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CORRESPONDENCE |
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| Year : 2022 | Volume
: 67
| Issue : 6 | Page : 777-779 |
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| Unilateral heliotrope rash, inverse gottron's papules and anti-MDA5 antibody positivity: A triad suggesting lung involvement |
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Adhyatm Bhandari1, T Muhammed Razmi1, Sanjeev Handa1, Uma Nahar Saikia2, Rahul Mahajan1
1 From the Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| Date of Web Publication | 23-Feb-2023 |
Correspondence Address:
Rahul Mahajan
From the Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijd.ijd_619_22
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How to cite this article:
Bhandari A, Razmi T M, Handa S, Saikia UN, Mahajan R. Unilateral heliotrope rash, inverse gottron's papules and anti-MDA5 antibody positivity: A triad suggesting lung involvement. Indian J Dermatol 2022;67:777-9 |
How to cite this URL:
Bhandari A, Razmi T M, Handa S, Saikia UN, Mahajan R. Unilateral heliotrope rash, inverse gottron's papules and anti-MDA5 antibody positivity: A triad suggesting lung involvement. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 23];67:777-9. Available from: https://www.e-ijd.org/text.asp?2022/67/6/777/370329 |
Sir,
Heliotrope rash is one of the early characteristics presenting signs of dermatomyositis. We report a case of unilateral heliotrope rash with inverse gottron's papules and anti-melanoma differentiation-associated gene 5 (MDA5) antibody positivity in a patient with amyopathic dermatomyositis. The unilateral periorbital swelling led to a delay in the diagnosis until the patient manifested later with characteristic cutaneous signs of dermatomyositis.
A 39-year-old female presented to us with asymptomatic unilateral periorbital swelling of 6-month duration. There was no history of photosensitivity, Raynaud's phenomenon, puffy fingers, muscle weakness, respiratory discomfort or any other systemic complaint. Mucocutaneous examination revealed marked erythema and pitting edema of the skin over the right periorbital region with an ill-defined border [Figure 1]. Her nail fold capillaries appeared normal. Musculoskeletal and neurological examinations were within normal limits. | Figure 1: Unilateral heliotrope rash - Erythematous to violaceous rash involving the periorbital area of the right eye and the right malar area
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She was investigated with a differential diagnosis of solid facial edema (Morbihan disease), orofacial granulomatosis and dermatomyositis. Complete blood counts and biochemistry were normal. Antinuclear antibody (ANA) was negative. Serum aldolase level was 6.1 U/L (0–7U/L), and N-acetyl-cysteine-(NAC)-activated creatine kinase (CK-NAC) level was 268 U/L (26–308 U/L). Histopathological examination of the periorbital skin revealed epidermal atrophy, upper dermal edema with lymphomononuclear infiltrates around appendages without mucin deposition.
A month later, she presented with typical Gottron's papules [Figure 2]a over knuckles and palmar digital creases [“inverse Gottron's papules,” [Figure 2]b]. Erythematous to violaceous rash with edema was seen on the V area and nape of the neck, extensor aspect of upper arm (shawl sign), upper back [Figure 2]c, and extensor aspect of upper thigh (holster sign). Cutaneous ulcerations were noted over Gottron's papules and the rash. Her motor power was normal. Anti-Jo1, anti-Mi2, anti-SRP, anti-PL-7, anti-PL-12, anti-PM-Scl, anti-Ku, anti-TIF1-γ and anti-U1 ribonucleoprotein (RNP) antibodies were negative, while anti-MDA5 antibody was positive. Muscle enzymes were again normal: CK-NAC, 238.6U/L (26 – 308 U/L) and aldolase, 5.9 U/L (0–-7 U/L). Electromyogram did not show any changes suggestive of myopathy. Electrocardiogram findings were within normal limits. Histopathology from Gottron's papules showed focal basal cell changes, dermal mucin deposition and features of vasculitis, consistent with the diagnosis of dermatomyositis [Figure 2]d. Malignancy workup was normal. High resolution computed tomography of the chest showed areas of bilateral subpleural patchy consolidation with basal predominance, suggestive of ILD. A final diagnosis of amyopathic dermatomyositis with ILD was made. She was prescribed oral prednisolone (60 mg/day) along with topical mometasone furoate 0.1% w/w cream. Heliotrope rash and other cutaneous signs improved gradually over two months. | Figure 2: Clinicopathologic findings at first month follow up visit - Gottron's papules (a, arrowhead). Inverse Gottron's papules (b, arrowhead). Confluent macular violaceous erythema over upper back with punched out ulcers (arrowhead and inset) noted on the shoulders (c). Histopath image of Gottron's papule showing interface changes with focal basal cell vacuolization and mild perivascular infiltrates along with fibrin deposition in the papillary dermis (d) (H and E × 200)
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A unilateral heliotrope may be an indicator of anti-MDA5 antibody positivity, which is classically known to be associated with amyopathic dermatomyositis and ILD.[1] Kume et al.[2] reported three cases of unilateral heliotrope rash with ILD who tested positive for anti-MDA5 antibodies. In another report with unilateral heliotrope rash, a patient was detected positive for anti-MDA5 antibody.[3]
Anti-MDA5 antibody-positive dermatomyositis presents with several unique cutaneous features like skin ulceration, inverse Gottron's papules, alopecia, and panniculitis. In addition, 90% of patients positive for anti-MDA5 antibody are ANA negative.[4] In a report by Irie K et al.,[5] six out of seven patients with inverse Gottron's papules had positive anti-MDA5 antibodies with features of ILD.
Persistent unilateral swelling of the periorbital area should prompt a physician to think of heliotrope rash. Unilateral heliotrope rash, inverse gottron's papules and anti-MDA5 antibody positivity have been reported to be associated with ILD. Careful examination helps in early identification of dermatomyositis and ILD changes, therefore, improving the overall survival rate and quality of life.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
 References | |  |
| 1. | Moghadam-Kia S, Oddis CV, Sato S, Kuwana M, Aggarwal R. Anti-melanoma differentiation-associated Gene 5 is associated with rapidly progressive lung disease and poor survival in US patients with amyopathic and myopathic dermatomyositis. Arthritis Care Res (Hoboken) 2016;68:689-94.  |
| 2. | Kume M, Arase N, Okiyama N, Koguchi-Yoshioka H, Tada T, Saruban H, et al. Unilateral heliotrope rash: A warning sign for anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis. Rheumatology (Oxford) 2021;60:e134-5. |
| 3. | Lam SC, Yuen HKL. Unilateral eyelid swelling as a sign of antimelanoma differentiation-associated gene 5 (anti-MDA5)-antibody-positive dermatomyositis. Ophthalmic Plast Reconstr Surg 2018;34:e209-11. |
| 4. | Fiorentino D, Chung L, Zwerner J, Rosen A, Casciola-Rosen L. The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): A retrospective study. J Am Acad Dermatol 2011;65:25-34. |
| 5. | Irie K, Matsumura N, Hoshi M, Yamamoto T. Inverse Gottron's papules in patients with dermatomyositis: An underrecognized but important sign for interstitial lung disease. Int J Dermatol 2021;60:e62-5. |
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