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Morphea in Child
See also in: External and Internal Eye,Hair and Scalp
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Morphea in Child

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

Synopsis

Morphea is a localized form of scleroderma without systemic involvement. The etiology currently is unknown, although potential exogenous triggers have been suggested, including infection with varicella zoster virus (VZV), Borrelia burgdorferi, or Epstein-Barr virus (EBV), local trauma, sunburn, surgery, and Bacillus Calmette-Guérin (BCG) vaccinations for tuberculosis. The initial event in the pathogenesis is thought to be the induction of vascular damage that leads to activation of the immune system, excessive collagen deposition, and subsequent fibrosis.

Morphea can be divided into the following clinical subtypes: linear, plaque type (including guttate and keloidal), generalized, bullous, and deep morphea.

In children, morphea is observed in a 2-3:1 female-male ratio, with the mean age of onset ranging from 7-10 years. The most common subtype in childhood-onset morphea is linear morphea, which includes limb or trunk involvement, "en coup de sabre," and progressive facial hemiatrophy, followed by the plaque variant.

Linear morphea is characterized initially by a linear erythematous or violaceous plaque, which develops into a yellow-white sclerotic plaque with a violaceous border ("lilac ring") as the disease progresses. It is usually limited to the dermis, although deeper involvement can occur. On the limbs, linear morphea is usually unilateral and can lead to limb-length discrepancies and possible limitations in mobility. The en coup de sabre variant is named for its location on the paramedian forehead that can cross onto the frontal scalp, with associated alopecia. This subtype can be associated with ocular, neurological, and odontostomatological complications. Progressive facial hemiatrophy, also known as Parry-Romberg syndrome, is thought to be a variant of en coup de sabre and involves diffuse unilateral subcutaneous atrophy of the face.

Codes

ICD10CM:
L94.0 – Localized scleroderma [morphea]

SNOMEDCT:
201049004 – Morphea

Look For

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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: 11/17/2017
Last Updated: 12/06/2017
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Morphea in Child
See also in: External and Internal Eye,Hair and Scalp
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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.
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