Fibrous and Fibrohistiocytic Proliferations of the Skin P1

FIBROUS AND FIBROHISTIOCYTIC PROLIFERATIONS OF THE SKIN P.IWWW.FACEBOOK.COM/GROUPS/DERMATOLOGYCOURSEONLINE

http://www.facebook.com/groups/dermatologycourseonline

CLASSIFICATION OF FIBROUS AND FIBROHISTIOCYTIC PROLIFERATIONS OF THE SKIN

1. Skin tags

2. Cutaneous angiofibroma

3. Dermatofibroma

4. Dermatomyofibroma

5. Superficial acral fibromyxoma

6. Sclerotic fibroma of the skin

7. Pleomorphic fibroma of the skin


8. Nodular fasciitis

9. Multinucleate cell angiohistiocytoma

10. Epithelioid fibrous histiocytoma

11. Connective tissue nevus

12. Infantile digital fibroma

13. Infantile myofibromatosis

14. Calcifying aponeurotic fibroma

15. Keloids and hypertrophic scars


16. Fibrous hamartoma of infancy17. Fibromatoses18. Plexiform fibrohistiocytic tumor 19. Atypical fibroxanthoma20. Dermatofibrosarcoma protuberans21. Giant cell fibroblastoma22. Fibrosarcoma23. Epithelioid sarcoma

SKIN TAGS

Multiple skin tags in the axilla

OVERVIEW

Skin tags are very COMMONBENIGN SOFT fleshyFIBROVASCULAR SKIN-COLOREDto PINK or occasionallyHYPERPIGMENTED, PEDUNCULATED papular tumors.

Up to 50% of all individuals haveat least ONE SKIN TAG.

SKIN TAGS

OVERVIEW

THEY ARE ALSO DESCRIBED AS:

ACROCHORDONS

PAPILLOMAS

FIBROEPITHELIAL POLYPS

SOFT FIBROMAS

SKIN TAGS

ETIOLOGY

It is NOT KNOWN what causes skintags. However, the following factorsmay play a role:

1. FRICTION2. GROWTH FACTORS,

particularly during pregnancyor in acromegaly.

3. INSULIN RESISTANCE.4. ?HPV

SKIN TAGS

CLINICAL FEATURES

Acquired THROUGHOUT LIFEand INCREASE in FREQUENCYwith AGE in both sexes.

Range in size from 1MM to 5CM.

Sites of predilection SKIN FOLDS: neck, axilla, groin, inframammary region & eyelids.

SKIN TAGS

CLINICAL FEATURES

They have a SMOOTH orFOLDED surface.

Can become IRRITATED orINFARCTED.

They tend to be MORENUMEROUS in OBESE personsand in those with TYPE 2DIABETES MELLITUS.

SKIN TAGS

HISTOPATHOLOGY

Benign FIBROVASCULAR tumorsdue to NORMAL EPIDERMIS orEPIDERMAL HYPERPLASIAoverlying a dermal stalk.

SKIN TAGS

HISTOPATHOLOGY

A CORE of DERMIS made up ofloosely arranged COLLAGENFIBERS and BLOOD VESSELSand/or SUBCUTANEOUS FATTYTISSUE.

SKIN TAGS

TREATMENT

Skin tags are benign & can be removed only for COSMETICREASONS.

SKIN TAGS

TREATMENT

Options include:

1. CRYOTHERAPY

2. SURGICAL EXCISION (oftensnipping with scissors)

3. ELECTROSURGERY

4. LASER e.g. CO2 laser

SKIN TAGS

Electrosurgery and snipping with scissors

Carbon dioxide laser removal

ANGIOFIBROMAS

Fibrous papule of the nose - smooth, dome-shaped, skin-colored papule

Fibrous papule of the face

Koenen’s tumor

Acral fibrokeratoma - light pink exophytic papule arising from the dorsal surface of the finger

Acquired digital fibrokeratoma- Slightly raised skin encircling the base of an acquired digital fibrokeratoma, creating a moat

Pearly penile papules - Multiple small white papules along the corona of the glans penis. Note the multilayered distribution.

OVERVIEW

SINGLE or MULTIPLE SMALL, RED, or SKIN-COLORED PAPULES.

DERMAL VESSELS withsurrounding FIBROSIS.

Several conditions related.

ANGIOFIBROMAS

CLINICAL TYPES

1. FIBROUS PAPULE OF THE FACE

2. ADENOMA SEBACEUM

3. ACRAL FIBROKERATOMA

4. PEARLY PENILE PAPULES (PPP)

ANGIOFIBROMAS

OVERVIEW

RELATIVELY COMMONCLINICALLY INDISTINCTSOLITARY, FIRM, SHINY papule;mostly on the NOSE less commonly on other areas of the FACE.

The exact reason is UNKNOWN.

FIBROUS PAPULE OF THE FACE

1

CLINICAL FEATURES

Usually ASYMPTOMATIC

Develops during LATEADOLESCENCE or EARLY ADULTLIFE on the nose, or less often, elsewhere on the face esp. the lips.

More SKIN COLORED than RED.


CLINICAL FEATURES

It is a firm DOME SHAPED SHINYlesion usually only a FEW MMS insize, sometimes bearing a centralhair. It is persists UNCHANGEDlifelong.

Occasionally, lesions are SESSILE, POLYPOID, or PAPILLOMATOUS.


Diffuse reddish colour and the pink or whitish colour in the lesion representing fibrosis

DDx

1. SMALL BASAL CELLCARCINOMA

2. PYOGENIC GRANULOMA

3. INTRADERMAL MELANOCYTICNEVUS

4. ADNEXAL TUMORS.


Histopathology of a fibrous papule - focal fibrosis & vascular proliferation in the upper dermis

HISTOPATHOLOGY

Well-circumscribed SLIGHTLYRAISED upper dermal lesioncomposed of: Proliferation of FIBROBLASTS STELLATE, SPINDLE cells and/orMULTINUCLEATED cells.FIBROTIC hyalinized STROMAcomposed mainly of COLLAGENbundles with DILATED BLD VVS.


HISTOPATHOLOGY

The EPIDERMIS is NORMAL, ACANTHOSIS or SLIGHTLYATROPHIC and FLATTENED RETERIDGES.

Occasionally, a sparseINFLAMMATORY CELLINFILTRATE of lymphocytes is present.


IMMUNOHISTOCHEMISTRY

The lesional cells stain for factor XIIIa and sometimes CD34.


TREATMENT

Dose NOT require anyTREATMENT May be removed forCOSMETIC reasons.

If desired it may be removed by

EXCISION BIOPSY

SHAVE BIOPSY

ELECTROSURGERY


OVERVIEW

MULTIPLE FACIALANGIOFIBROMAS IN TUBEROUS SCLEROSIS.

ADENOMA SEBACEUM

2

OVERVIEW

RARE benign SOLITARY, SKIN-COLORED DOME-SHAPED or TALL FINGERLIKE PROTRUSIONSwith a hyperkeratotic surface.

Often areas of TRAUMA or SUBCLINICAL INJURY.

ACRAL FIBROKERATOMA

3

VARIANTS

I. ACQUIRED DIGITAL FIBROKERATOMA (ADFK).

II. ACQUIRED PERIUNGUAL FIBROKERATOMA

III. SUBUNGUAL & PERIUNGUAL FIBROMAS OF TUBEROUS SCLEROSIS (KOENEN TUMORS) they tend to be multi-lobulated and involve several digits.

ACRAL FIBROKERATOMA

CLINICAL FEATURES

ASYMPTOMATICPROTUBERANCE.

MIDDLE-AGED adults

Lesions occurred not only on the FINGERS, but also on the proximal HAND, TOES, SOLE.

ACRAL FIBROKERATOMA

CLINICAL FEATURES

Usually not exceed 1.5 cm in height or diameter SOLITARY, SKIN-COLORED to PINK, CONE-SHAPED, KERATOTIC PAPULE.

CHARACTERISTIC COLLARETTE of SLIGHTLY RAISED skin that ENCIRCLES the BASE of the lesion creating a MOAT-LIKEconfiguration.

ACRAL FIBROKERATOMA

DDx

1. CORNS2. CUTANEOUS HORN3. INFANTILE DIGITAL

FIBROMATOSIS4. PYOGENIC GRANULOMA

(Lobular Capillary Hemangioma)5. SUPERNUMERARY DIGIT6. WARTS, NONGENITAL

ACRAL FIBROKERATOMA

Acquired digital fibrokeratoma

Massive orthokeratosis; core of thick collagen bundles oriented in vertical axis; small vessels in dermal papillae; no nerves, eccrine ducts, or cartilage

Domed-shaped papule with overlying hyperkeratosis. The dermal core is composed of increased collagen bundles and blood vessels oriented along the vertical axis of the lesion.

Close up showing the increased collagen bundles and blood vessels oriented along the vertical axis of the lesion.

HISTOPATHOLOGY

HYPERKERATOSIS most PRONOUNCED toward the SUMMIT of the lesion.

ACANTHOSIS, with ELONGATIONof the RETE RIDGES or can be slightly attenuated epidermis.

ACRAL FIBROKERATOMA

HISTOPATHOLOGY

Most commonly consists of a DERMALCORE composed of THICK, closely INTERTWINED COLLAGEN BUNDLESthat are often oriented along the VERTICAL AXIS of the lesion.

Between the collagen bundles are numerous CAPILLARIES, varying numbers of FIBROBLASTS, and thin ELASTIC fibers.

ACRAL FIBROKERATOMA

TREATMENT

SIMPLE EXCISION is curative; recurrence is rare.

ACRAL FIBROKERATOMA

OVERVIEW

ASYMPTOMATIC, MULTIPLESMALL PEARLY-WHITE DOME-SHAPED to FILIFORM PAPULES ina GROUP or ROWS along the SULCUS or CORONA of theGLANS PENIS CIRCUMFERENTIALLY.

PPP

4

OVERVIEW

They're NORMAL ANATOMICvariant present in between 8% and 43% of men.

More common in UNCIRCUMCISED men.

Often, lesions cause GREATANXIETY to patients until their BENIGN NATURE is clarified.

PPP

CLINICAL FEATURES

1-3 ROWS of tiny, 1–3 MMGLISTENING, FLESH-COLORED, WHITE or LIGHT PINK papules.PERSIST THROUGHOUT LIFE; however, they GRADUALLY may become less noticeable with increased AGE.

PPP

DDx

1. GENITAL WARTS

2. MOLLUSCUM CONTAGIOSUM

3. ECTOPIC SEBACEOUS GLANDS (FORDYCE SPOTS)

PPP

PPP- Papule with fibroblasts, rich vascularity, dense connective tissue

HISTOPATHOLOGY

Variable number of THIN-WALLED DILATED BLOODVESSELS in the dermis with a proliferation of FIBROBLAST cells which may be STELLATE or MULTINUCLEATED. CONCENTRIC FIBROSIS may also be found around skin APPENDAGE structures.

PPP

TREATMENT

REASSURANCE - No treatment is needed. Any destructive modality may be employed but SCARRING is a risk.

REMOVAL OPTIONS (To alleviate anxiety);

CRYOTHERAPYELECTRODESICCATION & CURETTAGESURGICAL EXCISION CO2 LASER

PPP

KELOIDS AND HYPERTROPHIC SCARS

Clawlike outline of a keloid

Keloid Formation After Laser Tattoo Removal

Massive recurrent facial keloid

OVERVIEW

Keloid are FIRM to HARD, SMOOTH, GROWTHS due to SPONTANEOUS SCARFORMATION and much LARGERthan the ORIGINAL WOUND.

KELOID & HYPERTROPHIC SCARS

OVERVIEW

Keloids may form on ANY PART of the body, although the EARS, UPPERCHEST & shoulders are especially prone.While most people never form keloids, others develop them after MINORINJURIES, burns, insect bites and acne. DARK SKINNED people form keloids more easily than Caucasians.


WHAT ARE HYPERTROPHIC SCARS?

As wounds heal, SCAR TISSUE forms, which at first is often ERYTHEMATOUS and somewhat PROMINENT. Over several MONTHS, a scar usually becomes FLAT and PALE. If there is a LOT of TENSION on a healing wound, the healing area is RATHER THICKER than USUAL. This is known as a HYPERTROPHIC SCAR.


WHAT ARE HYPERTROPHIC SCARS?

Hypertrophic scars remain LIMITED to the TRAUMATIZEDAREA.

Regress SPONTANEOUSLY within 12-24 months, although regression may NOT necessarily be COMPLETE.


PREVALENCE

The prevalence has been reported to be HIGHER in YOUNG FEMALESthan in young males, probably reflecting the greater frequency of EARLOBE PIERCING among females.

Keloids and hypertrophic scars affect BOTH SEXES equally in OTHERAGE GROUPS.


ETIOLOGY

The EXACT MECHANISMS of keloid and hypertrophic scar pathogenesis continue to be an ENIGMA for physicians and researchers alike.

The INCREASED PREVALENCE of keloids paralleling increased CUTANEOUS PIGMENTATIONsuggests a GENETIC BASIS or, at least, a genetic linkage.


ETIOLOGY

TRAUMA TO THE SKIN, both physical (e.g. earlobe piercing, surgery) and pathological (e.g. acne, chickenpox), is the primary cause of keloids. THE PRESENCE of FOREIGNMATERIAL, INFECTION, HEMATOMA, or increased SKIN TENSION can also lead to keloid or hypertrophic scar formation in susceptible individuals.


CLINICAL FEATURES

Keloids and hypertrophic scars do NOT usually cause SYMPTOMS, but they MAY be TENDER, PAINFUL, or PRURITIC or they may cause a burning sensation.

In addition to symptomatic relief, COSMETIC CONCERN is the primary reason patients seek medical intervention.


CLINICAL FEATURES

Keloids manifest as EXAGGERATEDGROWTHS of SCAR TISSUE, USUALLYin areas of PREVIOUS TRAUMA.

Keloids extend BEYOND the areas of trauma, PROJECTING ABOVE the LEVEL of the SURROUNDING SKIN, but they rarely extend into underlying subcutaneous tissue.


CLINICAL FEATURES

Keloids range in CONSISTENCY from SOFT and DOUGHY to RUBBERY and HARD. Early lesions are often ERYTHEMATOUS. Lesions become BROWNISH RED and then PALE as they age. Lesions are usually DEVOID of HAIRFOLLICLES and other functioning ADNEXAL GLANDS.


CLINICAL FEATURES

MOST lesions CONTINUE to GROWfor WEEKS to MONTHS and OTHERSgrow for YEARS. Growth is usually SLOW, but keloids occasionally enlarge rapidly, tripling in size within months. Once they stop growing, keloids do not usually cause symptoms and REMAINSTABLE or INVOLUTE SLIGHTLY.


CLINICAL FEATURES

In WHITE persons, keloids tend to be present, in decreasing order of frequency, on the FACE (with CHEEK and EARLOBESPREDOMINATING), UPPER EXTREMITIES, CHEST, PRESTERNAL AREA, NECK, BACK, LOWER EXTREMITIES, breasts & abdomen.In BLACK persons, the descending order of frequency tends to be EARLOBES, FACE, NECK, LOWER EXTREMITIES, breasts, chest, back, and abdomen.


CLINICAL FEATURES

Keloids on the EARS, NECK, and ABDOMEN tend to be PEDUNCULATED. Keloids on the CENTRAL CHEST and extremities are usually RAISED with a FLAT surface, and the base is often wider than the top.

Keloids OVERLYING a JOINT can CONTRACT and restrict movement.


CLINICAL FEATURES

Most keloids are ROUND, OVAL, or OBLONG with REGULAR MARGINS; however, some have CLAWLIKEconfigurations with irregular borders. KELOIDS may be DISTINGUISHEDfrom HYPERTROPHIC SCARS by their CLAWLIKE PROJECTIONS, which are absent in the hypertrophic scar.


CLINICAL FEATURES

MOST patients present with 1 OR 2 keloids; however, a FEWpatients, especially patients with spontaneous keloids, have MULTIPLE LESIONS, as do patients who develop keloids as a consequence of acne or chickenpox.


Broad, eosinophilic bundles of collagen are the characteristic finding in keloids, not present in hypertrophic scars

Central zone of hyalinized collagen

Thick hyalinized collagen bundles

HISTOPATHOLOGY

Keloids are comprised of FIBROBLASTS, FIBROUS TISSUEwhich REPLACES NORMAL DERMALSTRUCTURES, and IRREGULARLYARRANGED THICK BANDS of COLLAGEN in the dermis & SC tissue.THICK, HYALINIZEDHYPEREOSINOPHILIC BANDS in the CENTRAL PORTION of the nodule.


PREVENTION

1. Avoid performing nonessential cosmetic surgery in patients known to form keloids.

2. Close all surgical wounds with minimal tension.

3. Incisions should not cross joint spaces. 4. Avoid making midchest incisions, and

ensure that incisions follow skin creases whenever possible.

5. Use buried sutures, when necessary, for a layered closure and to reduce tension.


TREATMENT

HYPERTROPHIC SCARS generally SETTLE in TIME OR with TREATMENT, but KELOIDS may PERSIST and prove resistant to treatment.


STANDARD TREATMENTS

These include OCCLUSIVEDRESSINGS, COMPRESSIONTHERAPY, and INTRALESIONALCORTICOSTEROID INJECTION.


TREATMENT MEASURES

1. EMOLLIENTS

2. OCCLUSIVE DRESSINGS

3. COMPRESSION THERAPY

4. SURGICAL EXCISION

5. INTRALESIONAL CORTICOSTEROID INJECTION

6. CRYOTHERAPY

7. SUPERFICIAL X-RAY TREATMENT.

8. LASER THERAPY

9. LIGHT THERAPIES

10. SKIN NEEDLING


TREATMENT MEASURES

OCCLUSIVE DRESSINGS, include silicone gel sheets and dressings, nonsilicone occlusive sheets, dressings should be worn for 12 to 24 hours per day.


TREATMENT MEASURES

COMPRESSION THERAPY Mechanoreceptors induce apoptosis also extracellular matrix. Rigidity caused by compression may also inhibit the differentiation and proliferation of scar fibroblasts(button compression, pressure earrings, ACE bandages, elastic adhesive bandages, compression wraps, spandex or elastane (Lycra) bandages, and support bandages)


TREATMENT MEASURES

SURGICAL EXCISION (but in keloids, excision may result in a new keloid even larger than the original one) Excisional surgery alone has been shown to yield a 45-100% recurrence rate.


TREATMENT MEASURES

INTRALESIONAL CORTICOSTEROID INJECTION, as a single modality and as an adjunct to excision repeated every few weeks reducing collagen synthesis, altering glucosaminoglycan synthesis, and reducing production of inflammatory mediators and fibroblast proliferation during wound healing. The most commonly used corticosteroid is triamcinolone acetonide(TAC) in concentrations of 10-40 mg/mL administered intralesionally with a 25- to 27-gauge needle at 4- to 6-week intervals.


TREATMENT MEASURES

CRYOTHERAPY - 2, or 3 freeze-thaw cycles lasting up to 30 seconds repeated every 20-30 days. Insertion of a lumbar puncture needle through the long axis of the keloid, from one side to the other, passing the liquid nitrogen.


TREATMENT MEASURES

LASER THERAPY Ablative lasers Carbon dioxide, argon laser, and Nd:YAG laser (1064 nm)Nonablative lasers Pulsed dye laser because of its efficacy, safety, and relatively low cost, the PDL remains the laser treatment of choice for hypertrophic scars.


MEDICAL TREATMENTS

1. Retinoic acid

2. Intralesional interferon (IFN)

3. Intralesional 5-fluorouracil (5-FU)

4. Intralesional calcium channel blockers verapamil

5. Doxorubicin

6. Bleomycin

7. Imiquimod 5% cream

8. Tacrolimus

9. Tamoxifen

10. Botulinum toxin type A,

11. Over-the-counter treatments (e.g. onion extract; combination of hydrocortisone, silicon, and vitamin E).


LIGHT THERAPIES

1. Photodynamic therapy [PDT]

2. Intense pulsed light (IPL)

3. UVA-1

4. Narrowband UVB

5. Broadband UVB


REFERENCES

BOLONGIA DERMATOLOGY ESSENTIALS

BOLONGIA 3rd ed

WEEDON’S SKIN PATHOLOGY ESSENTIALS

GOOGLE IMAGES

DERMNETNZ.ORG

EMEDICINE.MEDSCAPE.COM

THANK YOU

WWW.FACEBOOK.COM/GROUPS/DERMATOLOGYCOURSEONLINE

http://www.facebook.com/groups/dermatologycourseonline

Fibrous and Fibrohistiocytic Proliferations of the Skin P1

Health & Medicine

Transcript of Fibrous and Fibrohistiocytic Proliferations of the Skin P1