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General Medicine

Morphea

Veronica J. Ruston, DO Reviewed 06/2018
 


BASICS

DESCRIPTION

  • A benign inflammatory skin disorder leading to excessive collagen deposition with thickening and sclerosis of the skin and SC tissues and characteristic plaque formation

    • Lesions are w...

DIAGNOSIS

HISTORY

  • Patient notes slowly progressive skin changes over months. Coin-sized lesions slowly enlarge over weeks to months.

  • Rarely, the lesions can cause some discomfort.

  • Usually, there are mult...

TREATMENT

MEDICATION

First Line

  • Active lesions can respond to treatment, inactive lesions cannot. Relapse rates present in 25–35% (3)[B].

  • Superficial subtypes generally respond to topical treatments.

    • Reco...

ONGOING CARE

Lesions may self-resolve but this usually takes 3 to 5 years. Recommend evaluation of joints, eyes, and CNS for involvement throughout course of active disease. Many patients with plaque ...

REFERENCES

1
Mertens J, Seyger M, Thurlings R, et al. Morphea and eosinophilic fasciitis: an update. Am J Clin Dermatol.  2017;18(4):491–512. [View Abstract on OvidMedline]
2
Careta MF, Romiti R. Lo...

ADDITIONAL READING

  • Fett N, Werth VP. Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol.  2011;64(2):217–230.

  • Li SC, Torok KS, Pope E, et al; and ...

CODES

ICD10

  • L94.0 Localized scleroderma [morphea]

  • L94.1 Linear scleroderma

ICD9

701.0 Circumscribed scleroderma 

SNOMED

  • 201049004 Morphea (disorder)

  • 128458002 plaque morphea (disorder)

  • 7513007 Generalized mor...

CLINICAL PEARLS

  • Morphea lesions are well-circumscribed plaques that are firm, waxy, and have an ivory color. They are usually multiple, start small, and may slowly enlarge over time. Presence on the t...

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Related Images
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FIG. 77.2. Face of a 19-year-old woman with diffuse cutaneous systemic sclerosis. Note loss of normal skin folds and retraction of lips.
FIG. 77.2. Face of a 19-year-old woman with diffuse cutaneous systemic sclerosis. Note loss of normal skin folds and retraction of lips.
FIG. 77.6. Close-up hand roentgenogram of a 46-year-old woman with limited cutaneous systemic sclerosis. Note extensive subcutaneous calcinosis.
FIG. 77.6. Close-up hand roentgenogram of a 46-year-old woman with limited cutaneous systemic sclerosis. Note extensive subcutaneous calcin...
Scleroderma. The dermis is characterized by large reticular dermal collagen bundles that are oriented parallel to the epidermis. The large size and loss of basket-weave pattern of these collagen bundles are abnormal. No appendages are apparent, as these are destroyed in the disease process.
Scleroderma. The dermis is characterized by large reticular dermal collagen bundles that are oriented parallel to the epidermis. The large ...
FIG. 77.11. Photomicrograph of the lung of a 52-year-old woman with diffuse cutaneous systemic sclerosis who died as the result of respiratory insufficiency. Note the dramatic interstitial fi osis and dilatation of air sacs (honeycomb lung).
FIG. 77.11. Photomicrograph of the lung of a 52-year-old woman with diffuse cutaneous systemic sclerosis who died as the result of respirat...
FIG. 77.20. A child with linear scleroderma involving the left lower extremity with evidence of a left knee flexion contracture. There is a plaque of morphea on the right thigh.
FIG. 77.20. A child with linear scleroderma involving the left lower extremity with evidence of a left knee flexion contracture. There is a...
FIG. 2.17. Juvenile dermatomyositis and scleroderma. A: Atrophic, hypopigmented lesions overlying extensor surfaces of interphalangeal joints, with periungual erythema typical of juvenile dermatomyositis. B: Linear scleroderma involving left lower extremity in a 12-year-old girl.
FIG. 2.17. Juvenile dermatomyositis and scleroderma. A: Atrophic, hypopigmented lesions overlying extensor surfaces of interphalangeal join...
scleroderma: early stage
scleroderma: early stage
Scleroderma: hand, amputated distal digits
Scleroderma: hand, amputated distal digits
Figure 10-195 SCLERODERMA: ACROOSTEOLYSIS. A. PA Hand. Note the early resorption of the distal tufts (arrows). Incidentally noted is a bandage (arrowhead). B. PA Hand. Observe the advanced resorption of the distal tufts.
Figure 10-195 SCLERODERMA: ACROOSTEOLYSIS. A. PA Hand. Note the early resorption of the distal tufts (arrows). Incidentally noted is a band...
Linear scleroderma: leg
Linear scleroderma: leg
Morphea: abdomen
Morphea: abdomen
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