The typical presentation of individuals with morphea includes a single or a plethora of inflammatory/sclerotic plaques. Though disease activity is usually limited after an average period of five years, there are some cases of patients with more persistent episodes or a condition with relapses. Aesthetic disfigurement and/or damage that leads to a certain loss of functionality are common consequences after active disease has subsided.

Hardened skin is one of the cardinal signs of morphea. The formed lesions are round, extended patches with a subtle pink/yellow appearance, along with a purplish rim. With the progression of the disease, the affected area becomes sleek, shiny and assumes and can be mistaken for a scar.

One report of the first stages of a morphea case described the dermatologic symptoms of the patient as resembling a case of poikilodermatous mycosis fungoides (MF). Histopathological and molecular analysis also produced findings compatible with MF. However, as the condition progressed, well-defined lesions and histopathological findings indicated the presence of morphea.

• Disability

  We present the case of a 4-year-old boy who presented with features of disabling pansclerotic morphea and eosinophilic fasciitis simultaneously, which to our knowledge has not been previously reported. [ncbi.nlm.nih.gov]

  However, it can affect a patient's life quality, especially in the case of pediatric patients, because of the possible disfigurement and disability; disability is mostly caused by the involvement of the subcutaneous regions in the sclerodermic areas. [symptoma.com]

  Childhood onset affect the face and slow progress to disability. [e-ijd.org]

• Amyloidosis

  Systemic sclerosis (scleroderma) Stasis dermatitis with fibrosis / lipodermatosclerosis Lipoatrophy Amyloidosis Porphyria cutanea tarda with sclerodermoid features Erythema nodosum Scleredema Scleromyxedema Drug / chemical-induced sclerodermoid lesions [visualdx.com]

  […] erythematosus panniculitis Linear melorheostosis Lipodermatosclerosis Morpheaform dermatofibrosarcoma protuberans Muckle-Wells syndrome Nephrogenic fibrosing dermopathy Niemann-Pick disease Phenylketonuria POEMS syndrome Porphyria cutanea tarda Primary systemic amyloidosis [wikidoc.org]

  Unusual milia amyloidosis as initial signs of multiple myeloma-associated systemic amyloidosis. International Journal of Dermatology. 2013; 52:981-982. HANAMI, Y*; YAMAMOTO, T*. Secondary amyloid deposition in a melanocytic naevus. [fmu.ac.jp]

• Fishing

  When you begin to feel better, resume your normal diet gradually, starting with rice, crackers and toast, cooked potatoes and carrots, then on to broiled skinless chicken and fish. [articles.chicagotribune.com]

  Muncho Lake Provincial Park offers excellent fishing and camping spot along the long, jade-green lake, while the hot springs at Liard River are without question one of the most popular stops on the Alaska Highway. [hellobc.com]

  During winter months ice fishing becomes the sport of choice. [en.wikipedia.org]

  Given that the chicken-dipper and fish-finger fragments don’t actually contribute anything to the petro-brew, why not just buy cooking oil straight from the shelves? “You could, no problem,” comes the reply. “The oil would work just as well. [telegraph.co.uk]

• Localized Scleroderma

  Results IgG or IgM anti–topo IIα antibody was detected in 76% (35 of 46) of patients with localized scleroderma, and in 85% (11 of 13) of patients with generalized morphea, the severest form of localized scleroderma. [doi.org]

  Localized scleroderma. Curr Opin Rheumatol. 2006 Nov. 18(6):606-13. [Medline]. Zulian F. Systemic manifestations in localized scleroderma. Curr Rheumatol Rep. 2004 Dec. 6(6):417-24. [Medline]. Chung L, Lin J, Furst DE, Fiorentino D. [emedicine.com]

  Despite many reports of neurological involvement in patients with the craniofacial linear localized scleroderma, it is extremely rare in patients with the other subtypes of localized scleroderma. [ncbi.nlm.nih.gov]

  Synonym(s): localized scleroderma, morphoea. [G. morphē, form, figure] Morphea The most common form of localized scleroderma. morphea [medical-dictionary.thefreedictionary.com]

• Sweating

  Skin biopsy showed rectified epidermis and mild hyperorthokeratosis, reticular dermis with perivascular and periadnexal infiltrates of lymphocytes and mononuclear cells, and reticular dermis and sweat gland sclerosis surrounded by a dense collagen tissue [ncbi.nlm.nih.gov]

  The plaques may lose their hairs and sweat glands and the skin can look and feel dry. Sometimes morphea can occur in a line and usually a!ects a limb in a unilateral fashion. This is known as linear morphea. When a whole limb is a! [sruk.co.uk]

  […] problems, and dysregulation of collagen production Clinical manifestation Poorly defined areas of nonpitting edema, with sclerosis developing as disease progresses; skin surface becomes smooth and shiny, with loss of hair follicles and decreased ability to sweat [link.springer.com]

  Lesions are usually asymmetric and can be associated with alopecia and decreased sweat production. Concomitant genital lichen sclerosus may be present in up to 40% of patients with plaque-type morphea. [visualdx.com]

• Hyperpigmentation

  Physical examination revealed several irregularly shaped, firm nodules with hyperpigmentation and induration on the trunk and upper arms (Panel A). [nejm.org]

  Mineral sun protection, either with mineral-only formulations of sunscreen or with those that combine mineral and chemical sun filters, is emphasized in those with morphea because it will best prevent hyperpigmentation in areas of active inflammation. [sherberandrad.com]

  […] phenomenon, Borrelia burgdorferi infection Clinical features Usually presents on trunk or extremities as one or several indurated plaques with ivory center and violaceous border (the "lilac ring") Also lesions confined to breast May have irregular areas of hyperpigmentation [pathologyoutlines.com]

  Some of these include: White atrophy Secondary neoplasia Post-inflammatory hyperpigmentation Multiple sclerosis Basal cell carcinoma Morphea Treatment Doctors usually find it difficult to treat the condition. [patienthelp.org]

• Alopecia

  Lesions are usually asymmetric and can be associated with alopecia and decreased sweat production. Concomitant genital lichen sclerosus may be present in up to 40% of patients with plaque-type morphea. [visualdx.com]

  Plaque type morphea that mimicking systemic lupus erythematosus in clinical appearance, such as alopecia and oral mucosal ulcers, is uncommon. A case of plaque type morphea mimicking systemic lupus erythematosus in a 20 year old woman was discussed. [ncbi.nlm.nih.gov]

  […] greater frequency in those with a personal or family history of autoimmune disorders Sometimes associated with other diagnosis, such as arthralgias, carpal tunnel syndrome, Raynaud phenomenon, spina bifida, lichen planus, lichen sclerosis, vitiligo, and alopecia [unboundmedicine.com]

  Associated skin diseases include alopecia areata, vitiligo, dystrophy of the nails, and ichthyosis. [sclero.org]

• Skin Discoloration

  Morphea is a localized or limited form of scleroderma, a condition that can cause a wide variety of problems, from skin discoloration to difficulty with the function of joints and muscles and other connective tissues. [fortishealthcare.com]

  Widespread areas of hardened, discolored skin. Numerous new patches of hard, discolored skin may seem to join together, a condition known as generalized morphea. Eye damage. [drugs.com]

  Morphea is a rare pathological condition of the skin, which is characterized by development of discolored patches on your skin. These patches are not painful and typically appear on the abdomen, trunk, chest and back. [epainassist.com]

• Seizure

  We report the case of a young girl who presented with hemiparesis, seizures, and subtle features consistent with a linear form of facial morphea (en coup de sabre). [ncbi.nlm.nih.gov]

  Linear morphea on the head (also called en coup de sabre ) may extend inwards to the brain and cause seizures. If morphea crosses a joint, the thickening may limit joint movement. Morphea tends to have a waxing and waning course. [archderm.jamanetwork.com]

  Linear morphea involving the scalp and face may develop seizures and neurologic problems. Seizures and neurological deficits may also complicate progressive facial hemiatrophy. [rheumaderm-society.org]

  Progressive facial hemiatrophy, also known as Parry-Romberg syndrome, is characterized by minimal cutaneous changes with significant unilateral atrophy of the underlying tissue of the face, often with underlying abnormalities and increased risk of seizures [visualdx.com]

Diagnosis of morphea is usually based on clinical data. A biopsy sample from the affected area may aid to establish a definitive diagnosis. Typical findings include irregular deposition of collagen, massive vascular walls and white cell infiltration at the border of the vessels. Laboratory tests are usually unremarkable.

• Borrelia Burgdorferi

  We present a case of a 16-year-old boy with morphea caused by Borrelia burgdorferi. We re-emphasise an immunohistochemical method, focus floating microscopy (FFM), to detect Borrelia burgdorferi spirochetes in tissue sections. [ncbi.nlm.nih.gov]

  Borrelia burgdorferi as causative agent has been discussed controversially. OBJECTIVES: To assess the evidence for infection with B. burgdorferi in patients with morphoea by focus-floating microscopy (FFM). [lookingatlyme.blogspot.com]

  Borrelia burgdorferi today is a possible etiologic factor [ 6 ]. [omicsonline.org]

  Definition / general Either localized (morphea) or systemic (involving skin of face, upper trunk, hands and arms, esophagus, heart and lungs) May have visceral disease without skin disease May be associated with Raynaud's phenomenon, Borrelia burgdorferi [pathologyoutlines.com]

• Toxoplasma Gondii

  We present a case of a male patient, which developed morphea lesions during the infection with Toxoplasma gondii. [sclero.org]

As discussed earlier, there is no known effective cure for morphea. Nevertheless, a wide range of therapeutic measures are available, which can be used supportively to reduce the profound symptomatology. Drugs usually help to avoid organ injuries and lessen joint pain. Other possibilities include surgical laser intervention and light therapy, which can be attempted to aesthetically correct the appearance of the skin. When joints are affected, patients can also benefit from physiotherapy, although a little degree of joint freezing is inevitable.

In cases with advanced stage, surgical intervention might be reserved for the treatment of extensively injured organs, such as a stenosed esophagus or a gangrenous finger due to Raynaud's syndrome.

Treatment aiming at the reduction of inflammation at the initial stages of morphea has shown better results than measures applied to diminish scars at a later stage. In cases where the disease has not yet reached its final phase, local application of some balms can fight the inflammation and symptoms: creams containing corticosteroids, tacrolimus ointment such as Protopic and imiquimod cream (Aldara) are all acceptable choices. UVA phototherapy can also be beneficial.

Several new possibilities for the supportive care have recently emerged. Combinations of anti-inflammatory medications, immunomodulators or anti-fibrotic drugs have been tried. IFN-γ targets the overproduction of collagen both directly and indirectly, while stimulating the Th1 response type. Additionally, IFN-γ has beneficial effects on fibroblast reproduction, that can lead to the development of normal fibroblasts. IFN-γ could also be applied locally, because it has the capability of constructing an effective protein delivery structure and can also transport the active ingredients to the diseased tissue with no adverse effects of systemic nature.

Patients should also be advised to adapt their lifestyles accordingly: physical activity, a healthy diet, stress reduction and the protection of the skin from excessive cold or sun, may all offer important improvement of the condition. Smoking should also be avoided as it can cause considerable vascular injuries, which can worsen scleroderma.

The prognosis of morphea is usually good, as it seldom leads to death. However, it can affect a patient's life quality, especially in the case of pediatric patients, because of the possible disfigurement and disability; disability is mostly caused by the involvement of the subcutaneous regions in the sclerodermic areas. Linear lesions continue to exist for longer periods of time than plaque lesions.

Morphea is a disease characterized by chronicity and its etiology is unclear to this day. It has been identified that the primary alterations involve collagen and extra-cellular matrix components, which are produced at a higher-than-normal rate and are increasingly deposited on the skin. In some occasions, morphea can extend as far as the subcutaneous tissue. It leads to disfigurement and a certain level of disability, which adversely affects the quality of life. It is rarely life threatening.

Although the pathogenetic background of localized scleroderma is unknown, it is potentially associated with borreliosis.

Morphea is a relatively rare disease, that is usually diagnosed in the age of childhood or early adulthood [4] [5] [6] [7]. Its incidence has been calculated to amount to 3 per 100,000 population per year in the USA [8].

Morphea is found more often in children than in adults. Although morphea can affect individuals of any age, almost half of the patients diagnosed with it develop the disease in their childhood years [7] [8] [9]. A study that assessed patients diagnosed in the adult life and patients with early onset disease, reported that the disease was diagnosed approximately at 45 years and 10 years, respectively [6].

Morphea exhibits a predilection for female patients, as they seem to be affected by it four times more frequently than male patients do. Other studies have estimated the ratio of men to women to be around 1:2.6 [8]

As localized scleroderma starts to develop, a great amount of mononuclear cells are influxed into the skin and adjacent blood vessels. This phenomenon has not yet been etiologically defined yet. Several studies indicate that localized scleroderma is characterized by autoimmunity. Histopathological analysis has revealed activated T-lymphocytes among the mononuclear cells, which also supports the theory of an autoimmune factor [10]. Other potential etiologic factors comprise genetic predisposition and environmental triggers [11] [12].

Following the penetration of the mononuclear cells, the skin is subject to various alterations. Small vessels underneath the epidermis are profoundly damaged, hypoperfused and exhibit considerable damage of the endothelium [13].

The endothelium displays subsequent inflammation, with a typical up-regulation of adhesion molecules, including the intercellular adhesion molecule 1 (ICAM-1) and vascular cell adhesion molecule 1 (VCAM-1). This occurs due to the activation of mediators and cytokines, for example IFN-γ, IL-1 and TNFs [14]. A particular adhesion molecule, E-selectin, has been found in an considerable number of patients suffering from morphea [15]: it influences the degree of mononuclear cells invasion. Furthermore, its presence seems to lead to an escalated number of sclerotic lesions [15]. With a reference to ICAM-1 and VCAM-1, they mediate the attachment of monocytes to the endothelial wall. ICAM-1 displays an elevation of 25% in patients with localized scleroderma and VCAM-1 an elevation of 19% [16].

All these steps lead to hardened skin, overproduction and deposition of collagen. This is the final phase of this condition, when most of the lesions are formed. It is also the phase most targeted by means of therapeutic measures.

Fibroblasts are responsible for collagenic synthesis. In morphea, they increase the rate of collagen production, possibly due to infection, damaged cells or an autoimmune process.

There are no specific measures to prevent scleroderma, as the etiology of the condition remains unknown. Silica dust and paint thinners have been incriminated for the triggering of morphea and their avoidance is believed to prevent the development of the disease to a certain extent.

Morphea is a disease that belongs to the wider category of scleroderma. In general, scleroderma has two types: the systemic and localized one, which can be divided into other categories as well [1].

Systemic scleroderma itself features two further categories, limited systemic sclerosis and diffuse systemic sclerosis. The diffuse type involves clinically observable lesions on the thorax, face, distal and proximal extremities, whereas the limited type is restricted to the face and arms or hands. Systemic symptoms that accompany both types include Raynaud's phenomenon or the participation of internal organs in the inflammatory process.

Localized scleroderma, on the other hand, solely affects the skin and no other organs. Morphea is one of the categories of localized scleroderma [2] [3]; it is an autoimmune disease, that induces the formation of sclerotic lesions on the skin. It causes aesthetic impairment due to the sclerotic skin and disability of varying degrees, particularly in the cases where the subcutaneous regions are involved as well.

Morphea is an autoimmune disease that affects the skin and, in some cases, the subcutaneous tissue. The disease leads to the formation of hardened lesions on various sites of the skin; this is caused by an abnormally increased production of collagen and several other substances, which are then transported and laid down on the skin.

It is not known what causes morphea, otherwise known as scleroderma. The theory of autoimmunity, presently linked to morphea, stipulates that in some cases, the organism starts to recognize parts of the body as foreign and attacks them.

Morphea, on the other hand, does not lead to life-threatening complications and is usually limited to the skin, accompanied by some phenomena which indicate a systemic involvement. A symptoms that is usually present in the patients is Raynaud's phenomenon: fingers turn white, blue and red when they are exposed to a cold temperature. Lastly, there is no really effective treatment for scleroderma: most creams, ointments, drugs and light therapies used, aim at the alleviation of the symptoms. The disease frequently stops being active after 3 to six years.

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