coexistence of linear scleroderma and systemic sclerosis
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CONNECTIVE TISSUE DISEASES
P6873A case of scleroderma with myocarditis presenting as acute heart failure
Corinne Taraska, MD, University of Washington, Seattle, WA, United States
The fibrosing autoimmune disease, scleroderma (systemic sclerosis, SSc), carries a10-year survival rate of 66%. Cardiac and pericardial disease occur in up to 80% of SScpatients with a wide array of presentations. We report a 44-year-old woman referredto our medical center for heart transplant with a 6-month history of orthopnea,exertional dyspnea, and weight gain diagnosed as nonischemic cardiomyopathy. Onevaluation, history revealed new Raynaud symptoms for 1 year. Physical examina-tion showed symmetric waxy yellow thickening of the skin from the fingers to theproximal forearms with sclerodactyly and limited finger flexion. Cardiac andpulmonary studies displayed mild restrictive lung disease, an ejection fraction(EF) of 6%, right ventricular enlargement, and severely reduced global systolicfunction with no evidence of infarct. Skin biopsy from the proximal forearmdemonstrated thick densely packed hyalinized collagen bundles with diminishedadventitial fat around eccrine glands. Laboratory values highlighted an elevatedantinuclear antibody titer (1:640), erythrocyte sedimentation rate, and creatinekinase and indeterminate Scl-70 antibodies. This combination of findings pointed tomyocarditis associated with scleroderma and myositis overlap as the cause of thispatient’s heart failure. In response, she was started on intravenous methylprednis-olone followed by a long prednisone taper. A repeat echocardiogram after 10 days oftherapy showed improvement in her EF to 30%. She was then given a single dose ofrituximab, ultimately discontinued because of hypotension, and subsequently begancyclophosphamide on the Euro-Lupus protocol. At 3 months of follow-up, she wastransitioned off cyclophosphamide and onto mycophenolate mofetil (MMF) formaintenance therapy. Now 6 months out, she continues on MMF with a stable EF,increased energy, and resolution of exertional dyspnea and orthopnea symptoms.This case demonstrates diffuse systemic sclerosis with myocarditis presenting ascongestive heart failure and improvement in cardiac function and related symptomsafter treatment with high dose glucocorticoids, cyclophosphamide and mycophe-nolate mofetil. There are few published reports on treatment of scleroderma relatedacutemyocarditis, and to our knowledge this is the first report of this combination oftherapies.
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cial support: None identified.
CommerP6837Annular elastolytic giant cell granuloma: Case report and treatment withdapsone
Stella Ramos-e-Silva, MD, Universidade Federal do Rio de Janeiro, Rio de Janeiro,Brazil; Juan Manuel Pineiro Macedo, Universidade Federal do Rio de Janeiro, Riode Janeiro, Brazil; Marien Siqueira Soto Lopes, MD, Universidade Federal do Riode Janeiro, Rio de Janeiro, Brazil; Rodrigo Pirmez, MD, Universidade Federal doRio de Janeiro, Rio de Janeiro, Brazil; Sueli Carneiro, MD, PhD, UniversidadeFederal do Rio de Janeiro, Rio de Janeiro, Brazil; Tiago Silveira Lima, MD,Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil
Background: Annular elastolytic giant cell granuloma (AEGCG), is also known asactinic granuloma and elastolytic granuloma annulare. It is an uncommon disorderof unknown pathogenesis, which lesions are prevalent in areas exposed to sunlight.Its onset is insidious characterized by erythematous papules, isolated or grouped,coalescing into annular plaques with incomplete or polycyclic arrangements andatrophic center. Histopathologically, the infiltrate is granulomatous with no forma-tion of palisade, with lymphocytes, histiocytes, and giant cells with phagocytosis ofelastic fibers, with no necrobiosis or mucin deposit.
Case report: A 62-year-old woman presented with multiple erythematous, circinatelesions on face, limbs and presternal region. Histopathologic examination revealedelastolytic fragmentation of elastic fibers underneath a normal epidermis andlocalized, increased collagenous tissue. The granulomatous infiltrate contained afew multinucleated and giant cells mixed with some eosinophils and lymphocytes.There was no evidence of necrobiosis of the collagen or of mucin deposition. Shewas treated with dapsone for 10 years, with progressive remissions and relapses, butnever with complete resolution.
Discussion: AEGCG is a rare granulomatous skin disease of unknown etiology thatwas recognised as a disease entity by Hanke et al. It is clinically similar to granulomaannulare (GA) but is located mostly in sun-exposed areas and less frequently oncovered skin as the GA and histopathologically is also separated. GA affects theconnective tissue of the reticular dermis and is characterized histologically by acentral area of collagen degeneration surrounded by a granulomatous infiltrationcalled palisading granuloma. Other differential diagnoses of AEGCG include necro-biosis lipoidica, which is clinically characterized as sclerotic reaction pattern and ishistologically presented by vascular wall thickening areas of necrobiosis, capillaryendothelial, palisading granulomas, lipid depositions, and proliferation. As for themanagement of AEGCG, various treatment options have to be considered forpatients with this rather rare disease showing that its treatment is still difficult andremains unsatisfactory. Our patient, although treated with dapsone for 10 years,regarded as effective even for resistant cases, still presents recurrence.
cial support: None identified.
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P6445Bilateral periorbital edema: Debut of systemic lupus erythematosus
Lara Angulo, Hospital Universitario 12 de Octubre, Madrid, Spain; Belen Rubio,Hospital Universitario 12 de Octubre, Madrid, Spain; Carlos Zarco, HospitalUniversitario 12 de Octubre, Madrid, Spain; Concepci�on Postigo, HospitalUniversitario 12 de Octubre, Madrid, Spain; Lidia Maro~nas, Hospital Universitario12 de Octubre, Madrid, Spain; Victoria Alegr�ıa, Hospital Universitario 12 deOctubre, Madrid, Spain
The bilateral eyelid edema is a cutaneous sign associated with multiple etiologies,such as infections, tumors, trauma, facial dermatitis, and autoimmune diseases.Dermatomyositis is the best known within autoimmune diseases, but periorbitaledema has also been described as a cutaneous manifestation of lupus erythematosusmore frecuently in ethnic skin. To date, there are only 18 cases reported in theliterature of SLE and 10 of them are the beginning of the disease. A 42-year-oldwoman presented with a 2-month history of asymptomatic, bilateral swelling of hereyelids and cheeks. As accompanying symptoms, the patient had more severefatigue in the last 2 weeks with arthralgia in several joints and diarrhea with nopathologic products. Additional tests highlight leukopenia, neutropenia, proteinuriain the borderline significance with traces of blood in the urine sediment and highIgG hypergammaglobulinemia. Autoimmunity tests showed ANA positive 1/640,anti-DNA positive 1/50, anti Sm positive, anti-RNP positive, Anti-Ro positive and anti-La positive. Therefore, the patient met criteria for systemic lupus erythematosusaccording to the classification of the ‘‘American College of Rheumatology.’’ Theclinical and laboratory tests allowed to exclude other diseases associated eyelidedema. In conclusion, we emphasize the unusual presentation of lupus erythema-tosus by bilateral periorbital edema, especially in people of color where it is moredifficult to assess the erythema.
cial support: None identified.
CommerP6660Coexistence of linear scleroderma and systemic sclerosis
Mariana Campos Souza Menezes, MD, Federal University of Rio de Janeiro, Rio deJaneiro, Brazil; Flauberto Souza Marinho, MD, Federal University of Rio deJaneiro, Rio de Janeiro, Brazil; I Lym Chan, MD, Federal University of Rio deJaneiro, Rio de Janeiro, Brazil; Rosangela S. Rodrigues, MD, PhD, FederalUniversity of Rio de Janeiro, Rio de Janeiro, Brazil; Tullia Cuzzi, MD, PhD,Federal University of Rio de Janeiro, Rio de Janeiro, Brazil
Background: Scleroderma is a word derived from Greek skleros (induration) derma(skin) and consists of a chronic disease of unknown etiology that includes changesconfined to the epidermis, dermis and subcutaneous tissue (localized sclerodermaor morphea) and thickening of collagen fibers in the dermis, fibrosis and vascularchanges in various organs (systemic sclerosis). There are few reports of coexistenceof the 2 entities (systemic and localized scleroderma) and/or progression ofmorphea to systemic sclerosis. Herewith, we report a case that illustrates thiscoexistence for its rarity and diagnostic dilemma.
Results: A white, 53-year-old woman, showed brown macules progressing to linearrigid plaques, diffusely arranged in the left hemibody, that emerge gradually from 12years of age. Histopathologic findings were compatible with linear sclerosis. Yearslater, patient developed Raynaud phenomenon, sclerodactyly, multiple telangiecta-sias, reticular livedo, loss of tissue in the fingertips, and positive anti-SCL70.Capillaroscopy showed the presence of dilated and giant capillaries and rankedpatient in the early stage of systemic scleroderma.
Conclusion: Scleroderma is a rare disease. Approximately 80% of affected patientsare female, most frequently black women. Linear scleroderma represents 20% ofcases of morphea, is more common in childhood and adolescence, and frequentlyoccurs before 18 years of age. Characterized by linear atrophic, hyperchromiclesions which may extend through the dermis, subcutaneous tissue, muscle and upto the bone, can cause significant deformations. Lesions are usually unilateral. It isknown that the coexistence of localized and systemic sclerodermawas described ina few case reports published until now. Localized scleroderma is not always a purelycutaneous disease. Some patients with localized disease, particularly adults, haveevidence of involvement of internal organs, association with other connective tissuediseases, and sometimes even transition to systemic sclerosis. The presence, in thiscase, of systemic involvement after years of treatment allows us to infer thecoexistence of linear scleroderma and systemic sclerosis. It is known that, as in theillustrated case, the majority of patients, approximately 92%, have systemicmanifestations only subsequently to skin lesions.
cial support: None identified.
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