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E-IJD® - CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 2  |  Page : 207
Acquired reactive perforating collagenosis – A rare cutaneous manifestation of anti-MDA5 dermatomyositis


From the Department of Dermatology, Hospital Queen Elizabeth, Karung Berkunci No. 2029, Kota Kinabalu, Sabah, Malaysia

Date of Web Publication13-Jul-2022

Correspondence Address:
Teck Sheng Gan
From the Department of Dermatology, Hospital Queen Elizabeth, Karung Berkunci No. 2029, Kota Kinabalu, Sabah
Malaysia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_1031_20

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How to cite this article:
Gan TS, Michelle Voo SY. Acquired reactive perforating collagenosis – A rare cutaneous manifestation of anti-MDA5 dermatomyositis. Indian J Dermatol 2022;67:207

How to cite this URL:
Gan TS, Michelle Voo SY. Acquired reactive perforating collagenosis – A rare cutaneous manifestation of anti-MDA5 dermatomyositis. Indian J Dermatol [serial online] 2022 [cited 2022 Aug 9];67:207. Available from: https://www.e-ijd.org/text.asp?2022/67/2/207/350828




Sir,

A 51-year-old woman was referred to our clinic for evaluation of a 4-month history of skin lesions of the face and upper extremities as well as ulcers on the lower extremities. She presented with progressive worsening dyspnoea and dry cough 5 months ago and was diagnosed with interstitial lung disease (ILD). She also had night fever, anorexia and significant weight loss. Physical examination revealed mid-facial erythematous patches and multiple ulcers with central serosanguinous crust at the dorsum aspect of the right hand [Figure 1]a, right elbow [Figure 1]b and posterior right thigh. Her proximal muscles power was reduced. Her myositis-specific antibodies results revealed strongly positive anti-MDA5. Other relevant investigation results such as CK, LDH, ANA, anti-dsDNA and ENA were not significant. Skin biopsy of the ulcer at the right arm showed degenerated basophilic collagen with vertically orientated collagen fibres that traversed the epidermis, neutrophil infiltration with endothelial damage of the medium size vessel and mucin deposition [Figure 2]a and [Figure 2]b. Her electromyography test showed increased insertional activity, complex repetitive discharges and positive sharp waves at left deltoid. A final diagnosis of anti-MDA5 dermatomyositis was made.
Figure 1: Multiple well demarcated punched-out round ulcers with violaceous border and central serosanguinous crust on: (a) dorsal aspect of right hand (b) right elbow

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Figure 2: Broadened dermal ridge containing degenerate basophilic collagen, parakeratotic debris and inflammatory cells, haematoxylin & eosin. (a) Magnification x 40 (b) Magnification × 100

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Patients with anti-MDA5 antibodies are more likely to have amyopathic DM associated with distinct systemic manifestations, including rapidly progressive interstitial lung disease (ILD), symmetrical polyarthritis and characteristic cutaneous phenotypes such as skin ulceration, tender palmar papules, oral ulcers and diffuse alopecia.[1] Cutaneous ulcers are often found on extensor surfaces of joints, digital pulps, periungual areas and sun-exposed areas. Factors contributing to the development of ulcers in DM are vasculopathy, vasculitis, interface dermatitis and pruritus.[2]

Acquired reactive perforating collagenosis (ARPC) is a chronic disease characterised by a pruritic hyperkeratotic papulonodular rash with transepidermal elimination of dermal components, including collagen, elastic tissue and keratin. The possible mechanism of ARPC involves an imbalance of the reparative process of altered extracellular matrix and oxygen supply in microangiopathy associated with superficial trauma such as scratching.[3] ARPC is usually associated with diabetes mellitus, chronic renal failure and haemodialysis. It may also represent a paraneoplastic phenomenon.

The association between ARPC and dermatomyositis is not established. Transforming growth factor (TGF)-β is a multifunctional protein that is involved in tissue repair and extracellular matrix (ECM) reorganization. Overexpression of TGF-β3 is associated with transepidermal elimination in ARPC.[4] After muscle injury, TGF-β1 gets involved in muscle repair by regulating ECM. However, overproduction of TGF-β1 induces excessive ECM deposition and fibrosis, eventually leading to incomplete muscle recovery and myopathy.[5]

Anti-MDA-5 DM with ILD is resistant to conventional immunosuppressive therapy and significantly associated with poor survival. Treatment with intravenous immunoglobulin (IVIg) in DM had been successful with clinical and histological improvement as well as reduction of endomysial inflammation. The downregulation of TGF-β1 following IVIg therapy suggests that excessive TGF-β1 contributes to the accumulation of ECM and chronic inflammatory response.[6]

Thus, the authors wish to highlight that ARPC may be one of the cutaneous manifestations of anti-MDA5-associated DM. Further studies are needed to confirm the role of TGF-β and will greatly add value to this relationship.

Acknowledgements

We would like to thank the Director-General of Health Malaysia for his permission to publish this article. We would also like to thank Dr Sandhya A/P Rajaintharan for her contribution to this article.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Hall JC, Casciola-Rosen L, Samedy LA, Werner J, Owoyemi K, Danoff SK, et al. Anti-melanoma differentiation-associated protein 5-associated dermatomyositis: Expanding the clinical spectrum. Arthritis Care Res (Hoboken) 2013;65:1307-15.  Back to cited text no. 1
    
2.
Narang NS, Casciola-Rosen L, Li S, Chung L, Fiorentino DF. Cutaneous ulceration in dermatomyositis: Association with anti-melanoma differentiation-associated gene 5 antibodies and interstitial lung disease. Arthritis Care Res (Hoboken) 2015;67:667-72.  Back to cited text no. 2
    
3.
Kestner RI, Stander S, Osada N, Ziegler D, Metze D. Acquired reactive perforating dermatosis is a variant of prurigo nodularis. Acta Derm Venereol 2017;97:249-54.  Back to cited text no. 3
    
4.
Kawakami T, Soma Y, Mizoguchi M, Saito R. Immunohistochemical analysis of transforming growth factor-β3 expression in acquired reactive perforating collagenosis. Br J Dermatol 2001;144:197-8.  Back to cited text no. 4
    
5.
Kim J, Lee J. Role of transforming growth factor-β in muscle damage and regeneration: Focused on eccentric muscle contraction. J Exerc Rehabil 2017;13:621-6.  Back to cited text no. 5
    
6.
Rosenstein RK, Martires K, Christman M, Terushkin V, Meehan SA, Seminara N, et al. Dermatomyositis, clinically presenting with cutaneous ulcers, with histopathologic evidence of perforating collagenosis. Dermatol Online J 2016;22:13030/qt6qd0q51w.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2]



 

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