A 45-year-old woman presents with oval-shaped lesions on her arms that she first noted a few months ago. The lesions began as an area of discrete erythema, progressed to induration, and then became dark, smooth, and shiny. She has a family history of rheumatoid arthritis. On examination, the patient has multiple hyperpigmented and hypopigmented indurated plaques with a shiny surface; her nails and fingertips are normal, and autoantibody tests were negative.
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Morphea, also known as localized scleroderma, refers to a broad spectrum of sclerotic skin diseases. The clinical presentation varies from mild asymptomatic sclerotic lesions to severe sclerosis involving muscles and joints that carries the risk of physical impairment.1-3
The annual incidence of morphea in the United States is 2.7 per 100,000.1 White women are most commonly affected.1,2 This condition has a bimodal population distribution, affecting both children and adults. The peak incidence of onset for pediatric patients is between 7 and 11 years of age; the peak age of onset in adults is between 44 and 47 years.2,3 Pediatric patients have a higher frequency of deep tissue involvement and a longer disease duration, leading to a higher morbidity.1
Although the pathogenesis of morphea is still being studied, it is thought that the disease results from vascular dysfunction, immune dysregulation, and extensive extracellular matrix formation.2 Susceptibility mechanisms (genetics and autoimmunity) and external stimuli (vaccination and friction) are thought to play a role in disease development.2,4 The likely pathogenesis involves a triggering event in a susceptible individual followed by recruitment of keratinocytes and fibroblasts, activation of epidermal-dermal signaling pathways, and increased immunoinflammation.4
Approximately 10% to 30% of patients with morphea have a positive family history of autoimmune disease.1 Therefore, it is theorized that there may be a genetic component to this condition. Epigenetic and genetic markers for morphea are currently under investigation.1,5 A study of 329 patients revealed that 16% developed lesions at surgical sites, injection sites, or penetrating trauma sites, suggesting that skin trauma may also be associated with the development of morphea.6 Morphea has also been reported as a rare side effect of radiation therapy or treatment with tumor necrosis factor-a inhibitors.2,7
Classification of morphea is based on the pattern and depth of lesions and includes circumscribed morphea, linear morphea, generalized morphea, pansclerotic morphea, and mixed morphea.2,8 Other classification schemes include eosinophilic fasciitis within the morphea spectrum.2
- Circumscribed morphea is further divided into superficial and deep morphea. Circumscribed superficial morphea, characterized by plaques limited to the dermis of the trunk, waist, and submammary region, is the most common subtype in adults and presents with local discomfort and disfigurement.2,8 Circumscribed deep morphea may extend into subcutaneous tissue, is often located symmetrically in the lower extremities, and has the potential to cause contractures and tissue atrophy.8
- Linear morphea is characterized by unilateral, deep, band-like cutaneous sclerosis and is the most common subtype in childhood-onset morphea.2,8 Limb-length differences and subcutaneous atrophy can occur.
- Generalized morphea is more common in adults than children and is characterized by large superficial plaques that spare the hands, face, and feet.8
- Pansclerotic morphea affects the skin, subcutaneous tissue, muscle, and bone. The lesion may also involve other areas of the body without internal organ involvement.2
- Mixed morphea is a combination of at least 2 subtypes. In the literature, the order of the concomitant subtypes, specified in brackets, will follow their predominant representation in the individual patient (ie, mixed morphea [linear-circumscribed]).2
- Eosinophilic fasciitis is characterized by symmetric deep sclerosis of the extremities after the development of pitting edema and erythema; systemic symptoms of weight loss and myalgia may often be present.2
Lesions have an early inflammatory stage followed by a sclerosing stage, then an atrophic stage. During the early stage, lesions are erythematous but as they progress they may develop a purple ring (“lilac ring”) around the fibrotic center.5 Morphea most commonly affects the chest and abdomen.6 Recurrences occur in approximately 20% to 40% of patients and are associated with early disease onset, linear distribution subtype, and initial sclerosis involving muscle and cartilage.9
Histologically, morphea is characterized by an infiltrate of lymphocytes and plasma cells in a perivascular and perieccrine distribution.2 Sclerotic skin demonstrates thick and homogeneous collagen bundles at varying levels of the dermis.2 Morphea may be further characterized by sclerosis pattern (confined to papillary dermis, reticular dermis, subcutis, or throughout), degree and location of inflammation, and which cell type is present.2
Early lesions may be mistaken for lichen sclerosis, erythema migrans, post-inflammatory hyperpigmentation, lipodystrophy, and scarring.5 Systemic sclerosis, pseudoscleroderma, and mixed connective tissue disease are included in the differential diagnosis of generalized morphea.5 Morphea can be differentiated from systemic sclerosis through the absence of Raynaud phenomenon, gastrointestinal problems, sclerodactyly, and nailfold capillary changes.2
The diagnosis of morphea is largely based on clinical findings. The Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) may be helpful in diagnosing and monitoring the disease in adults and children.2,5 There are no serologic markers specific for morphea; therefore, antibody screening is only indicated in cases of unclear presentation or to confirm or rule out systemic sclerosis.2,5 Eosinophilic fasciitis is the exception to this, as routine testing of absolute eosinophil count and inflammatory markers is recommended in the initial phase of the disease and for the detection of disease reactivation.2
When choosing a treatment, it is important to consider disease activity, depth of involvement, and side effects. Standard therapy for limited skin lesions includes topical corticosteroids, topical calcipotriol, and/or ultraviolet A1 phototherapy.2,5 Topical therapies are often sufficient for superficial lesions due to the high rate of spontaneous remission in 3 to 5 years.8
Systemic therapies, such as methotrexate and/or systemic corticosteroids, are used in forms of morphea with extensive skin involvement or those affecting adipose tissue, joints, fascia, and muscle.5,8 Long-term use of oral systemic corticosteroids alone is not indicated due to high recurrence rates.2
Based on clinical features and pathologic examination, the patient in this case was diagnosed with circumscribed superficial morphea. She was prescribed a high-potency topical corticosteroid to be applied once a day for 4 weeks. Mild improvement was noted at 1-month follow-up. She continued to be followed in the clinic and showed gradual improvement over several years.
Samantha Morgan, BS, and Eleanor Johnson, BA, are medical students at Baylor College of Medicine. Christopher Rizk, MD, is a dermatologist affiliated with Elite Dermatology in Houston, Texas.
- Torok KS, Li SC, Jacobe HM, et al. Immunopathogenesis of pediatric localized scleroderma. Front Immunol. 2019;10:908-908.
- Mertens JS, Seyger MMB, Thurlings RM, Radstake TRDJ, de Jong EMGJ. Morphea and eosinophilic fasciitis: an update. Am J Clin Dermatol. 2017;18(4):491-512.
- Mertens JS, Seyger MMB, Kievit W, et al. Disease recurrence in localized scleroderma: a retrospective analysis of 344 patients with paediatric‐ or adult‐onset disease. Br J Dermatol.2015;172(3):722-728.
- Saracino AM, Denton CP, Orteu CH. The molecular pathogenesis of morphoea: from genetics to future treatment targets. Br J Dermatol. 2017;177(1):34-46.
- Kreuter A, Krieg T, Worm M, et al. German guidelines for the diagnosis and therapy of localized scleroderma. J Dtsch Dermatol Ges. 2011;14(2):199-216.
- Grabell D, Hsieh C, Andrew R, et al. The role of skin trauma in the distribution of morphea lesions: a cross-sectional survey of the Morphea in Adults and Children Cohort IV. J Am Acad Dermatol.2014;71(3):493-498.
- Trivedi A, DeWitt CM, McGevna L. Radiation-induced circumscribed superficial morphea after brachytherapy for endometrial adenocarcinoma. Int J Womens Dermatol.2017;3(4):234-236.
- Martini G, Fadanelli G, Agazzi A, Vittadello F, Meneghel A, Zulian F. Disease course and long-term outcome of juvenile localized scleroderma: experience from a single pediatric rheumatology centre and literature review. Autoimmun Rev. 2018;17(7):727-734.
- Litaiem N, Bacha T, Drissi H, Zeglaoui F. An evaluation of long‐term outcomes and recurrence rates in patients with morphea. Int J Dermatol. 2019;58(4):E90-E92.