Morphea - Types, Causes, Symptoms, Diagnosis, and Treatment

Introduction

Morphea, or localized scleroderma, is a rare fibrous disease characterized by inflamed fibrous skin due to increased collagen deposition. As a result, normal skin texture is lost and shows a shiny appearance. Morphea can affect the skin, subcutaneous fat, deep fascia, underlying muscle, and bone. It is observed that females are affected more than males. Morphea is estimated with a high incidence rate among children.

What Is Morphea?

Morphea is a rare fibrous skin disorder affecting females and children. Morphea is characterized by increased collagen deposition leading to inflammation, thickening, and skin hardening. The exact cause of morphea is unknown. However, localized genetic factors and other external triggers could contribute to morphea. Characteristic features of morphea include bruise patches, thick skin, hyperpigmentation, shiny skin, and blood vessels; morphea is subdivided into various subtypes based on its anatomical distribution, morphology, and depth of tissue involvement.

What Are the Types of Morphea?

According to Zulien et al. classification, morphea is classified into the following types:

• Circumscribed morphea.

• Linear morphea.

• Generalized morphea.

• Pansclerotic morphea.

• Mixed morphea.

Mentioned below are the types of morphea,

1. Limited Morphea - Circumscribed morphea is present in one or more than one site of the body. It is asymptomatic, and oval-shaped patches are present.

Variants of circumscribed morphea include guttate morphea and atrophoderma (thinning of the dermis) of Pasini and Pierini.

2. Linear Morphea - Linear morphea is commonly seen in children. Features are widely seen in limbs, the trunk, and the craniofacial region. Mild forms are usually unilateral; in severe cases, it is bilateral and spread widely. Linear morphea includes linear atrophoderma of the moulin (an uncommon skin disorder marked by atrophic, hyperpigmented, unilateral cutaneous lesions that resemble bands), en coup de sabre (a localized form of scleroderma called linear morphea that is limited to the frontoparietal area (forehead)), Parry Romberg, or progressive hemifacial atrophy (a gradual decline in the soft tissues and skin on a particular side of the face).

3. Generalized Morphea - Features of this type of morphea are present in three or more body sites. Plaques are present in a disseminated pattern.

4. Pan Sclerotic Morphea - Pan sclerotic morphea is usually progressive with circumferential sclerosis. It affects most parts of the body. Pan sclerotic morphea is common in children and is associated with eosinophilic fasciitis in adults.

5. Mixed Morphea - This is the combination of an individual's linear and limited morphea.

While linear morphea and generalized morphea are more severe varieties, limited morphea is regarded as minor. When determining the severity of an illness, the depth of tissue infiltration is also crucial; subdermal involvement suggests a more serious condition.

What Are the Clinical Features of Morphea?

Clinical features of morphea are present with inflammation, sclerosis, hyperpigmentation, atrophy, and deep-tissue involvement. In the phase of inflammation, bruise-like patches are present. The skin is thick and shiny. Over some time, usually months or years, hyperpigmentation is seen. Dermal atrophy causes the blood vessels to be visible, and profound atrophy affects the muscles, fat, and bone. Deep tissue involvement is observed in the severe form of morphea.

Mentioned below are the clinical features of morphea,

• Pink, bruise patches.

• Thick and fibrous skin.

• Shiny skin.

• The color is ivory white.

• A purple border surrounds the inflammation.

• Hyperpigmentation and the visible blood vessels.

• Deep tissue involvement.

• In fascia: Puckered skin with orange peel appearance.

• In bone: Pain, bone tissue loss, and contractures.

What Are the Symptoms of Morphea?

Most mild cases of morphea do not generate any symptoms. Involvement of deep tissues results in symptoms. Fatigue, lethargy, arthritis, arthralgia, muscle soreness, heartburn, eye irritation, blurred vision, episcleritis, and keratitis are among the systemic symptoms associated with the severe type of morphea.

Mentioned below are the signs and symptoms of morphea,

• Itching.

• Pain.

• Permanent hair loss.

• Tingling, pain, and mild weakness (superficial nerve sclerosis).

• Joint pain.

• Contractures (limited joint movement).

• Shortness of breath.

• Difficulty in chewing.

• Jaw pain.

• Headaches, nerve palsy, and seizures in skull and brain involvement.

What Are the Causes of Morphea?

The exact cause of morphea is not known. However, it is found that certain genetic factors and autoimmune diseases can cause morphea. Severe forms of morphea are familial cases. Morphea seems to occur after external factors.

The external triggers of morphea are,

• Repeated friction, which also includes minor friction along the waistband and inguinal area.

• Injection site.

• Vaccination site.

• Bite of an insect.

• Surgery.

• Radiotherapy.

• Trauma.

• Deep wound.

What Are the Risk Factors of Morphea or Scleroderma?

The following variables may influence the probability of experiencing morphea:

• White ladies are more likely to have morphea.

• People of any age can be impacted by the illness. It typically manifests in the mid-40s or anywhere between the years of two and 14.

• An illness like this may run in families. There is a higher likelihood that morphea and other autoimmune illnesses run in families among those who have the condition.

How to Diagnose Morphea?

Morphea is diagnosed with personal history and clinical examination. The extent of the disease is determined by biopsy, blood tests, and imaging. Histopathological findings include atrophy in the epidermis, thick collagen, loss of sweat glands, hair follicles loss, loss of subcutaneous fat, and infiltration of lymphocytes and plasma cells. A blood test is also done. Magnetic resonance imaging or MRI is done in generalized morphea.

Diagnostic features of morphea include,

• Proper history.

• Physical examination.

• Skin biopsy.

• Histopathological features:

  • Atrophy in the epidermis.

  • Thyalinised collagen.

  • Loss of sweat glands.

  • Loss of hair follicles and fat.

  • Inflammatory cell infiltration: Lymphocytic and plasma cell infiltration.

• Hematological findings:

  • Increased eosinophil count.

  • Erythrocyte sedimentation rate or ESR.

  • High C-reactive protein or CRP.

  • Thyroid function tests are abnormal.

  • Rheumatoid factor.

• Magnetic resonance imaging or MRI - To determine how far the morphea spreads below the skin, magnetic resonance imaging (MRI) may be used. This is particularly useful in cases of generalized morphea and craniofacial linear morphea.

What Is the Treatment of Morphea?

Treatment is done to reduce the progression of morphea. Various treatment modes are involved: topical therapy, systemic therapy, phototherapy, physiotherapy, and surgery in severe cases of morphea. Treatment photographs can monitor treatment effects, LoSCAT, localized scleroderma cutaneous assessment tool, and infrared thermography.

Various modes of treatment for the treatment of morphea are given below.

Topical Therapy: Topical steroids, ointment, or cream for relieving itching sensation, such as Tacrolimus and Calcipotriol imiquimod cream.

Phototherapy: Phototherapy has anti-inflammatory properties and can help soften morphea. It is possible to employ ultraviolet radiation at various wavelengths.

• Although UVA1 is rarely available, it is effective when deep tissue layers are involved because it penetrates the deepest.

• It might be beneficial to use systemic or topical photochemotherapy (PUVA or psoralen + UVA).

• Since narrowband UVB barely penetrates the surface, it is less effective.

Systemic Treatment: The goal of systemic treatment is to halt the disease's progression and turn off its active mechanism. A minimum of two to four years is often required, though relapses can happen.

• Systemic corticosteroids.

• Methotrexate.

• Mycophenolate mofetil.

• Abatacept.

• Ciclosporin.

Physical Rehabilitation. Physical rehabilitation may help patients maintain their range of motion if the condition impairs their joints.

Surgery: Surgery, which includes autologous fat transfer, may be helpful in some circumstances to treat atrophy.

What Are the Complications of Morphea?

Complications that may arise from Morphea include the following:

• Individuals with morphea may develop negative effects on their physical image and self-worth. This occurs particularly in people with pigmented patches on their faces, arms, and legs.

• The range of motion of the joints could be impaired if the condition occurs in the legs or arms.

• Multiple newly formed areas of hard, discolored skin tend to fuse to form a condition known as generalized morphea.

• Sometimes, individuals with this condition might get hair loss and sweat gland loss in the affected area.

• Eye damage may also occur in children experiencing head and neck morphea.

Conclusion

Morphea, known as localized scleroderma, is a rare skin and subcutaneous disorder. Morphea is most commonly reported in children and women. The benign form of morphea is a self-limiting disorder. However, relapse and extracutaneous complications will increase the risk of morbidity. Long-term treatment is advised to reduce the disease's symptoms and progression. Mycophenolate mofetil and Abatacept give patients promising effects, thereby reducing the risk of morbidity.