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RHEUMATOLOGY QUIZ |
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Year : 2016 | Volume
: 11
| Issue : 3 | Page : 178-180 |
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Rheumatology Quiz
Vivek Arya
Department of Medicine, PGIMER, Dr R M L Hospital, New Delhi - 110 001, India
Date of Web Publication | 11-Aug-2016 |
Correspondence Address: Vivek Arya Department of Medicine, PGIMER, Dr R M L Hospital, New Delhi - 110 001 India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0973-3698.188277
How to cite this article: Arya V. Rheumatology Quiz. Indian J Rheumatol 2016;11:178-80 |
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 except
- sparing of the face
- absence of calcinosis
- absence of nail fold capillary changes
- lack of auto antibodies
- All the following features are seen in morphea except
- skin lesions become hyper-pigmented
- sweat glands and hair follicles are lost in lesional skin
- histopathology of skin lesions is identical to that in systemic sclerosis
- absence of auto antibodies
- Which of the following treatments has been shown to be most effective for plaque morphea?
- topical corticosteroids
- excision of plaque with skin grafting
- oral methotrexate
- D-penicillamine
- All the following are true of linear scleroderma except
- occurs more commonly in adults
- lesions may involve muscle and bones
- en coup de sabre lesions are associated with Parry-Romberg syndrome More Details
- phototherapy and calcipotriene are both effective treatments
- Scleromyxedema is associated with
- hypothyroidism
- hyperthyroidism
- acromegaly
- paraproteinemia
- Histopathological features of the skin lesions of scleromyxedema include all the following except
- fibroblast proliferation
- mucin deposition in the dermis
- atrophic hair follicles
- increased elatin fibers
- Which of the following treatments is not effective in scleromyxedema?
- systemic corticosteroids
- imatinib mesylate
- melphalan
- thalidomide
- All of the following drugs can cause sclerosis resembling systemic sclerosis except
- buprenorphine
- bleomycin
- L-tryptophan
- pentazocine
- All the following are features of scleredema adultorum of Buschke except
- sparing of hands and feet
- waxy papular deposits on the skin
- non-pitting edema
- deposition of acid mucopolysaccharides in the dermis
- The "groove sign" is seen in
- nephrogenic systemic fibrosis
- chronic graft versus host disease
- Schulman disease
- atrophoderma of Pasini and Pierini
View Answer
Answers 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;44:274-9 - Several 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;160:1075-82 - Most 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.
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