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Morphea

BASICS

DESCRIPTION

- Three phases: active/edematous, inactive/sclerotic/fibrotic, and atrophic lesions - Morphea is classified into five types (Mayo Classification): - Plaque: most common subtype especially in adults, well-circumscribed, and confined to dermis - Generalized: 4+ plaques involving 2+ body sites - Bullous: rare - Linear: most common subtype in children - Deep: more severe and involving all layers from skin to bone, causing limb contractures and muscle atrophy

  • A benign inflammatory skin disorder leading to excessive collagen deposition with thickening and sclerosis of the skin and SC tissues and characteristic plaque formationLesions are well-circumscribed, flat, firm to touch, and have a waxy feel. They may be more easily felt than seen. They are ivory-colored and have a lilac halo border.Lesions are not associated with generalized scleroderma/systemic sclerosis based on lack of sclerodactyly, Raynaud phenomenon, and nail fold capillary changes.
  • Three phases: active/edematous, inactive/sclerotic/fibrotic, and atrophic lesions
  • Morphea is classified into five types (Mayo Classification):Plaque: most common subtype especially in adults, well-circumscribed, and confined to dermisGeneralized: 4+ plaques involving 2+ body sitesBullous: rareLinear: most common subtype in childrenDeep: more severe and involving all layers from skin to bone, causing limb contractures and muscle atrophy
  • Synonym(s): localized scleroderma (LoS); scleroderma circumscripta

EPIDEMIOLOGY

Incidence

  • Predominant sex: female > male (3:1)
  • Peak incidence of plaque subtype in 3rd to 4th decade
  • 50% of morphea cases present during childhood.
  • Linear morphea can develop before age 10 years in 20% of patients.
  • 0.3 to 3 cases per 100,000/year

Prevalence

  • Onset in children is between ages 2 and 14 years.
  • 50 cases per 100,000 at age 18 years
  • 220 cases per 100,000 at age 80 years

ETIOLOGY AND PATHOPHYSIOLOGY

  • Sclerosis is limited to the skin and systemic sclerosis is absent.
  • Biopsy shows lymphocytic perivascular infiltration of the dermis, decreased number of blood vessels and eccrine (sweat) glands.
  • Causation thought to be from vascular damage and enhanced collagen production, though likely with underlying autoimmune etiology
  • Some association with past trauma, including surgery and vaccination administration, or radiation to the area

RISK FACTORS

Unknown. Raynaud phenomenon is risk factor for systemic scleroderma, not localized. ‚

COMMONLY ASSOCIATED CONDITIONS

  • Occurs with slightly greater frequency in those with a personal or family history of autoimmune disorders
  • Sometimes associated with other diagnosis, such as arthralgias, carpal tunnel syndrome, Raynaud phenomenon, spina bifida, lichen planus, lichen sclerosis, vitiligo, and alopecia areata

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 multiple lesions and they are present bilaterally.
  • Patients can have symptoms of malaise, fatigue, arthralgias, and myalgias.
  • In linear morphea, there is only one lesion.

PHYSICAL EXAM

  • Initially may be soft patches or plaques. Become indurated plaques of skin 2 to 15 cm; usually multiple lesions are present. Eventually sclerose and atrophy
  • Mauve-colored, shiny, and develop a lilac-colored border; lesions can be yellow-, white- or ivory-colored. Less commonly may appear as bands or nodules
  • More commonly present on the trunk in areas of friction, trauma, or body folds. Hair loss in the plaque is noted.

DIFFERENTIAL DIAGNOSIS

  • Consider testing for Borrelia burgdorferi because this disease can cause sclerotic plaques, but no firm data indicate Borrelia causes morphea.
  • Progressive systemic sclerosis, lichen sclerosis, eosinophilic fasciitis, scleroderma, late porphyria cutanea tarda, chronic graft-versus-host disease, lipodermatosclerosis
  • Indurated reticulated plaques

DIAGNOSTIC TESTS & INTERPRETATION

Initial Tests (lab, imaging)

  • Biopsy of the lesion is the gold standard (1)[C].
  • Some sources recommend testing for B. burgdorferi because this disease can cause sclerotic plaques. B. burgdorferi does not cause morphea.
  • Basic rheumatologic lab work may be considered (ANA and RF).
  • Would expect to be negative in LoS
  • Ultrasonography demonstrates increased cutaneous blood flow during active phase; 100% sensitive and specific. Can also be used to determine severity of musculoskeletal involvement and response to treatment (2)[C]
  • Consider MRI if musculoskeletal involvement is suspected.
  • Consider workup for scleroderma if other CREST symptoms are present (calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias) or symmetric distribution.

Diagnostic Procedures/Other

  • Punch biopsy at the leading edge will confirm the diagnosis.
  • Biopsy through the thick skin may require a bit more pressure than usual.

Test Interpretation

  • Inflammatory cells in the dermis
  • Dermis is edematous and the collagen is infiltrated with eosinophils.
  • Dense collagen is present.
  • There is a loss of hair follicles and sebaceous glands.

TREATMENT

MEDICATION

First Line

  • Active lesions can respond to treatment, inactive lesions cannot. Relapse rates present in 25 " “35% (3)[B].
  • Methotrexate with or without corticosteroids, has been shown to have the most benefit.Recommend 1mg/kg/wk SC to max of 25 mg/wk plus folic acid supplementation for 12 to 24 months (2)[C],(3)[B]
  • Prednisone 1 mg/kg/day for 3 months (3)[B]
  • Phototherapy with PUVA or UVA1 are widely used; expect about 30 sessions until improvement is seen (4)[C]. Can be used in all skin types without serious side effects
  • Topical tacrolimus 1% and calcipotriene had modest benefit (2)[C].

Second Line

  • Intralesional steroids can be somewhat helpful. However, lesions are so dense that the steroid may have a hard time diffusing throughout the lesion and there can be patchy softening.
  • Mycophenolate mofetil (MMF) after failure to MTX and/or phototherapy (2,4)[C]
  • Physical therapy
  • Reconstructive surgery, especially if on face

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 form will progress to linear or generalized. ‚

PROGNOSIS

  • It is common for new lesions to appear periodically with long periods of remission (2,3).
  • Prolonged disease course in 1/3 of patients often with permanent sequelae (3)[B].

COMPLICATIONS

  • Lesions can be very difficult to treat, resulting in functional and cosmetic deficits in about 10% of patients (5).
  • Children may have growth disturbance, functional limitations, and other orthopedic complications with at least 25% reporting disability as adults (2).
  • > 20% with extracutaneous manifestations including seizures, arthritis, and uveitis.

REFERENCES

11 Nouri ‚ S, Jacobe ‚ H. Recent developments in diagnosis and assessment of morphea. Curr Rheumatol Rep. 2013;15(2):308.22 Careta ‚ MF, Romiti ‚ R. Localized scleroderma: clinical spectrum and therapeutic update. An Bras Dermatol. 2015;90(1):62 " “73.33 Piram ‚ M, McCuaig ‚ CC, Saint-Cyr ‚ C, et al. Short- and long-term outcome of linear morphoea in children. Br J Dermatol. 2013;169(6):1265 " “1271.44 Sartori-Valinotti ‚ J, Tollefson ‚ MM, Reed ‚ AM. Updates on morphea: role of vascular injury and advances in treatment. Autoimmune Dis. 2013;2013:467808.55 Ravelli ‚ FN, Andriolo ‚ BNG, Vascocellos ‚ MRA, et al. Interventions for morphea. Cochrane Database Syst Rev. 2014;(6):CD005027.

ADDITIONAL READING

  • Fett ‚ N, Werth ‚ VP. Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol. 2011;64(2):217 " “228.
  • Li ‚ SC, Torok ‚ KS, Pope ‚ E, et al. Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma. Arthritis Care Res (Hoboken). 2012;64(8):1175 " “1185

CODES

ICD10

  • L94.0 Localized scleroderma [morphea]
  • L94.1 Linear scleroderma

ICD9

701.0 Circumscribed scleroderma ‚

SNOMED

  • Morphea (disorder)
  • plaque morphea (disorder)
  • Generalized morphea
  • Linear scleroderma (disorder)
  • Lichen sclerosus et atrophicus (disorder)

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 trunk is common.
  • Punch biopsy of leading edge is diagnostic.
  • Difficult and challenging to treat with frequent relapses experienced.
  • High potential for significant morbidity requiring long-term care and decreased quality of life.