Morphea

Overview
Morphea, also known as localized scleroderma, is a thickening and hardening of the skin and subcutaneous tissues from excessive collagen deposition. Morphea includes specific conditions ranging from very small plaques only involving the skin to widespread disease causing functional and cosmetic deformities. Morphea involves isolated patches of hardened skin and discriminates from systemic sclerosis by its supposed lack of internal organ involvement.

Classification
The most widely used classification divides morphea into five general subtypes: plaque morphea, generalized morphea, linear scleroderma, bullous morphea, and deep morphea. This classification scheme does not include the mixed form of morphea in which different morphologies of skin lesions are present in the same individual. Up to 15% of morphea patients may fall into this previously unrecognized category.

Epidemiology
Morphea is an uncommon condition that is thought to affect 1 in 100,000 people. Adequate studies on the incidence and prevalence have not been performed. Morphea also may be under-reported as physicians may be unaware of this disorder and smaller morphea plaques may be less often referred to a dermatologist or rheumatologist. As in many other connective tissue or autoimmune disorders, morphea mainly involves women with a W:M ratio of 3:1.

Etiology
Physicians and scientists do not know what causes morphea. Case reports and observational studies suggest there is a higher frequency of family history of autoimmune diseases in patients with morphea. Tests for autoantibodies associated with morphea have shown results in higher frequencies of anti-histone and anti-topoisomerase IIa antibodies. Case reports of morphea co-existing with other systemic autoimmune diseases such as primary biliary cirrhosis, vitiligo, and systemic lupus erythematosus lend support to morphea as an autoimmune disease.

Diagnoses of Morphea should be Distinguished from

 * Annular lichenoid dermatitis of youth
 * Ataxia-telangiectasia
 * Atrophoderma of pasini and pierini
 * Carcinoid syndrome
 * Cheiroarthropathy due to diabetes mellitus
 * Eosinophilia myalgia syndrome
 * Eosinophilic fasciitis
 * Erythema migrans
 * Fixed drug eruption
 * Graft versus host disease
 * Inflammatory granuloma annulare
 * Interstitial and granulomatous dermatitis
 * Interstitial mycosis fungoides
 * Keloid and hypertrophic scar
 * Lichen sclerosus et atrophicus
 * Linear atrophoderma of Moulin
 * Linear lupus erythematosus panniculitis
 * Linear melorheostosis
 * Lipodermatosclerosis
 * Morpheaform dermatofibrosarcoma protuberans
 * Muckle-Wells syndrome
 * Nephrogenic fibrosing dermopathy
 * Niemann-Pick disease
 * Phenylketonuria
 * POEMS syndrome
 * Porphyria cutanea tarda
 * Primary systemic amyloidosis
 * Progeria
 * Radiation fibrosis
 * Reflex sympathetic dystrophy
 * Restrictive dermopathy
 * Scleroderma
 * Sclerodermoid conditions caused by chemical/toxin exposures
 * Polyvinyl chloride
 * Epoxy resins
 * Pesticides
 * Dry cleaning solvents
 * Silica dust
 * Sclerodermoid conditions caused by iatrogenic agents
 * Bleomycin
 * Gemcitabine
 * L-Tryptophan
 * Melphalan isolated limb perfusion
 * Pentazocine injections
 * Silicone or paraffin implants
 * Taxanes
 * Uracil-Tegafur
 * Vitamin K injections
 * Scleromyxedema
 * Stiff skin syndrome
 * Sweet syndrome (early)
 * Werner syndrome
 * Winchester syndrome

Treatment
Throughout the years, many different treatments have been tried for morphea including topical, intra-lesional, and systemic corticosteroids. Antimalarials such as hydroxychloroquine or chloroquine have been used. Other immunomodulators such as methotrexate, topical tacrolimus, and penicillamine have been tried. Ultraviolet A (UVA) light, with or without psoralens have also been tried. UVA-1, a more specific wavelength of UVA light, is able to penetrate the deeper portions of the skin and thus, thought to soften the plaques in morphea by acting in two fashions:


 * 1) by causing a systemic immunosuppression from UV light.
 * 2) by inducing enzymes that naturally degrade the collagen matrix in the skin as part of natural sun-aging of the skin.

As with all of these treatments for morphea, the difficulty in assessing outcomes in an objective way has limited the interpretation of most studies involving these treatment modalities.

Additional Resources

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