT
EOSINOPHILIC CUTICLE.
HISTOLOGY OF SYRINGOMA
HISTOLOGY OF SYRINGOMA
SHOWING TYPICAL "TADPOLE/ COMMA
SHAPED" EPITHELIAL TUMOR STRAND
Rx OF SYRINGOMA As it is a benign neoplasm and in most cases are left alone.
If they become cosmetically disturbing, destructivetreatment options may be employed.
Although there is a risk of scarring treatment options include;
1. Trichloroacetic acid
2. Cryotherapy
3. Punch excision
4. Electrosurgical destruction
5. Dermabrasion
6. Laser ablation especially in disseminated lesions
TREATMENT OF SYRINGOMA
SYRINGOMA BEFORE ELECTROSURGERY SYRINGOMA AFTER ELECTROSURGERY
POROMA
POROMA
Poroma represents a group of benign adnexal
neoplasms with poroid differentiation (derived
from cells of the terminal sweat duct and
connected to the epidermis).
Poroma can be a proliferation of either
apocrine or eccrine lineage
POROMA
POROMA
POROMA
POROMA
C/P OF POROMA Poromas usually present as solitary skin-colored to reddish
papules, plaques or nodules and may resemble pyogenic granuloma but may also appear as a pigmented lesion.
Most lesions are asymptomatic, but minor pain can accompany some lesions
They can appear on any cutaneous surface but those with eccrine differentiation are most commonly found on the palms, soles and scalp (with or without an associated nevussebaceus).
When sessile vascular plaques surrounded by thin groove appear on the palms and soles.
POROMATOSIS: Rarely, multiple poromas develop, either in an acral or in a widespread distribution.
HISTO. OF POROMA Circumscribed proliferation of compact cuboidal “poroid” cells with
small monomorphous nuclei and scanty eosinophilic cytoplasm. The narrow ducts are often lined by a row of cuboidal cells. According to extension it may be;
1. Intra-epidermal poroma: nests of cells with tubular differentiation are confined to the surface epidermis.
2. Juxta-epidermal poroma: In continuity with epidermis but also involve the superficial dermis.
3. Intra-dermal poroma: confined wholly within the dermis.
HISTOCHEMISTRY: shows positive carcinoembryonic antigen (CEA) immunostaining labels the luminal surface of both apocrine and eccrine ducts.
OUTLINES OF THE 3 VARIANTS OF POROMA
INTRAEPIDERMAL POROMA JUXTA-EPIDERMAL POROMA INTRADERMAL POROMA
JUXTAEPIDERMALPOROMA
PRESENTS HISTOPATHOLOGICALLY IN
CONTINUITY WITH THE EPIDERMIS,
CREATING A SEBORRHEIC KERATOSIS-
LIKE PROFILE.
POROMA, JUXTA-EPIDERMAL PATTERN
THIS POROMA DISPLAYS A PATTERN COMMON TO BOTH
ECCRINE AND APOCRINE POROMA.
THERE ARE INTER-ANASTOMOSING CORDS COMPOSED
OF SMALL COMPACT “POROID” CELLS, AND THE
INTERVENING STROMA IS HIGHLY VASCULARIZED AND
RESEMBLES GRANULATION TISSUE.
INTRADERMAL POROMA SHOWS A SHARPLY CIRCUMSCRIBED BORDER
AT SCANNING MAGNIFICATION. HIGHLY
VASCULARIZED AND FOCALLY SCLEROTIC
STROMA IS ALSO EVIDENT.
INTRADERMAL POROMA AT HIGHER MAGNIFICATION, DUCTAL
DIFFERENTIATION IS CONSPICUOUS.
Rx OF POROMA
Poroma is a benign adnexal neoplasm and thus treatment is
optional.
Superficial lesions may be treated by shave or electrosurgical
destruction.
Superficial or deeper lesions may also be treated with simple
surgical excision.
REFERENCES
Bolognia 3rd Ed.
http://dermnetnz.org
Google Images
globalskinatlas.com