Year : 2016 | Volume
: 11 | Issue : 3 | Page : 178--180
Department of Medicine, PGIMER, Dr R M L Hospital, New Delhi - 110 001, India
Department of Medicine, PGIMER, Dr R M L Hospital, New Delhi - 110 001
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Arya V. Rheumatology Quiz.Indian J Rheumatol 2016;11:178-180
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Arya V. Rheumatology Quiz. Indian J Rheumatol [serial online] 2016 [cited 2022 Dec 5 ];11:178-180
Available from: https://www.indianjrheumatol.com/text.asp?2016/11/3/178/188277
V Arya , V Dhir
The following questions relate to localized scleroderma and mimics of systemic sclerosis.
All the following features distinguish scleroderma mimics from systemic sclerosis exceptsparing of the faceabsence of calcinosisabsence of nail fold capillary changeslack of auto antibodiesAll the following features are seen in morphea exceptskin lesions become hyper-pigmentedsweat glands and hair follicles are lost in lesional skinhistopathology of skin lesions is identical to that in systemic sclerosisabsence of auto antibodiesWhich of the following treatments has been shown to be most effective for plaque morphea?topical corticosteroidsexcision of plaque with skin graftingoral methotrexateD-penicillamineAll the following are true of linear scleroderma exceptoccurs more commonly in adultslesions may involve muscle and bonesen coup de sabre lesions are associated with Parry-Romberg syndromephototherapy and calcipotriene are both effective treatmentsScleromyxedema is associated withhypothyroidismhyperthyroidismacromegalyparaproteinemiaHistopathological features of the skin lesions of scleromyxedema include all the following exceptfibroblast proliferationmucin deposition in the dermisatrophic hair folliclesincreased elatin fibersWhich of the following treatments is not effective in scleromyxedema?systemic corticosteroidsimatinib mesylatemelphalanthalidomide All of the following drugs can cause sclerosis resembling systemic sclerosis exceptbuprenorphinebleomycinL-tryptophanpentazocineAll the following are features of scleredema adultorum of Buschke exceptsparing of hands and feetwaxy papular deposits on the skinnon-pitting edemadeposition of acid mucopolysaccharides in the dermisThe "groove sign" is seen innephrogenic systemic fibrosischronic graft versus host diseaseSchulman diseaseatrophoderma of Pasini and Pierini
View AnswerAnswers to the Quiz on pages 56-57
1a, 2d, 3c, 4a, 5d, 6d, 7b, 8a, 9b, 10c
Facial involvement may occur in scleroderma mimics also; in addition, Raynaud phenomenon is not seen in scleroderma mimics.A variety of auto antibodies like ANA, anti-histone, anti-nucleosome, anti U3-RNP, anti fibrillin and anti cardiolipin are seen in patients with morphea.
Takehara K, Sato S. Localized scleroderma is an autoimmune disorder. Rheumatology (Oxford) 2005;44:274-9Several therapies have been used. These include topical tacrolimus, imatinib, bosentan, methotrexate, UVA, mycophenolate and retinoic acid.
Kroft EB, Creemers MC, ven den Hoogen FH, et al. Effectiveness, side effects and period of remission after treatment with methotrexate in localized scleroderma and related sclerotic skin diseases: an inception cohort study. Br J Dermatol 2009;160:1075-82Most commonly seen in children and adolescents. Lesions involving the face or scalp: en coup de sabre lesions. Parry-Romberg syndrome: hemi-facial atrophy of tissues below the forehead with little involvement of the skin. This disorder and linear scleroderma frequently co-exist and are often bilateral.
Tollefson M M, Witman P M. En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol 2007;56:257-63. About 83% of patients with scleromyxedema have an IgG paraprotein.
Boin F, Hummers LK. Scleroderma-like fibrosing disorders. Rheum Dis Clin North Am. 2008;34:199-220.Elastin fibers are decreased and the epidermis may be thinned out. Other treatments found to be effective include intravenous immunoglobulin, stem cell transplantation, bortezomib, cyclosporine and cyclophosphamide.
Littlejohn GO, Urowitz MB, Smythe HA, et al. Radiographic features of the hand in diffuse idiopathic skeletal hyperostosis (DISH): comparison with normal subjects and acromegalic patients. Radiology 1981;140:623-9. Pentazocin, an injectable opioid which is often abused, can cause severe, extensive and treatment resistant sclerosis.
Hertzman A, Toone E, Resnik CS. Pentazocine induced myocutaneous sclerosis. J Rheumatol 1986;13:210-4.Waxy papular skin deposits are a feature of scleromyxedema not scleredema!Schulman disease or eosinophilic fasciitis may cause this sign: a dimpled appearance of the medial aspect of the upper arm with elevation where the collapsed brachial vein leaves an induration.
Al Hammadi A, Turchin I. Groove sign and eosinophilic fasciitis. J Cutan Med Surg. 2008;12:49
Atrophoderma of Pasini and Pierini is a form of morphea, with depressed hyperpigmented plaques with a sharp border and lacking inflammation.