Morphea in Adult
See also in: External and Internal Eye,Hair and ScalpAlerts and Notices
Synopsis
The estimated annual incidence of morphea is 3.4-27 cases per 1 000 000. There is a bimodal peak of onset: 7 and 11 years for pediatric-onset disease and 44 and 47 years for adult-onset disease. Women are affected more frequently than men (2.4-5:1). Up to 20% of patients with morphea have extracutaneous disease or concomitant autoimmune disease such as psoriasis, vitiligo, inflammatory bowel disease (Crohn, ulcerative colitis), diabetes mellitus, and thyroiditis.
Morphea can be divided into several clinical subtypes, including circumscribed or plaque type, linear (including en coup de sabre and Parry-Romberg syndrome), generalized, and less common variants such as bullous, pansclerotic, and mixed morphea. Eosinophilic fasciitis, also known as Shulman syndrome, is often considered a part of the clinical spectrum of morphea.
In circumscribed or plaque-type morphea, classic lesions of morphea are round or oval erythematous to violaceous plaques. As lesions progress, sclerosis occurs at the center of the lesions, leading to an indurated, yellow-white, waxy center with an erythematous to violaceous border, resembling a "lilac halo." 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.
In the linear subtype, there is band-like cutaneous sclerosis that is usually unilateral and can cause contractures and limb-length discrepancies in up to 10% of patients. En coup de sabre is a specific subtype of linear morphea that affects the paramedian forehead and scalp, and it can be accompanied by alopecia as well as ocular, neurological, and odontostomatologic complications. 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.
Generalized morphea is a rare variant that is defined as more than 4 plaques larger than 3 cm and/or involving 2 or more body sites. Individuals with this variant are more likely to have systemic symptoms including myalgias, arthralgias, and fatigue. This variant may be difficult to distinguish from scleroderma.
Pansclerotic morphea is a debilitating variant that affects subcutaneous tissues and even bone. There is associated muscle atrophy, joint contractures, and nonhealing ulcers.
Complications include:
- Joint mobility and impairment of muscle growth – in linear or deep morphea crossing joint lines
- Ocular or oral complications – in craniofacial morphea
- Carpal tunnel syndrome – in morphea at the wrist
- Chronic ulcers or squamous cell carcinoma – in pansclerotic morphea
Codes
ICD10CM:L94.0 – Localized scleroderma [morphea]
SNOMEDCT:
201049004 – Morphea
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Morphea and lichen sclerosus may present together. This presentation is known as lichen sclerosus / morphea overlap.
- 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 – Caused by some chemotherapeutic agents, polyvinyl chloride (PVC), injection site reaction from vitamin K, enfuvirtide, silicone, paraffin, and many other compounds.
- Nephrogenic fibrosing dermopathy
- Acrodermatitis chronica atrophicans
- Chronic graft-versus-host disease
- Radiation fibrosis
- Cutaneous metastases
- Reflex sympathetic dystrophy
- Dermatofibrosarcoma protuberans
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Management Pearls
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.Subscription Required
References
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Last Reviewed:04/03/2023
Last Updated:04/06/2023
Last Updated:04/06/2023
Morphea in Adult
See also in: External and Internal Eye,Hair and Scalp