Morph

What is the morph?

The morpho (American spelling, morphea) is characterized by an area of inflammation and fibrosis (thickening and hardening) of the skin due to increased collagen statement. Also known as located scleroderma. The term scleroderma covers various types of morph and systemic sclerosis.

The morpho subtypes vary depending on the location of the affected skin. Any morpho subtype can also cause deep or subdermal involvement of the underlying fat, fascia, muscle or bone.

Unlike systemic sclerosis, morph does not cause:

• Thickening of the skin of the fingers and toes (sclerodactyly)
• Specific autoantibodies in the blood (as an anti-centromere antibody or anti-Scl70)
• Abnormal small blood vessels on the fingers (nail fold capillaries)
• Fibrosis and / or vascular Damage to internal organs.

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Who gets the morph?

The morph is rare and is estimated to have a incidence 1 to 3 per 100,000 children. It is three times more common in women than men and often begins in childhood. But not hereditarytrue HLA The subtypes (HLA-DRB1 * 04: 04 and HLA-B * 37) are associated with an increased risk of morpho.

What is the cause of the morph?

The precise cause of the morph is unknown.

• Located genetic factors seem to play a role; for example, cutaneous mosaicism can be important in linear morpho, which follows Blaschko lines of epidermal developing.
• Up to 40% of patients with severe morpho forms have a personal or family history of autoimmune disease (eg, thyroid disease, vitiligo) or rheumatologic disease (eg, rheumatoid arthritis)

For unknown reasons, the morph often develops after an external trigger such as:

• Bug bite or tick bite (the role of Borellia burgdorferi, cause of Lyme disease is controversial)
• Injection (eg, Bleomycin, silicone) or vaccination
• Repeated friction
• Surgery
• Radiotherapy
• Penetrating wound
• Extreme exercise
• Repeated minor friction along the waist, bra strap, and inguinal region in isomorphic disseminated license plate morph

TraumaMorpho-related may occur at the affected site, or at distant unrelated sites.

What are the subtypes and clinical characteristics of the morph?

When describing the morph, consider three characteristics.

• Anatomical distribution: location and if it is limited, linear, generalized or mixed
• Morphology: inflammatory, sclerotic, depigmented or atrophic
• Tissue involvement depth: skin, fat, fascia, muscle, bone.

Anatomical distribution

Morpho subtypes are limited, linear, generalized, or mixed.

Limited plate morph

• The most common type in adults.
• Oval shaped patches from 1 to 20 cm in diameter.
• Two or fewer body sites.

Rare types of limited plaque morpho include guttata morpho and atrophoderma Pasini and Pierini.

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Linear morph

• Most common subtype in children
• It is found in the extremities, trunk or head (craniofacial)
• Follow lines and eddies called Blaschko lines
• Usually unilateral, but maybe bilateral and extended in severe cases

Linear moulin atrophoderma is a rare subtype of atrophy and pigmented linear morph.

The craniofacial linear morph was previously subclassified as:

• En coup de saber (linear atrophic band +/- sclerosis, classically on the forehead or scalp)
• Parry Romberg / progressive hemifacial atrophy (atrophy of fat, muscle and bone +/- sclerotic changes +/- dyspigmented, affecting one side of the face).

These types of craniofacial linear morpho occur together and vary according to morphology and depth of tissue involvement.

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Generalized morph

Generalized morph affects three or more sites on the body. There are two main patterns;

• Scattered plaque morphea: scattered plates with unaffected skin involved. It can be isomorphic (occurring at friction sites such as the waistband, bra line, and inguinal folds) or non-isomorphic (scattered plaques anywhere).
• Panclerotic morph: circumferential, confluent sclerosis, which is generally rapidly progressive and affects most of the body surface area. This occurs in infancy (disables childhood panclerotic morpho) and adulthood (when overlapping with eosinophilic fasciitis)
• Generalized morph saves digits and periareolar skin.

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Mixed pattern morph

• Mixed pattern morph occurs when more than one anatomic subtype is present.
• The most common mixed pattern is linear limb morph, with limited plaque morph on the trunk.

Limited morph is considered mild, while linear morph and generalized morph are more severe subtypes. The depth of tissue involvement is also important when evaluating disease severity; Subdermal involvement implies a more serious disease.

Morphology

The specific appearance of the morph varies and may include:

• An inflammatory phase: pink, purple, or bruised patches
• Sclerosis: The skin becomes hard, thickened, bound, shiny, ivory white, and may have a purple border of surrounding inflammation
• Dyspigmentation and atrophy. This develops after a variable period of time (usually months or years); hyperpigmentation is more common than hypopigmentation. Shallow (dermal) atrophy is seen as shiny skin, often with visible blood vessels. A deeper atrophy involving fat, muscle, or bone occurs as concave indentations or discrepancies in the circumference of the limb.

While the morph classically passes through each of these morphologic stages, this is not always the case.

Deep tissue compromise on morph

Any morpho subtype can affect connective tissue under the skin, such as fat, fascia, muscles, bones, joints, or, rarely, the brain (in the craniofacial linear morph). Deep tissue involvement is a marker of serious disease.

When the fascia, the connective tissue underneath the fat, is involved:

• The skin may look wrinkled, with a cobblestone or orange peel appearance.
• There may be gutters along the blood vessels (the groove sign)

Bone or joint involvement results in loss of bone tissue, pain, and / or restricted joint movement (contractures)

Eosinophilic fasciitis is a form of panclerotic morph with deep fascial involvement. Circumferential inflammation and sclerosis of the fascia (the connective tissue under the fat) frequently coexist with overlying dermal sclerosis or other anatomical morpho subtypes, indicating that it is debatable whether a separate condition should be considered.

The symptoms of morpho

The morph can be asymptomatic, or symptoms of skin or deeper tissues may arise, or may be due to extracutaneous manifestations

Symptoms due to changes in the skin.

• Some patients experience itching and / or pain from actively inflamed morpho areas.
• Annexed The structures are destroyed by sclerosis. This is of particular relevance to the linear morph of the scalp, resulting in permanent hair lost.
• Sclerosis can catch superficially nerves and cause pain, tingling, and sometimes mild weakness.

Symptoms due to a deeper involvement of the tissue.

• Deep involvement on the joints causes joint pain or limited joint movement (contractures).
• Constriction of the chest wall in the generalized panclerotic morph can cause difficulty in breathing.
• The involvement of the teeth and jaw in the craniofacial linear morpho can cause oral and dental problems. Temporomandibular joint involvement causes difficulty chewing, jaw blockage, or pain.
• Involvement of the skull and / or brain can cause headaches or, in some cases, neurological symptoms such as nervous paralysis or seizures.

Systemic symptoms

Up to 30% in patients with more severe types of linear or generalized morph may present with non-specific extracutaneous inflammatory symptoms. These include:

• Fatigue, lethargy
• Nonspecific joint pain and / or inflammation (arthralgia, arthritis)
• Muscle pain
• Reflux / heartburn
• Raynaud's phenomenon (cold hands with red / white / blue color changes)
• Dry eyes, irritation, or blurred vision due to ocular implication (most commonly episcleritis, previous uveitis, keratitis) - related or not related to the morpho site

These extracutaneous manifestations imply that morpho is a systemic inflammatory condition. By contrast, systemic sclerosis causes direct damage and fibrosis of the lungs, heart, kidneys, and / or the gastrointestinal tract, which does not occur in the morpho.

How is morpho diagnosed?

The diagnosis of morpho is often made clinically, without the need for further testing. BiopsyBlood tests and imaging may be done if the diagnosis or extent of the disease is unclear.

Skin biopsy

A skin biopsy allows the skin, subcutaneous fat (and sometimes fascia) to be examined under a microscope. Classic findings on the morph include:

• Sharply square biopsy ("cookie cutter" sign)
• Atrophic epidermis
• Thickened, hyalinized collagen, with possible loss of appendicular structures (such as sweat glands and hair follicles)
• Fat loss / entrapment
• Inflammatory cell infiltrate (lymphocytes +/- plasma cells).

Blood test

• Eosinophils count (can be increased)
• Inflammatory markers (ESR or CRP can be elevated)
• Anti-nuclear antibody (may be elevated, but ENA specific systemic sclerosis antibodies as anti-Sc70 are negative)
• Thyroid function tests (may be abnormal; consider risk of associated autoimmune thyroid disease)
• Rheumatoid factor or anti-CCP antibodies if arthralgia / arthritis (may be elevated)

Image

Magnetic resonance imaging (Magnetic resonance) can be performed to assess how deep the morph extends beyond the skin, for example in generalized morph, linear joint-crossing morph, and craniofacial linear morph.

How is morph treated?

There is no cure for morph. Treatment is aimed at stopping the activity and progression of the ongoing disease.

Current therapy

Topical therapy can help limited and superficial forms of morph and can reduce itching.

• Topical steroids
• Tacrolimus ointment
• Calcipotriol ointment or cream
• Imiquimod cream

Phototherapy

Phototherapy can soften morph and has anti-inflammatory effects

Different wavelengths of Ultraviolet radiation can be used.

• UVA1 penetrates more deeply and is therefore useful when deeper subcutaneous tissues are involved, but is rarely available.

• Topical or systemic photochemotherapy (PUVA, i.e. psoralen + UVA) may be helpful.

• The narrow UVB band is less effective since it only penetrates superficially.

Systemic treatment

Systemic treatment is intended to prevent progression and deactivate the active process of the disease. Usually needed for at least 2 to 4 years, but relapse it can happen.

• Methotrexate

• Systemic corticosteroids

• Mycophenolate mofetil

Specific treatment decisions in the morph are guided by the morph subtype and its severity.

Superficial limited morphia - mild

1. Topical therapy

2. Phototherapy (UVA1, nbUVB, PUVA)

Generalized or onset superficial linear morpho in adults: moderate

1. Topical therapies and phototherapy.
2. Systemic therapy

Deep morpho forms and pediatric appearance of linear morpho - severe

1. Methotrexate +/- systemic corticosteroids (pulsed IV and / or oral)

2. Mycophenolate mofetil +/- corticosteroids (IV pulsed and / or oral)

3. Combined therapies

• Combine 1^{S t} and 2^{North Dakota} online treatments
• Add hydroxychloroquine
• Add phototherapy (UVA1 or PUVA if available)
• Other options

• Cyclosporine

• Abatacept

• Tocilizumab
• Extracorporeal photopheresis
• Other experimental and informed therapies

Physical therapy to improve joint mobility should be done with caution in the active morph, as the resulting trauma can potentially act as a continuous trigger for the disease.

In some cases, surgery may be beneficial, such as autologous fat transfer, to improve atrophy.

Monitoring response to treatment

Disease progression and response to treatment can be monitored using photographs, the localized scleroderma skin assessment tool (LoSCAT), and other highly specialized tests, such as infrared thermography.

Localized scleroderma skin assessment tool

LoSCAT is a validated tool used to assess disease activity and damage over time. Evaluates 16 body sites and includes two LS domains.

• The modified localized scleroderma severity index (mLoSSI), which measures disease activity
• The Localized Scleroderma Damage Index (LoSDI), which measures damage.

Score for mLoSSI

1. Erythema0: no erythema; 1: slight erythema / pink; 2: red / clearly erythema; and 3: dark red or marked erythema /violaceous.
2. Skin thickness: 0: normal and freely mobile skin thickness; 1: slight increase in thickness, mobile; 2: moderate increase in thickness, impaired skin mobility; 3: marked increase in thickness or lack of mobility of the skin.
3. New injury/ extent of injury: development of new injury and / or enlargement of an existing injury in the last month (score of 3 if present).

LoSDI score

1. Dermal atrophy: 0: normal skin; 1: mild skin atrophy, that is, shiny skin; 2: moderate atrophy, that is, visible blood vessels; and 3: severe skin atrophy, that is, an obvious sign of "falling cliffs."
2. Subcutaneous atrophy: 0: normal subcutaneous thickness; 1: flattening; 2: obvious concave surface; and 3: severe loss of subcutaneous fat (> 2/3 loss).
3. Dyspigmentation: assess both hyper or hypopigmentation, whichever is more prominent: 0: normal skin pigment; 1: mild; 2: moderate; and 3: severe dyspigmentation.

What is the natural history of the morpho?

The morpho can follow a long course, which can be recurrent and remitting, or chronically active. The milder forms of the disease tend to become inactive in 3 to 5 years.

Relapse can occur after successful treatment, especially in morph that begins in childhood.

Extended courses of systemic treatments of 4 to 5 years or more may be required to minimize the risk of relapse.