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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 39  |  Issue : 2  |  Page : 98-101

Chronic plaque psoriasis on diffuse systemic sclerosis: a rare association


Department of Dermatology, Venereology and Leprology, Government Medical College, Amritsar, Punjab, India

Date of Submission19-Jun-2018
Date of Acceptance11-Apr-2019
Date of Web Publication03-Jul-2019

Correspondence Address:
Parul Chojer
Currently working as Senior Resident, Sanjay Gandhi Memorial Hospital, New Delhi, 110083; H no. 119 Phulkian Enclave, Patiala, 147001, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ejdv.ejdv_26_18

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  Abstract 


Diffuse systemic sclerosis is a multisystem autoimmune disease that is characterized by generalized vascular involvement that results in tissue necrosis and secondary fibrosis. Psoriasis is a chronic inflammatory skin disease. Both of these diseases are autoimmune in origin. Psoriasis is, however, rarely associated with systemic sclerosis, but few cases have been reported, and a common genetic and immunological background shared by these diseases has been suggested. Here we report a case of a 20-year-old female patient with diffuse systemic sclerosis who developed chronic plaque psoriasis with Psoriasis area and severity Index (PASI) of 14.0 and body surface area of 32% with diffuse skin sclerosis and respiratory involvement.

Keywords: association, chronic plaque psoriasis, diffuse systemic sclerosis


How to cite this article:
Chojer P, Mahajan BB. Chronic plaque psoriasis on diffuse systemic sclerosis: a rare association. Egypt J Dermatol Venerol 2019;39:98-101

How to cite this URL:
Chojer P, Mahajan BB. Chronic plaque psoriasis on diffuse systemic sclerosis: a rare association. Egypt J Dermatol Venerol [serial online] 2019 [cited 2019 Sep 23];39:98-101. Available from: http://www.ejdv.eg.net/text.asp?2019/39/2/98/262036




  Introduction Top


Vascular changes characterized by functional and structural abnormalities of microcirculation play a central role in the pathogenesis of collagen vascular disorders. Diffuse systemic sclerosis is a connective tissue disease characterized by fibroblast proliferation and excessive accumulation of extracellular matrix by activated fibroblasts in affected skin as well as various internal organs [1],[2]. Psoriasis, an autoimmune chronic inflammatory skin disease is characterized by infiltration of mononuclear cells and proliferation of keratinocytes [3]. It is thought to be related to a polygenic predisposition and a number of environment trigger factors like stress, trauma, infection, and drugs, and it shares both immunological and genetic predisposing factors with other autoimmune disorders, especially systemic lupus erythematosus and rheumatoid arthritis [4],[5]. Although association between psoriasis and systemic sclerosis is rare, yet chronic plaque psoriasis developing on diffuse systemic sclerosis is a rarity and hence is being reported.


  Case report Top


A 20-year-old female patient presented with multiple erythematous scaly plaques over scalp, trunk, bilateral upper, and lower limbs since one and half years ([Figure 1] and [Figure 2]). She also had history of Raynaud’s phenomenon and digital ulceration since 5 years which was followed by tightness over face ([Figure 3]), trunk, and bilateral upper and lower limbs; arthralgia; difficulty in breathing; and diffuse hyperpigmentation of skin after about a year. For this, she took multiple treatments but was not relieved. On examination she had sclerodactyly, bound down skin, flexion deformity, Modified Rodnan Skin Sclerosis score 30, Psoriasis area and severity Index (PASI) 14 with body surface area 32%, and positive candle wax and Auspitz sign. Nailfold capillaroscopy revealed late scleroderma pattern with bizarre capillaries, avascular areas, and extensive vascular architectural disorganization ([Figure 4]). Laboratory tests revealed hemoglobin 7.5 g/dl, total leukocyte count 3000/mm3, dimorphic picture on peripheral blood film, erythrocyte sedimentation rate-75, and presence of antinuclear antibody and Scl70 antibodies by enzyme-linked immunosorbent assay (strongly positive). Fasting blood sugar, serum lipid profile, and liver and renal function tests were within normal limits, suggesting no evidence of metabolic syndrome. Pulmonary function tests revealed ESA obstruction with peak expiratory flow less than 70%. Peak expiratory flow and high-resolution computed tomography showed emphysematous changes with fibrotic strands in right lobe. Barium study results were normal. Two skin biopsies were done, and the biopsy showed features of broad collagen bundles and entrapped skin adnexal structures, and the other biopsy from erythematous plaque revealed parakeratosis, acanthosis, elongation of rete ridges, and mild epidermal spongiosis suggestive of systemic sclerosis and psoriasis ([Figure 5]). Radiological examination of bilateral hands and feet revealed terminal tuft erosions suggestive of acroosteolysis ([Figure 6] and [Figure 7]). She was started on symptomatic and supportive therapies in the form of calcium channel blocker, vitamin E, cilostazol, and emollients. Specific therapy in the form of injection methotrexate 15 mg weekly was started. The patient was followed up, where psoriatic lesions started improving in 2–4 weeks, and skin symptoms improved over a period of 6–9 months, and patient is still on methotrexate with regular follow-up.
Figure 1 Gross facial examination showing perioral furrowing.

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Figure 2 Photograph showing erythematous scaly plaques over the dorsum of bilateral hands and sclerodactyly.

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Figure 3 Photograph showing erythematous scaly plaques over dorsum of bilateral feet and subungual hyperkeratosis of left great toe nail.

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Figure 4 Examination of the nail at ×20 magnification. Nailfold examination under polarized light shows avascular areas (blue arrow) and bizarre capillaries (red arrow).

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Figure 5 Photomicrograph of the patient showing thick collagen bundles in dermis and few adnexal structures (H&E ×400).

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Figure 6 Radiological examination of bilateral hands revealing terminal tuft erosions.

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Figure 7 Radiological examination of bilateral feet showing terminal tuft erosions.

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  Discussion Top


Systemic sclerosis is a multisystem autoimmune connective tissue disease of unknown etiology [6] characterized by generalized vascular involvement that results in tissue ischemia and secondary fibrosis. There are two main types: limited and diffuse. Very few cases of concomitant psoriasis and systemic sclerosis have been reported [7],[8],[9], with the largest series in the literature involving only three cases [9],[10]. Of particular interest is the study by Harrison et al. [10], in which the prevalence of psoriasis in patients with systemic sclerosis was 5.3%. Some authors have suggested that the two entities may share a common genetic and immunologic basis [9],[10] and although the HLA DRw52 serotype has been associated with both systemic sclerosis and psoriasis, studies have failed to establish an HLA pattern in patients with both diseases. There are few possible explanations for this observation. First, it may have occurred by chance. Second possible explanation is that the two conditions may share a common etiology. A link between psoriasis and autoimmunity is suggested by higher-than-expected prevalence of ANA in patients with psoriasis. It has been suggested that both psoriasis and autoimmune diseases including systemic sclerosis may result from a common underlying defect in the immune system. It is therefore interesting to note that both conditions share certain pathological features, such as dermal inflammation, abnormalities of vascularization, acroosteolysis, and possibly abnormalities of the nailfold capillaries. As vascular abnormalities are seen in both these diseases, it can be considered that several cytokines released following vascular injury may play a role in their induction. Thus, such case of association of psoriasis with collagen vascular disorder especially systemic sclerosis is rare, and this case has been reported because of its rarity.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Krieg T, Meurer M. Systemic sclerosis.Clinical and pathophysiologic aspects. J Am Acad Dermatol 1998; 18:457–481.  Back to cited text no. 1
    
2.
LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr, Rowell N, Wollheim F. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988; 15:202–205.  Back to cited text no. 2
    
3.
Arbiser JL. Angiogenesis and the skin: a primer. Am Acad Dermatol 1996; 34:486–497.  Back to cited text no. 3
    
4.
Pedraz J, Daudén E, Psoriasis I. Etiopatogenia, clínica y diagnostic. Manual de Dermatología. Madrid: Aula Médica; 2010. 171–184  Back to cited text no. 4
    
5.
Wolf K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ. Fitzpatrick’s dermatology in general medicine. 7th ed. USA: The McGraw Hill companies; 2009. 2402.  Back to cited text no. 5
    
6.
Costner M, Grau R. Update on connective tissue diseases in dermatology. Semin Cutan Med Surg 2006; 25:207–220.  Back to cited text no. 6
    
7.
Korotkii NG, Sharanova GI, Udzhukhu V, Antonova NA, Panchenko MA. A case of severe form of psoriasis and systemic scleroderma. Vestn Dermatol Venerol 1984; 5:48–49.  Back to cited text no. 7
    
8.
Khamaganova IV. Association of scleroderma and psoriasis. Vestn Dermatol Venerol 1986; 6:66–68.  Back to cited text no. 8
    
9.
Yamamoto T, Katayama I, Nishioka K. A rare association of systemic sclerosis with psoriasis vulgaris. J Dermatol 2000; 27:346–349.  Back to cited text no. 9
    
10.
Harrison B, Herrick A, Griffiths C. Psoriasis and diffuse systemic sclerosis: a report of three patients. Rheumatology (Oxford) 2000; 39:213–215.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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