Morphea - External and Internal Eye
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, diabetes mellitus, and thyroiditis. About 3% of pediatric patients have ocular manifestations including eyelid and eyelash changes, anterior segment inflammation, strabismus, pseudopapilledema (optic disc drusen), and refractive errors. Ocular involvement may be associated with central nervous system abnormalities.
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. Ocular manifestations have been associated with the en coup de sabre form (two-thirds of patients), the linear form (one-fifth), and plaque or generalized forms (remaining).
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, such as impaired vision, retroocular pain, and epilepsy. 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. It may be associated with ptosis, extraocular muscle dysfunction, anterior uveitis, episcleritis, glaucoma, xerophthalmia, and keratitis.
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.
L94.0 – Localized scleroderma [morphea]
201049004 – Morphea
- Morphea and lichen sclerosus may present together. This presentation is known as lichen sclerosus / morphea overlap.
- Systemic sclerosis (scleroderma)
- Stasis dermatitis with fibrosis / lipodermatosclerosis
- Porphyria cutanea tarda with sclerodermoid features
- Erythema nodosum
- 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