|Year : 2019 | Volume
| Issue : 2 | Page : 98-101
Chronic plaque psoriasis on diffuse systemic sclerosis: a rare association
Parul Chojer, Bharat Bhushan Mahajan
Department of Dermatology, Venereology and Leprology, Government Medical College, Amritsar, Punjab, India
|Date of Submission||19-Jun-2018|
|Date of Acceptance||11-Apr-2019|
|Date of Web Publication||03-Jul-2019|
Currently working as Senior Resident, Sanjay Gandhi Memorial Hospital, New Delhi, 110083; H no. 119 Phulkian Enclave, Patiala, 147001, Punjab
Source of Support: None, Conflict of Interest: None
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 2020 Jan 25];39:98-101. Available from: http://www.ejdv.eg.net/text.asp?2019/39/2/98/262036
| Introduction|| |
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 ,. Psoriasis, an autoimmune chronic inflammatory skin disease is characterized by infiltration of mononuclear cells and proliferation of keratinocytes . 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 ,. 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|| |
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 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|| |
Systemic sclerosis is a multisystem autoimmune connective tissue disease of unknown etiology  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 ,,, with the largest series in the literature involving only three cases ,. Of particular interest is the study by Harrison et al. , 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 , 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.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]