MORPHEA

By Gary M. White, MD

morphea An indurated plaque on the left flank of a 60-year-old man. Note the classic violaceous border.


Morphea is a fibrotic condition of the skin in which indurated plaques develop. It is most common in adult women on the trunk, but may occur in children as well.

Scleroderma Variants

Disease Features
Morphea (Localized) Indurated, sclerotic plaques, usually on the trunk.
Generalized Morphea Two or more body regions involved. May have muscle atrophy, but no systemic involvement.
Superficial morphea Multiple hypopigmented to hyperpigmented patches and plaques. They usually involve intertriginous sites symmetrically and lack significant induration, contractures, or atrophy.
Progressive Systemic Sclerosis Raynaud's, woody edema of hands, sclerodactyly, internal organ involvement, e.g., pulmonary fibrosis.
CREST Syndrome Calcinosis, Raynaud's, esophageal dysmotility, sclerodactyly, and telangiectasias.
Linear Scleroderma Linear indurated lesions. May follow lines of Blaschko.
Morphea Profunda Process involves the deep subcutaneous tissue including the fascia.
Pansclerotic Morphea Widespread involvement of the deeper tissue including the fat fascia, muscle, and bone.
Atrophoderma of Pasini and Pierini (APP) Brown, atrophic depressed lesions, usually on the trunk of women.
Linear Atrophoderma of Moulin Similar to APP but linear and may follow lines of Blaschko.
En Coup de Sabre Linear atrophic, indurated lesion running along scalp and forehead.
Parry Romberg Atrophy of one side of the facial skin with possible involvement of the muscle, fat, cartilage, bone, and brain.

Antibodies

Various antibodies may be found including ANA, anti-single stranded DNA, anti-histone antibodies (AHA) and RF. AHAs were found in 87% of patients with generalized morphea vs. 42% of patients with localized disease. Eighty percent of patients with morphea have a positive ANA.

Radiation Therapy

Cases of morphea occurring only in the radiation port of breast cancer patients have been reported. See here.

Clinical

Solitary or multiple areas of erythema and induration in a woman is characteristic. Morphea in middle-aged women often occurs at sites of pressure. The center may develop an ivory color and a surrounding violaceous halo. The trunk and proximal extremities are typically involved. In general, patients with morphea should not have disease of the face (e.g., restriction of opening the mouth, telangiectatic mats) or distal extremities (e.g., Raynaud's phenomenon, dilated capillary loops, acrosclerosis). These findings would indicate systemic sclerosis.

Very rarely, patients may go on to develop systemic sclerosis. Nodular lesions may rarely develop. Generalized morphea is characterized by widespread skin involvement (no consensus on criteria for diagnosis, but one article defined it as four or more lesions on at least two areas of the body) but without Raynaud's phenomenon, acrosclerosis, and involvement of internal organs as in scleroderma. Rarely, morphea and lichen sclerosus may coexist (see below). Cases of "bullous morphea" may actually represent secondary appearance of bullous lichen sclerosus. Skin trauma may play a role in the distribution of the skin lesions [JAAD 2014;71;493].

A study of two hundred thirty-five cases of morphea showed that it is usually self-limited with a tendency to spontaneously regress after 3 to 5 years [Arch Derm. 1956;74:629–639].

Workup

If extensive, checking porphyrin levels may be indicated to rule out the induration associated with porphyria cutanea tarda. If the case occurs outside the US, workup for Borrelia infection should be done.

A genital exam should be performed. In one study, 38% of asymptomatic patients with morphea had vulvar lichen sclerosus.

TREATMENT

Corticosteroids

Common first-line therapy is to apply a high-potency topical steroid (e.g., clobetasol BID) or inject triamcinolone (e.g., 5-10 mg/cc) into any "active," inflamed areas. Occlusion may be employed. Continuous therapy should be limited to 3 months to avoid side effects, followed if indicated by intermittent therapy.

Tacrolimus

A DBPC trial showed tacrolimus 0.1% ointment applied twice daily to be beneficial if given over 3-4 months.

Calcipotriene

Topical calcipotriene BID may also be beneficial, especially when nightly occlusion (e.g., with plastic wrap) is used to increase penetration of the medication. Combining it with betamethasone dipropionate has also been reported effective.

Imiquimod

Daily topical imiquimod 5% cream 3-5 times per week has been shown to help reduce dyspigmentation, induration, erythema, and telangiectasias [J Cutan Med Surg. 2015 Mar-Apr;19(2):132-9]. This treatment appears safe in children [Dermatology 2011;223(4):363-9].

Systemic Steroids

Prednisone (0.5 - 1.0 mg/kg/d) is often used for more severe morphea, including linear, generalized and deep subtypes. A minimum of 3 months of therapy is often needed to see results.

Methotrexate

For rapidly progressive, generalized morphea, methotrexate may be used. This applies to children as well. Three options are available: 1. methotrexate as monotherapy, 2. pulsed methotrexate and methylprednisolone given intravenously and 3. pulsed methotrexate and prednisone given orally. Adult doses of methotrexate range from 12.5-25 mg/week. Pediatric dosing for methotrexate is 15 mg/m2 BSA/week with a maximum of 25 mg/week [JEADV 2017;31;1401].

Mycophenolate mofetil

MM may be given if methotrexate fails [Rheumatology (Oxford) 2009;48;1410].

UVA1

UVA1 can be effective in morphea, be it plaque, linear, or generalized. In one study, sixty percent of patients responded, however, relatively high rates of recurrence were seen [JAAD 2014;70:481-8].

PUVA

Standard, bath and topical cream PUVA had all been used with benefit. Therapy is usually given 2-3 per week for a minimum of 30 sessions.

Miscellaneous

Other therapies reported in isolated cases include cyclosporine, azathioprine, hydroxychloroquine chloroquine, phenytoin, colchicine, retinods, abatacept, infliximab, rituximab and imatinib. Everolimus, an mTOR inhibitor, at a dose of 0.5 mg BID and a duration of 6 months was very helpful in a 57-year-old woman with generalized morphea [JAMA Derm 2016;152;1170].

References

Superficial morphea. Dermatology Online Journal 2017 (5)

Additional Pictures

Always palpate the lesion to appreciate the induration.
morphea morphea

Morphea morphea

Lichen sclerosus and morphea overlap.
Morphea Morphea

References

20-year-old woman with morphea over the shoulder leading to some decreased range of motion addressed with physical therapy. The patient also had lupus. Dermatology Online Journal 17(10)

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