What is Morphea?
It is an inflammatory condition that leads to sclerotoic changes in the skin. The disorder is also called “localized scleroderma” since it restricts to one or more patchy areas of skin that hardens and undergoes pigmentation. However, it seldom evolves into scleroderma, which is a chronic systemic autoimmune condition of the connective tissue. As there is no involvement of the internal organs, the dermatological disease is also referred to as “circumscribed scleroderma”.
The localized skin problem is normally categorized into the following groups:
In this type of the disorder, the deep subcutaneous tissue, comprising of fibrous bands, collagen, elastin fibers and sweat glands, are severely affected. The condition worsens when sufferers begin to show symptoms of toxic oil syndrome, eosinophilia-myalgia syndrome, and eosinophilic fasciitis.
Morphea-lichen sclerosus et atrophicus overlap
The characteristic features of this type include, the white patches of Lichen sclerosus et atrophicus and painful lesions. In most cases, the condition affects females.
The sclerodermic condition is marked by formation of hard plaques on the skin as well as hypopigmentation. Progressive enlargement of the plaques, however, can be debilitating as it leads to muscle atrophy.
In this case, the scleroderma is localized and unilateral, particularly in young children. As the condition involves the bones and muscles, it could give rise to severe complications.
This particular form of the disorder involves the lower extremities in addition to hardening of the dermis, panniculus and fascia. Here, the underlying muscles are likely to get affected.
Atrophoderma of Pasini and Pierini
Excessive deposition of collagen can cause development of large sharp-bordered lesions with no evagination. However, this particular variant of circumscribed scleroderma is rare as it generally occurs in less than 200,000 Americans. Some of its other names include:
- Morphea plana atrophica
- Sclérodermie atrophique d’emblée
- Dynamic and atrophic variation of scleroderma
Frontal linear scleroderma/en coupde sabre/morphea en coup de sabre
This type is manifested by linear atrophy and skin furrows in the frontal or frontoparietal scalp.
In general, localized scleroderma is represented by small macules/plaques or streaks of hardened skin on the abdomen, chest, back, arms, legs, eyelids or forehead. Symmetrical colored patches are common in the groin, armpits, and breasts of some middle-aged women. In some instances, patients may develop painful nodules or lesions that have drop-like markings (guttate). As long as the hard patches are limited to the superficial layers, the disease is not fatal. Still, the severe forms of the condition, having visceral involvement, can cause functional impairment, such as joint immobility, and facial anomalies. In atrophoderma of Pasini and Pierini, oval patches of hardened and hyperpigmented skin appear on the upper and lower extremities. These round plaques usually have an ivory/yellow center, surrounded by a violet/purplish colored region. Their surface is often described as “smooth, hairless and shiny”. Localized scleroderma can have some extracutaneous manifestations in the form of seizures, dysphagia and arthralgia.
The exact etiology of the sclerotic condition is yet to be determined by physicians and medical researchers. Few key research studies have shown a strong link between scleroderma and immunocompromised disorders. Some of these are listed below:
- Systemic lupus erythematosus
- Primary biliary cirrhosis
- Lichen planus
- Lichen sclerosus
Surprisingly, radiation therapy can induce dermatological changes, including skin thickening and spontaneous inflammation. Although not common, in some patients the onset of the disorder could be related to tick bites, pregnancy, measles and chicken pox. Localized injuries and certain genetic factors have often been associated with the problem.
Injury to the endothelial cells can evoke overproduction of collagen in the skin. This is due to a sudden surge in the levels of adhesion molecules and fibrogenic T-helper 2 cytokines. Early appearance of the lesions can be attributed to the release of eosinophils, CD4+ T cells and macrophages. Excess collagen is a result of increased fibroblast proliferation. Presence of antimatrix metalloproteinase antibodies is another pathophysiological mechanism for this condition.
The aim of physical assessment of skin is to look for signs of hyperpigmentation and areas of thickened skin. The preliminary test is followed by a skin biopsy to identify the nature of the lesions. Plaque-related scleroderma should receive a deep punch biopsy for finding out the presence of subcutaneous fat. If deep layers of skin are involved then an incisional biopsy could prove to be more useful. Healthcare professionals may examine the blood for increased amounts of anti-histone and anti-topoisomerase 2a antibodies. The test is justified for patients who have a history of autoimmune disorders. Functional deformities, specifically of the bones and muscles, could be detected with the aid of radiography.
Morphea Differential Diagnosis
During diagnosis, it is mandatory for the examiners to exclude conditions that give rise to similar symptoms. Some of these include:
- White atrophy
- Secondary neoplasia
- Post-inflammatory hyperpigmentation
- Multiple sclerosis
- Basal cell carcinoma
Doctors usually find it difficult to treat the condition. Hence, the prime goal of the treatment is to control the inflammatory activity instead of clearing the well-developed sclerotic lesions. The inflammatory nodules can be reduced with topical, intra-lesional, and systemic corticosteroids. Topical tacrolimus and calcipotriene can be used for mild lesions. Use of immunomodulators like methotrexate and penicillamine can be beneficial for patients with autoimmune disorders. Ultraviolet A-1 light therapy is a potent method to treat the plaques. The UVA light is known to penetrate the deeper layers of the skin and cause systemic immunosuppression, which in turn releases enzymes for degeneration of collagen matrix. The following medications are often prescribed by physicians for improvement of the plaques:
- Oral retinoids
- Mycophenolate mofetil
The non-contagious condition may resolve after a certain period of time. Brown stains and depressed regions on the skin are common even after the elimination of the plaques. In case of muscle atrophy, the condition of the patients may deteriorate if proper treatment is not administered in time.
Take a look at some of the images of Morphea to know more about the condition.