FASCIA PATHOLOGY

Definition and anatomy

The dense connective tissue that envelopes muscles grossly, and also surrounds every bundle of muscle fibers and each individual muscle cell. This connective tissue is inextricably linked with the muscle, and is continuous with the tendons and periosteum

Fascia is composed of cells including fibroblasts,macrophages ,mast cells and extracellular matrix. The extracellular matrix (ECM) is composed of ground substance, collagen and elastin fibers. Its richly innervated

Fibrosis and adhesions

One of the hallmarks of fascia is its mutability and remodeling in response to mechanical stress. However, under certain conditions excess mechanical stress, inflammation or immobility this process can result in excessive and disorganized collagen and matrix deposition resulting in fibrosis and adhesions eg in plantar fascitis

What is Necrotizing Fasciitis?

life-threatening, progressive, rapidly spreading, inflammatory infection located in the deep fascia. infection rapidly destroy the skin and soft tissue beneath it Also known as: flesh-eating bacteria. Other names: -hemolytic streptococcal gangrene, Meleney ulcer, acute dermal gangrene, hospital gangrene, and necrotizing cellulitis. 3 types of NF. Type I : a polymicrobial flora. Type II Group A -Streptococcus bacteria (most common case) Type III : marine vibrio gram-negative rods.

How does one contract NF?

exposed to an individual with an opening in their skin.
direct contact with someone carrying the bacteria the bacterium being carried by the person itself.

enter through weakened skin, as a contusion, a bruise, a blister, or even an abrasion.

Cofactors that Increase risks

Diabetes Alcoholism Immuno-suppression Severe illnesses: heart, lung, or liver disease Obesity

Whats going on inside your body?

Bacteria eat away at tissue between skin and muscle Increase in sensitivity or anaesthetic feel to the skin itself Inflammatory response by immune system Bacterial toxins released Cytokines impede function of phagocytic cells Anaerobes thrive speeding up necrotic process Endothelial cells become damaged; Increased permeability of the lining of vessels in the body Poor blood supply inhibit: Inflammatory response process Ability for the immune system to properly work Ability to transfer antibiotics to the affected fascial layer Vasoconstriction and thrombosis edema hypoxia necrosis of the fascia, skin, soft tissue, and muscles. Additional necrosis involving the subcutaneous nerves.

Exams and Laboratory Testing

1. Blood samples 2. Testing for elevated or lowered creatinine, glucose, CPK, bicarbonate, albumin, and calcium levels. 3. X-ray 4. CT, and MRI scanning 5. And most importantly antibiotic culture and sensitivity tests

Eosinophilic fasciitis
Also known as Shulmans syndrome A rare condition resulting in widespread eosinophilic infiltration and inflammation of the fascia. More common in people ages 30 to 60 Results in significant fibrosis of the fascia and adhesions

Symptoms
Bone pain or tenderness Carpal tunnel syndrome Muscle weakness Tenderness and swelling of upper and lower limbs Thickened skin with puckered appearance

Pathology
In early disease there is oedema of the fascia and subcutaneous tissue with a lymphocytic infiltrate containing plasma cells and eosinophils. With time the collagen becomes thickened and sclerotic with extension into the subcutaneous fibrous tissue

The tissue eosinophils may be focal around adnexal structures or diffuse within the tissue infiltrate In more extensively involved cases the changes can extend to the dermis The epidermis is typically not involved, though mild atrophy can be seen in a minority of cases

Tests done
Gamma globulins ESR MRI Skin biopsy:Full thickness

FASCIA TUMOURS

NODULAR FASCITIS
Common lesion that typically presents as a rapidly growing mass on the flexor forearm, chest, back or elsewhere Arises from superficial fascia, occasionally intramuscular or intravascular Similar lesions may also develop within the breasts, bladder,cervix, intra-articular, prostate, vagina and vulva

Epidemiology:Peaks at age 40 years; prior trauma in 10% of cases Benign behavior, but sometimes misdiagnosed as sarcoma based on cellularity, mitotic figures and rapid growth

Gross
Tan-white-gray, myxoid appearance, usually 3 cm or less (reconsider diagnosis if greater than 3 cm) Nodular with poorly defined margins May be centered in subcutis, may grow into skeletal muscle

Microscopy
Zonation effect with hypocellular central region and hypercellular periphery Composed of uniform, plump, immature, spindled to stellate fibroblasts or myofibroblasts without atypia, with a feathery, "tissue-culture" like growth pattern due to abundant ground substance Often with mucoid/myxoid pools (microcysts), a very useful diagnostic finding Uniform elongated nuclei with punctate nucleoli and without significant nuclear atypia Cellular areas may have storiform or fascicular patterns (S or C shaped)

 Often frequent mitotic figures (but no atypical forms), lymphocytes and macrophages, red blood cell extravasation, bands of keloid-type collagen Vasculature is usually prominent Walls of small to medium sized vessels are involved by reactive process at periphery of lesion May infiltrate adjacent fat May have metaplastic bone, focal cystic areas, ganglion type cells but no cells with large, hyperchromatic, atypical nuclei

Cranial fascitis
Nodular fascitis variant Infants or children < 2 years old Rare; usually boys Soft tissue of scalp and underlying cranial bones Develops from galea aponeurotica, erodes outer table of cranium and may infiltrate dura Some cases show dysregulation of Wnt/-catenin pathway, suggesting a potential relationship to desmoid fibromatosis rather than nodular fasciitis

May be related to birth trauma, craniectomy or be spontaneous Congenital in a minority of cases Painless Typically associated with focal calvarial lytic change at the lesional site Grows quickly like nodular fasciitis, but same benign behavior

Morphology
Gross:1-3 cm white to pink, mildly mucoid to rubbery mass,unencapsulated but well circumscribed Micro:Loosely arranged broad fascicles of delicate fusiform cells with edematous to myxoidappearing interstitium Nuclear atypia minimal or absent Mitotic figures ranged from 1-5 per 10 high power fields No compact cellularity, no short fascicles, no regional variations compared to nodular fasciitis

Radiography
Lytic defect of skull with sclerotic rim on xray Treatment: Surgical excision

Intravascular Fascitis
Variant of nodular fasciitis that involves wall and lumen of small to medium-sized veins and arteries Slower growth than classic nodular fasciitis but same behavior Uncommon Typically seen in 1st through 3rd decades, but can occur at any age Slightly more common in men

Most often found in upper extremity or head and neck region Less common sites include the lower extremity and trunk Etiology:Unclear Conservative excision

Morphology
Usually 2 cm or less Nodular or plexiform Resembles nodular fasciitis (proliferation of plump spindle cells in a loose "tissue culture" arrangement), but with a less prominent mucoid matrix and numerous osteoclast-like giant cells May be intra- or extravascular

Dupuytrens Contracture
Also known as palmar Nodular proliferative process of palmar aponeurosis, surrounding adipose and occasionally dermis, due to fibroblasts, myofibroblasts and fibrocytes Most common type of fibromatosis (1-2% of population), prevalence increases with age (24% at age 65+)

Puckers overlying skin as it ages Causes flexion contracture of digits 4 and 5 due to cord-like expansion of digital aponeurotic slips Does not involve deep structures such as tendons or skeletal muscle May be caused by fibrogenic cytokines

Morphology
Small nodules or nodular masses associated with aponeurosis and subcutaneous fat, with grayyellow-white cut surface (color depends on collagen content) Proliferative phase: Uniform, plump, immature spindle cells (myofibroblasts and fibroblasts) with bland nuclei and indistinct nucleoli Moderate collagen and elongated vessels

Older lesions: More dense collagen, less cellularity Variable mitotic figures Occasional attachment to dermis or cartilaginous metaplasia Usually no infiltration of surrounding tissue beyond subcutis

Plantar Fibromatosis
Nodular proliferative process of plantar aponeurosis and surrounding adipose due to fibroblasts, myofibroblasts and fibrocytes Heterogeneous group of conditions with plantar location, mature collagen and fibroblasts, but no malignant featuresCommon in boys < 10 years old and teenagers Associated with palmar and penile fibromatosis, also continuous phenobarbital treatment for epilepsy Usually NOT associated with contractures

Morphology
Gross: 2-3 cm nodules associated with aponeurosis and subcutis, with gray-yellowwhite cut surface (color depends on collagen content) Micro Proliferative phase: Hypercellular collection of uniform, plump, immature spindle cells with bland nuclei and indistinct nucleoli Moderate collagen and elongated vessels Variable multinucleated giant cells

Older lesions: Denser collagen, less cellularity Often prominent chronic inflammation, variable mitotic figures and hemosiderin

Penile fibromatosis
Also known as Peyronies disease Fibrous thickening of dermis and Bucks fascia between corpora cavernosa and tunica albuginea, causing curvature towards side of lesion and restricting movement of these structures during erection Etiology may be related to Parc protein or Wnt2

 Typically age 40+ years, rarely age 40 or less Various etiologies (microvascular trauma , urethritis, sclerosing inflammatory process, idiopathic), appears to differ from other superficial fibromatoses although associated with them Prevalence 3-9%, associated with plaques, pain, induration, deviation, palmar fibromatosis Usually dorsolateral penis, 30% have inflammatory component

Morphology
Disorganization of collagen of tunica albuginea with formation of nodules, often hyalinizing fibrosis, perivascular lymphocytic infiltrate in 1/3, linear band of calcification in 1/4