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Morphea - External and Internal Eye
See also in: Overview,Hair and Scalp
Other Resources UpToDate PubMed

Morphea - External and Internal Eye

See also in: Overview,Hair and Scalp
Contributors: Christine S. Ahn MD, FAAD, William W. Huang MD, MPH, FAAD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Morphea is a cutaneous fibrosing connective tissue disorder that represents a localized form of scleroderma (or systemic sclerosis). Morphea is distinguished from systemic sclerosis by the absence of sclerodactyly, Raynaud phenomenon, and nail fold capillary changes. The pathogenesis of morphea is not fully understood, but autoimmune dysfunction and imbalance of collagen production and destruction, as well as inflammation and vascular changes, are thought to be key components of the disease.

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.

Codes

ICD10CM:
L94.0 – Localized scleroderma [morphea]

SNOMEDCT:
201049004 – Morphea

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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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.

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References

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Last Reviewed:11/20/2017
Last Updated:12/06/2017
Copyright © 2021 VisualDx®. All rights reserved.
Morphea - External and Internal Eye
See also in: Overview,Hair and Scalp
Morphea : Hyperpigmented patch, Taut, shiny skin, Thickened skin, Hardened skin
Clinical image of Morphea
A close-up of a shiny and wrinkled, white and yellowish plaque with a pink rim.
Copyright © 2021 VisualDx®. All rights reserved.