A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report
© Bonilla-Abadía et al.; licensee BioMed Central Ltd. 2012
Received: 20 September 2012
Accepted: 18 December 2012
Published: 20 December 2012
The localized scleroderma (LS) known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS) and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis.
We report an uncommon case of a white 53 year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide.
Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient.
KeywordsLocalized scleroderma Morphea Multiple autoimmune syndrome Central nervous system vasculitis
The localized scleroderma (LS) is distinguished from systemic sclerosis not only by the absence of vasospasm, structural vascular damage, and involvement of internal organs, but also by the distribution of the skin lesions. LS, also known as morphea, presents a variety of clinical manifestations that can include systemic nvolvement. Morphea is characterized by sclerosis of the skin and in some cases underlying tissue. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting the possible autoimmune basis of morphea. We report an uncommon case of LS as part of a multiple autoimmune syndrome associated with pneumonitis and central nervous system vasculitis.
A 53 year old white female patient Jeovah witness was referred to our institution because of general and respiratory symptoms of several days consisting in malaise, weakness, decreased appetite, dyspnea, nonproductive cough, bleeding disorder compatible with idiopathic thrombocytopenic purpura (ITP) and sudden headache associated with diplopia and blurred vision. Her medical history included localized scleroderma type morphea in the lower limbs, vitiligo since 25 years ago, autoimmune hypothyroidism, carpal tunnel and obesity.
LS associated with autoimmune diseases, presence of autoantibodies, treatment and response
Associated autoimmune condition
Response to treatment
Plaques over the trunk
Vitiligo, Hashimoto’s thyroiditis
Antithyroglobu- lin antibody, anti-thyroid peroxidaz
On her trunk and right arm.
Necrotizing vas- culitis of arterioles within the subcutaneous fat and muscles. Mononeuritis multiplex.
Lupus anticoaglant test, Anti-DNA antibody, (LE) test, Anticardiolipin-β2GPI antibody
Pulse therapy with 1 g methyl prednisolone for 3 days, cyclophosphamide (50 mg/d).
Umbilical region, right hemi thorax and back of the right hand
Idiopathic thrombocytopenic purpura
ANAS, Anti DNA
Idiopathic thrombocytopenic purpura
53 year old woman
Atrophic violaceous plaques in the lower limbs
Vitiligo, autoimmune hypothyroidism, neumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis
Anti-microsomal and Anti-thyroglobulin antibodies
Methylprednisolone 1 gr/day per five days) and intravenous cyclophosphamide (1 gr monthly
Is likely that LS have an autoimmune origin supported by the reports described and its association with other autoimmune diseases, in this case forming part of MAS and with the aggravation to be associated with life-threatening conditions requiring aggressive treatment. This makes it important to look for systemic conditions in patients with localized scleroderma that may worsen the clinical localized disease.
Written informed consent was obtained from the patient for publication of this manuscript and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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