Version July 2025
INTRODUCTION —
Morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes in the skin. Morphea is distinct from systemic sclerosis (scleroderma), an autoimmune connective tissue disorder characterized by acral or diffuse cutaneous sclerosis and frequent systemic manifestations. Use of the term "morphea" diminishes the likelihood of confusion between these two disorders, which can lead to unnecessary patient anxiety. (See "Clinical manifestations and diagnosis of systemic sclerosis (scleroderma) in adults".)
Patients with morphea present with single or multiple inflammatory, sclerotic, and/or atrophic plaques (picture 1A-H). Disease activity typically persists for three to six years; some patients develop more persistent or recurring involvement. Cosmetic disfigurement or functional impairments due to dyspigmentation, atrophy, or limitation of range of motion often remain after the resolution of active disease. (See "Morphea (localized scleroderma) in adults: Management", section on 'Prognosis'.)
The clinical manifestations and diagnosis of morphea in adults will be reviewed here. The treatment and prognosis of morphea in adults and the features and management of morphea in children are discussed separately.
●(See "Morphea (localized scleroderma) in adults: Management".)
●(See "Juvenile localized scleroderma".)
EPIDEMIOLOGY —
Morphea is a relatively uncommon disorder that affects adults and children [1-4]. In one study of medical record data from a population in Minnesota, the annual incidence of morphea was approximately 3 per 100,000 people between 1960 and 1993 [5].
However, morphea may be more common than previously reported. An analysis of data from 2019 and 2020 collected in two large health care databases in the United States (MarketScan and TriNetX) found a crude incidence rate of 1.73 per 10,000 participants in MarketScan and 1.21 per 10,000 participants in TriNetX [6]. The crude prevalence rates for active morphea were 5.22 per 10,000 participants and 5.04 per 10,000 participants, respectively.
Although morphea can occur at any age, many patients (50 to 65 percent) develop the disease as adults [4,5,7]. In a nested case-control study of 110 patients with adult-onset morphea and 77 patients with juvenile-onset disease conducted at an academic medical center, the mean ages of disease onset were 45 and 10 years, respectively [7].
Females are more susceptible to morphea than males. In a retrospective study of 82 patients with morphea, the female-to-male ratio was 2.6:1 [5].
PATHOGENESIS —
The pathogenesis of morphea is poorly understood. Theories on the pathogenesis of morphea are often extrapolated from studies of systemic sclerosis [8]. A variety of factors, including autoimmunity, genetics, and environmental factors, may play a role in morphea. (See "Juvenile localized scleroderma", section on 'Etiology and pathogenesis'.)
●Immune dysfunction – It is generally accepted that immune dysfunction is involved in the development of morphea [4,9-11]. Although further study is needed, the available data suggest that different subtypes of morphea have a common molecular underpinning [12,13].
A role of autoimmunity in the development of morphea is supported by the following:
•A clinically evident inflammatory stage often precedes the development of sclerosis [14].
•Histopathologic studies of early lesions demonstrate an influx of large numbers of mononuclear lymphocytes (primarily activated T lymphocytes), plasma cells, and eosinophils [8].
•Cytokines associated with T helper type 1 (Th1) immune response (chemokine ligand [CXCL] 9, CXCL10, interferon [IFN]-gamma) are associated with active morphea and are found circulating in the blood and in the skin [15]. In a study that assessed serum levels of various cytokines in patients with morphea, CXCL9 levels correlated with disease activity [12].
Additional work in animal models of early sclerosing skin lesions showed that abrogation of CXCL9 via knockdown of the gene and the receptor decreased the development of sclerosis, supporting a pathogenic role for CXCL9 [16]. Moreover, mutations in STAT4 (the signal transduction molecule associated with this chemokine family) have been detected in some patients with pansclerotic morphea [17]. (See 'Pansclerotic morphea' below.)
•Increased autoantibody levels are present in some patients with morphea, particularly those with generalized or linear variants [4,9,10]. (See 'Subtypes' below.)
●Genetics – Genetic susceptibility may contribute to morphea. In addition to the detection of STAT4 mutations in some patients with familial pansclerotic morphea [17], increased frequencies of HLA I and II alleles associated with multiple autoimmune disorders have been detected in patients with morphea [18].
Familial case clusters have been reported [4,19,20]. Increased rates of autoimmune disorders have also been detected in family members of patients with morphea [4,9,21].
●Environmental factors – Multiple environmental factors (eg, radiation, infections, skin trauma [eg, friction, surgery], or environmental exposures) have been proposed as contributors to disease expression [22]. However, avoidance of medically necessary surgery or radiation therapy is not indicated for patients with morphea.
CLINICAL MANIFESTATIONS —
Morphea is divided into several subtypes [23], all of which transition through an early inflammatory stage followed by sclerosis and subsequent atrophy (figure 1). The depth of involvement may be superficial (primarily dermal) or deep (involving the deep dermis plus the subcutis, fascia, and/or bone).
Lesion evolution — The course of a morphea lesion includes inflammatory, sclerotic, and atrophic stages (figure 1). It is not uncommon for a patient with longstanding morphea to have a mixture of plaques at different stages of evolution. (See "Morphea (localized scleroderma) in adults: Management", section on 'Disease activity'.)
●Inflammatory stage – The initial sign of disease is often an inflammatory, erythematous patch or edematous plaque. These lesions may be accompanied by symptoms of pruritus, pain, or tightness. This stage is most responsive to therapy with immunosuppressives. (See "Morphea (localized scleroderma) in adults: Management".)
●Sclerotic stage – Sclerotic lesions in the skin manifest as firm, bound-down plaques or nodules. Active lesions often exhibit an erythematous, indurated border (picture 1A, 1I). As lesions evolve (or with successful treatment), the peripheral erythema becomes postinflammatory hyperpigmentation. This is the first sign of successful treatment.
Lesions transitioning toward inactivity will have central hypopigmentation, peripheral hyperpigmentation, alopecia due to secondary loss of hair follicles, and a shiny cutaneous surface (picture 1L).
●Atrophic stage – After a period of 12 to 24 months, sclerotic plaques soften and transition into hypopigmented or hyperpigmented atrophic plaques (picture 4B). The development of atrophy is a sign that a morphea lesion is inactive.
Atrophy in lesions that primarily involve the dermis may present as areas of fine, cigarette paper-like skin or shallow (cliff drop-like) depressions. Patients with deep morphea may be left with deep indentations after the resolution of active disease, reflecting loss of soft tissue and muscle.
Hair regrowth in affected sites on the trunk and extremities often occurs in this stage. However, alopecia is typically permanent on the scalp.
Deep involvement — Deep soft tissue involvement is linked to increased risk for specific extracutaneous manifestations, including underlying fasciitis, myositis, arthritis, and limitation of range of motion. (See 'Deep morphea' below.)
Subtypes — Morphea subtype classification is useful for both the diagnosis of morphea and risk stratification. Knowledge of the specific subtypes can aid in diagnosis and risk assessment for extracutaneous manifestations and complications.
●Subtype classification – The Padua criteria classification scheme, which divides morphea into circumscribed, generalized linear, pansclerotic, and mixed subtypes, is one of the most commonly used classification schemes for morphea. This classification scheme is supported by a cross-sectional study of 944 adults and children with morphea in which the Padua criteria allowed for the classification of almost all patients (95 percent) into a specific disease subtype [24]. Pansclerotic morphea is considered a severe presentation of generalized morphea (table 1).
●Morphea descriptors – A number of other previously described morphea subtypes are better classified as descriptors because they occur in any of the subtypes listed above and, therefore, do not describe a distinct group of patients. These include deep morphea (defined as lesions with pathology involving the subcutis or underlying soft tissue and beyond), keloidal morphea, and bullous morphea. (See 'Morphea descriptors' below.)
Circumscribed (plaque) morphea — Circumscribed morphea, also known as plaque morphea, presents as single or multiple well-defined, oval-to-round plaques that fail to meet criteria for generalized morphea (picture 1A and picture 1B and picture 1C). Circumscribed lesions are the most common manifestation of morphea in adults and, like the generalized variant, are more frequently observed in adults than in children. (See 'Generalized morphea' below.)
In some patients, linear or generalized morphea initially presents with features consistent with circumscribed disease, indicating the need to follow patients with circumscribed lesions over time. In general, circumscribed morphea is not associated with substantial extracutaneous manifestations even when deep involvement is present. (See "Juvenile localized scleroderma", section on 'Circumscribed (plaque) morphea'.)
Generalized morphea — Generalized morphea is defined by the presence of ≥4 morphea plaques involving at least two different anatomic sites (picture 1F, 1J) [25,26]. Lesions of generalized morphea frequently begin on the trunk before spreading acrally, and the fingers and toes are spared. With few exceptions, generalized morphea occurs in adults over the age of 40 years.
Coalescent plaque morphea — This subtype of generalized morphea has two patterns of cutaneous distribution that are useful to identify because they are associated with different extracutaneous manifestations [26]:
●Symmetric – The symmetric distribution is characterized by lesions distributed symmetrically over the arms, breasts, abdomen, back, and lower extremities. Although female predominant overall, most males with generalized morphea have this subset. These lesions are more frequently deep and associated with fasciitis, myositis, inflammatory arthropathy, limited range of motion in underlying joints, and pain.
●Isomorphic – The isomorphic distribution occurs almost exclusively in postmenopausal females and is characterized by lesions distributed in areas of chronic friction (ie, waistband, bra straps, inguinal creases, axilla, etc). These lesions exhibit dermal involvement and overlying lichen sclerosus more commonly. (See 'Lichen sclerosus' below.)
In patients with the isomorphic pattern, limited range of motion is rarely encountered. However, mucous membrane (genital more often than oral mucous membrane) involvement is more common, affecting up to 20 percent of patients. The genital lesions resemble lichen sclerosus histologically and clinically [26]. (See 'Lichen sclerosus' below and 'History and physical examination' below.)
In contrast to systemic sclerosis, generalized morphea does not present with sclerosis primarily involving acral skin or sclerodactyly. Raynaud phenomenon and hallmark autoantibodies associated with systemic sclerosis are also absent. (See 'Differential diagnosis' below and "Clinical manifestations and diagnosis of systemic sclerosis (scleroderma) in adults".)
Pansclerotic morphea — In the pansclerotic type of generalized morphea, deep morphea lesions occur in a generalized, circumferential distribution and involve the majority of the body surface area, excluding fingers and toes (picture 1G) [27,28]. Although originally reported in children, pansclerotic morphea also occurs in adults. Pansclerotic morphea may be associated with a STAT4 mutation in some cases [17]. (See 'Pathogenesis' above.)
The pansclerotic subtype is the most severe form of morphea and is characterized by disfigurement, limited range of motion, contracture, pain, and severe functional impairment. Because of the severe sclerosis over large parts of the trunk, patients experience symptoms of difficulty swallowing, shortness of breath, and uncomfortable sense of tightness after meals. (See "Juvenile localized scleroderma", section on 'Pansclerotic morphea'.)
Linear morphea — Linear morphea (also known as linear scleroderma) manifests as morpheaform plaques arranged in a linear distribution.
Linear morphea most often appears in childhood, but may have onset into the 20s, and is the most common subtype of morphea in children [1,3-5,7,29-32]. In one series, 95 of 291 patients (33 percent) with linear morphea had adult-onset disease, typically arising in the patients' 20s [32]. (See "Juvenile localized scleroderma", section on 'Linear scleroderma'.)
General findings — Linear morphea lesions most often occur on the extremities or face but can also appear on the trunk, where the differential diagnosis includes circumscribed morphea (picture 2 and picture 1E and picture 3). Patients may have single or multiple sites of involvement. The results of one retrospective study of 65 children with linear morphea suggested that linear morphea can follow the lines of Blaschko [33]. (See "Juvenile localized scleroderma", section on 'Linear scleroderma'.)
Involvement of deep tissues (subcutaneous tissue, muscle, or bone) in linear morphea can lead to functional impairment resulting from fasciitis, myositis, or inflammatory arthropathy. The effects can include muscle atrophy (which may produce muscle weakness), joint contractures, and, in growing children, limb length discrepancies (picture 4A-B). Bone marrow inflammation and bone changes have also been reported in patients with linear morphea [34,35]. (See 'Deep morphea' below.)
Craniofacial morphea — Craniofacial morphea (eg, en coup de sabre and progressive facial hemiatrophy) includes linear lesions of morphea affecting the head.
En coup de sabre — En coup de sabre is a type of linear morphea that affects the head and neck. Lesions manifest as hyperpigmented, atrophic plaques that resemble the cut of a sword (picture 1D, 1K-N).
The forehead is the most common site of involvement; lesions may extend onto the scalp, where they cause permanent alopecia. Other classic sites of involvement include the temple and chin. (See "Juvenile localized scleroderma", section on 'Linear scleroderma of the head/face'.)
Progressive facial hemiatrophy — Progressive facial hemiatrophy (Parry-Romberg syndrome) is a variant of linear morphea characterized by unilateral atrophy of the skin, soft tissues, muscles, and/or bones of the face (picture 5). Facial atrophy may be accompanied by dermal linear morphea lesions on the face (en coup de sabre) or elsewhere. (See "Juvenile localized scleroderma", section on 'Linear scleroderma of the head/face'.)
Mixed morphea — "Mixed morphea" is a term used to describe the simultaneous presence of more than one subtype.
Morphea descriptors — Morphea descriptors can occur in any morphea subtype and, therefore, do not describe a distinct group of patients. These include deep morphea, keloidal morphea, and bullous morphea.
Deep morphea — Deep morphea, or morphea profunda, is a form of morphea that primarily involves the deep dermis and subcutaneous tissue, mostly of the extremities, and may extend to underlying fascia and muscle. Plaques are poorly circumscribed (picture 1H) with an indurated texture and feel tightly tethered to underlying fascia and muscle. Areas of depression (the "groove sign") may be present at sites with underlying tendons and ligaments, reflecting tethering to these underlying structures.
The presence of deep morphea is more frequently associated with functional limitations and pain than morphea with more superficial involvement. (See 'Extracutaneous manifestations' below.)
Deep morphea may be indistinguishable from eosinophilic fasciitis. (See 'Differential diagnosis' below.)
Other descriptors — Keloidal morphea and bullous morphea have been described in the literature (picture 6). It is increasingly accepted that keloidal morphea is the occurrence of keloids within a lesion of morphea based on the histopathologic findings in these lesions. For this reason, we avoid the term "nodular morphea," a term that has been used interchangeably with keloidal morphea.
Bullous morphea has largely been described in dependent areas in patients with deeply sclerotic lesions. It is our opinion that this represents secondary changes resulting from edema in the affected extremity rather than a subtype of morphea [36].
Extracutaneous manifestations — Extracutaneous manifestations and extracutaneous complications of morphea may include musculoskeletal, pulmonary, esophageal, neurologic, ocular, and oral disorders. The evaluation for extracutaneous findings is based on the clinical presentation (table 2). (See 'Additional evaluation' below.)
●Musculoskeletal disorders – Fasciitis, myositis, or inflammatory arthritis associated with morphea may contribute to arthralgias, joint swelling, myalgias, limb contractures, and limited range of motion (picture 7) [4,37].
Musculoskeletal disorders are relatively common and are most likely to occur in patients with linear morphea, generalized morphea, or deep tissue involvement [37]. In one prospective cohort study of patients with morphea, 127 of 605 adults (21 percent) had musculoskeletal manifestations [37].
Musculoskeletal involvement typically underlies the cutaneous lesions of morphea. Occasionally, it occurs outside the area of cutaneous lesions.
●Neurologic, ocular, and oral disorders – Approximately 4 percent of individuals with linear lesions affecting the head, including en coup de sabre and/or hemifacial atrophy, have neurologic or ocular complications, such as seizures; headaches; trigeminal neuralgia; fasciculations of underlying musculature; uveitis; episcleritis; and adnexal abnormalities involving the eyelids, eyelashes, or lacrimal glands [21,38-40].
Linear morphea of the head, particularly of the facial skin overlying the cheek and mandible, may be associated with dental malocclusion, altered dentition, and atrophy of the tongue and salivary glands. A destructive gingivitis has also been reported in patients with facial morphea lesions, usually of the linear subtype. This is characterized by inflammation of the gingiva followed by regression of gingiva and bone, likely representing the same pathologic process as in the overlying skin [41].
●Pulmonary and esophageal disorders – Pulmonary and esophageal abnormalities occur in a minority of patients with morphea; however, in the vast majority of cases, the abnormalities have been asymptomatic and only detectable with investigational studies [42]. More commonly, symptoms of dysphagia or dyspnea may develop secondary to the restrictive effects of extensive cutaneous sclerosis, and examination of the lungs and esophagus reveals absence of any pathologic findings.
Evaluation for pulmonary or esophageal abnormalities is not indicated in the absence of symptoms.
LABORATORY ABNORMALITIES —
Laboratory abnormalities may occur in association with morphea. However, in the absence of additional signs or symptoms supporting further investigation, routine testing for these abnormalities is not indicated in patients with morphea. (See 'Associated disorders' below.)
●Serum autoantibodies – Many adults and children with morphea have elevated antinuclear antibody (ANA) levels. Reported rates of ANA positivity among patients with morphea range from 18 to 68 percent [4,7,30,43,44].
The increased prevalence of ANA positivity compared with the general population was evident in a nested case-control study of 187 adults and children with morphea conducted at an academic medical center [7]. A positive ANA was detected in 34 percent of patients with morphea compared with only 11 percent of 651 matched controls.
Other autoantibodies that are detected less frequently than ANA in patients with morphea include anti-single-stranded deoxyribonucleic acid (ssDNA), anti-double-stranded deoxyribonucleic acid (dsDNA), antihistone, antitopoisomerase II alpha, antiphospholipid, and rheumatoid factor [4,10,43,45-50].
Although associations between certain autoantibodies and the severity of linear morphea have been reported [7,44], the prognostic significance of autoantibodies in morphea remains unclear. Testing for autoantibodies is not indicated in the absence of clinical signs that suggest the presence of another autoimmune disorder. (See 'Autoimmune and other disorders' below.)
●Other laboratory abnormalities – Peripheral eosinophilia, hypergammaglobulinemia, and increased erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels may occur with many types of active disease. The utility of serial measurements of these parameters to follow disease activity is still unclear.
Based on our observations, when eosinophilia is present in patients with deep morphea, the level of eosinophilia may correlate with disease activity. This finding may reflect the overlapping features of deep morphea and eosinophilic fasciitis. (See 'Differential diagnosis' below.)
ASSOCIATED DISORDERS
Lichen sclerosus — Clinical findings consistent with extragenital or genital lichen sclerosus may occur in patients with morphea, particularly in postmenopausal females with generalized isomorphic morphea (picture 8). (See 'Coalescent plaque morphea' above and 'History and physical examination' below and 'Periodic genital examination' below.)
●Relationship with morphea – The nature of the relationship between lichen sclerosus and morphea is controversial. It is unclear whether the appearance of lesions consistent with lichen sclerosus represents the simultaneous occurrence of two separate disorders or the development of clinical findings that resemble lichen sclerosus in lesions of morphea.
A prospective study of 76 patients with morphea and 101 controls supports increased risk for genital lichen sclerosus in patients with morphea [51]. Genital lichen sclerosus was detected in 38 percent of patients with morphea compared with only 3 percent of controls (odds ratio 19.8, 95% CI 5.7-106.9).
Risk for concomitant lichen sclerosus may differ based on morphea subtype. In a prospective cohort of 735 adults and children with morphea, the frequency of genital lichen sclerosus was low in the overall group (4 percent), but genital involvement was most frequent in female patients with generalized morphea [52].
●Clinical manifestations – Extragenital or genital lichen sclerosus usually manifests as atrophic, white patches with finely wrinkled surfaces (picture 8). Hyperpigmented or hypopigmented presentations of extragenital lichen sclerosus may also occur in individuals with darkly pigmented skin (picture 9).
Extragenital lichen sclerosus most commonly occurs on the trunk and proximal extremities. The labia minora, labia majora, glans penis, penile prepuce, and penile foreskin are frequent sites for genital disease (picture 10A-B). (See "Extragenital lichen sclerosus: Clinical features and diagnosis" and "Vulvar lichen sclerosus: Clinical manifestations and diagnosis" and "Balanitis in adults".)
Autoimmune and other disorders — Autoimmune disorders may occur at a relatively high frequency in patients with morphea [4,7]. In a study of 123 adults with morphea, 37 (30 percent) had another autoimmune disease [4]. Concomitant autoimmune disease was most common in adults with generalized morphea.
Raynaud phenomenon, a finding that may occur in association with autoimmune disease, has also been reported in a minority of children and adults with morphea but is generally not considered a finding associated with morphea [4,30]. (See "Clinical manifestations and diagnosis of Raynaud phenomenon".)
In the absence of additional signs or symptoms supporting further investigation, routine testing for concomitant autoimmune disease is not indicated in patients with morphea.
DIAGNOSIS —
In many patients, the diagnosis of morphea can be made based on the clinical findings. However, histopathologic studies and radiologic imaging can be useful to confirm a diagnosis or evaluate the extent of disease (table 2).
The recognition of deep cutaneous involvement is important to support appropriate evaluation and management of patients at risk for functional impairment and other complications (table 2) [37]. (See 'Extracutaneous manifestations' above and 'Patients with findings suggestive of deep or musculoskeletal involvement' below.)
History and physical examination — The patient history should include an assessment of the course and progression of lesions and associated symptoms (pruritus, pain, tightness).
A full skin examination should be performed to determine the extent and estimated depth of skin involvement. Patients should also be examined for contractures, limb length discrepancy, reduced range of motion, and other functional impairments. A genital examination should be performed on patients with generalized morphea or extragenital lichen sclerosus et atrophicus overlap with morphea to detect signs of genital lichen sclerosus. (See 'Lichen sclerosus' above.)
Infrequently, patients with systemic sclerosis develop morpheaform plaques [53]. Therefore, patients with findings consistent with morphea should be asked about a history of Raynaud phenomenon (a characteristic early feature of systemic sclerosis) and should be evaluated clinically for other signs and symptoms of systemic sclerosis, such as nail fold capillary changes, sclerodactyly, or acrosclerosis. (See 'Differential diagnosis' below and "Clinical manifestations and diagnosis of systemic sclerosis (scleroderma) in adults", section on 'Evaluation for suspected systemic sclerosis'.)
Skin biopsy — Skin biopsies are generally reserved for cases in which the diagnosis is in question. Magnetic resonance imaging (MRI) is the preferred test for the evaluation for deep involvement. (See 'Patients with findings suggestive of deep or musculoskeletal involvement' below.)
●Biopsy technique – Skin biopsy specimens should include subcutaneous fat, if possible. A punch biopsy or incisional biopsy can be performed. (See 'Patients with findings suggestive of deep or musculoskeletal involvement' below and "Skin biopsy techniques".)
The biopsy specimen can be taken from the inflammatory or indurated border, if present, or from a central sclerotic area. The pathologist should be informed of the clinical features of the site from which the biopsy is taken.
For lesions with minimal clinical change, a second biopsy from unaffected skin in a location similar to the lesion site (eg, contralateral skin) should be performed to assist with the interpretation of the lesional pathologic findings.
●Histopathologic findings – The pathology of morphea varies based on biopsy site, lesion stage, and lesion depth. The detection of histopathologic findings that suggest involvement of the deep dermis or subcutis should prompt an evaluation for deep morphea. (See 'Patients with findings suggestive of deep or musculoskeletal involvement' below.)
•Inflammatory lesions – Biopsies performed from inflammatory lesions demonstrate an interstitial, periadnexal, and/or perivascular inflammatory cell infiltrate composed primarily of lymphocytes and plasma cells. Eosinophils, mast cells, and macrophages may be present. Inflammation may extend into the subcutaneous tissues. In addition, tissue edema, enlarged tortuous vessels, and thickened collagen bundles may be observed.
•Sclerotic lesions – As sclerosis progresses, lesions demonstrate homogenization of the papillary dermis and thickened collagen bundles extending into the reticular dermis or beyond (picture 11). Fat surrounding eccrine glands diminishes, and advanced sclerotic lesions exhibit compression and loss of appendageal structures. Few blood vessels are present, and those that remain exhibit fibrotic walls and narrowed lumina. In specimens from deep morphea, the deep reticular dermis, subcutis, and fascia show sclerotic changes.
•Atrophic lesions – The atrophic phase of morphea is characterized by loss of inflammatory cell infiltrate, less sclerosis, and an absence of appendageal structures. Telangiectasia may be evident. In some cases, basal keratinocytes display pigment along with underlying melanophages [54].
•Association with symptoms – The histopathologic findings may correlate with patient symptoms [36,54]. A study of 83 adults and children with morphea found an association between a bottom-heavy pattern of sclerosis and symptoms of pain and tightness [36]. The study also found a nonstatistically significant trend toward an association between severe inflammation and symptoms of pain and functional limitation. A study of 137 adults and children with morphea found similar results as well as an association between the presence of tissue eosinophils or basal pigmentation and poor treatment response [54].
ADDITIONAL EVALUATION —
Patients with morphea can develop extracutaneous complications that require additional evaluation (table 2). The likelihood of extracutaneous involvement can be risk stratified based on the subtype of morphea and its clinical features. (See 'Extracutaneous manifestations' above.)
Patients with findings suggestive of deep or musculoskeletal involvement — Patients who present with findings that suggest morphea that extends beyond the dermis, as suggested by involvement of the deep dermis or subcutis on pathology or clinical signs (eg, tightly bound-down quality on skin examination, muscle pain, tightness, weakness, contractures, or limb length discrepancies), should undergo MRI of the affected areas to assess lesion depth and involvement of soft tissue [35,55]. Ultrasonography is an alternative when MRI is not feasible [56]. We also refer patients with clinical signs of musculoskeletal involvement to a rheumatologist.
●MRI – MRI findings in morphea can include fascial thickening, fascial enhancement, articular synovitis, tenosynovitis, perifascial enhancement, myositis, enthesitis, bone marrow involvement, and subcutaneous septal thickening [55].
●Ultrasound – Ultrasound is not useful for diagnosis of morphea but may be used to assess the depth of involvement and response to treatment of morphea lesions. Ultrasound should only be undertaken when there is a collaborating radiologist with appropriate expertise.
•Findings – Typical ultrasound findings in morphea include increased dermal thickness, increased dermal blood flow, increased echogenicity of the subcutaneous tissue, and decreased dermal echogenicity [57]. Atrophic lesions have decreased dermal and subcutaneous thickness without an increase in blood flow [57]. The contralateral unaffected site should be assessed for comparison.
•Considerations for use – Ultrasound assessment should be reserved for situations in which there is a collaborating radiologist with appropriate expertise. The utility of ultrasound in morphea depends on the use of the correct equipment and settings, the selection of appropriate sites for imaging, and the accurate interpretation of ultrasound findings. When following the disease over time, it is critical that the same area is subjected to repeat scans.
●Rheumatology referral – For patients primarily managed by dermatologists, consultation with a rheumatologist is helpful for assessing the need for additional diagnostic evaluation of musculoskeletal symptoms and comanagement. We routinely refer patients with symptoms of joint swelling or pain, reduced weight bearing, decreased range of motion, contractures, or limb length discrepancy to rheumatology.
MRI and ultrasound have also been used to monitor disease activity. (See "Morphea (localized scleroderma) in adults: Management", section on 'Assessment of response'.)
Data are insufficient to determine whether routine performance of MRI to detect asymptomatic musculoskeletal involvement is beneficial for patients without clinical findings suggestive of deep involvement. A retrospective study of 43 patients with deep circumscribed, linear, or generalized (plaque or pansclerotic) morphea found MRI evidence of musculoskeletal involvement in 6 of 16 patients (38 percent) in whom such involvement was not clinically suspected [55].
Patients with craniofacial morphea
●General approach – Patients with lesions involving the head or face (eg, en coup de sabre, progressive facial hemiatrophy) are at risk for ocular, central and peripheral nervous system, and oral involvement and should have a history, review of systems, and physical examination to identify changes related to these extracutaneous manifestations.
•Ocular assessment – We refer patients with craniofacial morphea to an ophthalmologist to assess for ocular complications. Patients should receive a baseline ophthalmologic examination and a repeat examination annually.
In addition, we evaluate patients for ocular symptoms at each morphea follow-up visit and educate patients and caregivers to notify us if ocular symptoms occur. If ocular symptoms occur, more frequent ophthalmologic assessment is warranted.
In addition, we obtain an MRI of the periocular area in patients with periocular morphea lesions to assess for involvement of the periocular soft tissue and orbit.
•Oral assessment – A thorough oral examination including the palate, tongue, uvula, dentition, and gingiva should be performed in addition to evaluation of the temporomandibular joint. Referral to dentistry or oral and maxillofacial surgery is beneficial for patients with evidence of dental involvement or jaw dysfunction. (See "Temporomandibular disorders in adults", section on 'Evaluation'.)
•Neurologic assessment – We refer patients with associated neurologic symptoms to a neurologist for evaluation. In particular, localizing neurologic symptoms or deficits, severe migraines, or seizures warrant urgent referral and evaluation by a neurologist. Collaboration with clinicians with expertise is important to determine whether neurologic findings reflect active inflammation requiring systemic immunosuppressive therapy or neurologic residual damage.
Routine radiologic imaging of the brain is not indicated in patients without neurologic symptoms.
Peripheral nervous system involvement can present with symptoms of neuropathic pain, muscle fasciculations, or dysesthesia. Central nervous system involvement may be asymptomatic or include headaches, seizures, focal neurologic deficits, or neuropsychiatric abnormalities [58]. Headache is the most common presentation of central nervous system involvement and is often ipsilateral to the morphea lesion with migraine-like symptoms. Common brain MRI findings in patients with neurologic involvement include hyperintensities in the subcortical white matter or other sites, focal tissue atrophy, and calcifications [58].
Cases of central nervous system vasculitis or retinal vasculitis requiring emergent treatment have also been reported [59,60].
Periodic genital examination — Genital involvement occurs most frequently in postmenopausal females with generalized morphea, manifesting as lesions with the appearance of lichen sclerosus et atrophicus. Periodic examination of the genitalia is prudent, particularly in this population. (See 'Lichen sclerosus' above and "Vulvar lichen sclerosus: Clinical manifestations and diagnosis" and "Vulvar lichen sclerosus: Management".)
DIFFERENTIAL DIAGNOSIS —
Other disorders can present with clinical features that resemble morphea.
●Systemic sclerosis – Systemic sclerosis (scleroderma) is an autoimmune disorder characterized by progressive sclerosis of the skin and internal organ involvement. Systemic sclerosis generally begins with the Raynaud phenomenon, and patients commonly exhibit initial puffiness and eventual sclerosis in the fingertips (sclerodactyly), frequently accompanied by nailfold capillary change. These changes are absent in patients with morphea. (See "Clinical manifestations and diagnosis of systemic sclerosis (scleroderma) in adults".)
●Lipodermatosclerosis – Lipodermatosclerosis is a fibrosing panniculitis that usually occurs on the lower legs in association with chronic venous insufficiency. Patients present with indurated, sometimes circumferential plaques that may give the lower leg the appearance of an inverted champagne bottle (picture 12A-B). (See "Clinical manifestations of lower extremity chronic venous disorders", section on 'Lipodermatosclerosis (C4b)'.)
●Carcinoma en cuirasse and postirradiation morphea – Lesions involving the breast should be biopsied to exclude carcinoma en cuirasse, a rare condition that occurs most frequently in the setting of breast cancer. Tumor infiltrates skin and subcutaneous tissue, causing diffuse sclerotic changes on the chest. Morpheaform changes in the breast also rarely occur as a complication of radiation therapy (picture 13) [61-63]. (See "Clinical manifestations and evaluation of locoregional recurrences of breast cancer", section on 'Postmastectomy recurrence'.)
●Atrophoderma of Pasini and Pierini – Atrophoderma of Pasini and Pierini may represent morphea diagnosed in the atrophic stage and presents with single or multiple well-demarcated, depressed patches due to dermal atrophy (picture 14). The areas of depression exhibit a cliff drop-like appearance. Lesions are usually round or oval, ranging in size from a few centimeters to large, expansive lesions. The trunk is most commonly involved, and lesions tend to have a symmetrical distribution. (See "Atrophoderma of Pasini and Pierini".)
●Eosinophilic fasciitis – Eosinophilic fasciitis, or Shulman syndrome, is a disorder that is characterized by the rapid onset of symmetric, painful, large, edematous plaques that evolve into fibrotic lesions (picture 15). Some cases appear to be precipitated by trauma. (See "Eosinophilic fasciitis".)
The extremities are the most common sites of involvement. Similar to deep morphea, linear depressions (the "groove sign") may be present in involved areas. Occasionally, there are associated dermal lesions of morphea [64,65]. Laboratory abnormalities include peripheral eosinophilia, hypergammaglobulinemia, and an elevated erythrocyte sedimentation rate (ESR).
A history of sudden onset plus histopathologic findings demonstrating a characteristic marked thickening of the fascia accompanied by a mixed inflammatory infiltrate with lymphocytes, histiocytes, plasma cells, and eosinophils may help to distinguish this disorder from deep morphea. However, in some cases, the disorders cannot be distinguished [66]. Clinical findings consistent with morphea and eosinophilic fasciitis may also coexist [67-69].
Additional disorders in the differential diagnosis are provided in a table (table 3).
SOCIETY GUIDELINE LINKS —
Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Morphea (localized scleroderma)".)
SUMMARY AND RECOMMENDATIONS
●Overview – Morphea, also known as localized scleroderma, is a relatively uncommon idiopathic inflammatory disorder that leads to the development of sclerotic plaques in the skin. The disorder occurs in both adults and children and preferentially affects females. (See 'Epidemiology' above.)
●Pathogenesis – The pathogenesis of morphea is not well understood. The disorder is likely to have an autoimmune basis; genetic and environmental factors may also play a role in the development of this disease. (See 'Pathogenesis' above.)
●Clinical subtypes – Morphea has a variety of clinical presentations. Subtypes include circumscribed, generalized, pansclerotic, linear, and mixed morphea (picture 1A-H). Mixed morphea describes the simultaneous presence of more than one subtype of morphea. (See 'Subtypes' above.)
●Clinical manifestations – Lesions of morphea typically begin as inflammatory patches or plaques that evolve into firm, sclerotic plaques (picture 1A). Involvement may be limited to the dermis or may extend to underlying subcutaneous fat, muscle, or bone. Atrophic changes often remain after lesion resolution. (See 'Clinical manifestations' above.)
Clinical findings of lichen sclerosus may also occur. (See 'Lichen sclerosus' above.)
●Extracutaneous involvement – Musculoskeletal, neurologic, ocular, oral, and other disorders may occur in association with morphea. Musculoskeletal disorders are relatively common and are most likely to occur in patients with linear morphea, generalized morphea, or deep tissue involvement. (See 'Extracutaneous manifestations' above.)
●Diagnosis – The recognition of characteristic clinical findings is often sufficient for the diagnosis of morphea. Biopsy can be useful when the diagnosis is in question. Serologic tests are not helpful for the diagnosis of morphea.
Biopsies should always extend at least into the subcutaneous fat. For sclerotic lesions, particularly those with modest clinical change, a second biopsy from unaffected skin in a similar location will assist with the interpretation of the pathologic findings. (See 'Diagnosis' above.)
●Additional evaluation – The clinical evaluation should always include an assessment for contractures, functional impairment, and signs or symptoms of extracutaneous involvement. Based on the clinical presentation, further evaluation may include radiologic imaging, specialist referral (eg, rheumatology, neurology, ophthalmology, dentistry), and/or additional clinical evaluation (table 2).
Radiologic imaging (MRI or ultrasound) to assess the extent of involvement is indicated for patients in whom clinical examination suggests that morphea extends deeper than the dermis (eg, tightly bound-down quality on skin examination, muscle weakness, contractures, or limb length discrepancies). Periodic examination of the genitalia is helpful for identifying patients with concomitant lichen sclerosus. (See 'Additional evaluation' above.)
