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Observation
September 2016

Unilateral Gottron Papules in a Patient Following a Stroke: Clinical Insights Into the Disease Mechanisms and Pathophysiology of Cutaneous Dermatomyositis

Author Affiliations
  • 1Department of Dermatology, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts
 

Copyright 2016 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.

JAMA Dermatol. 2016;152(9):1062-1063. doi:10.1001/jamadermatol.2016.1537

Dermatomyositis (DM) is an autoimmune disorder with a characteristic pattern of skin and muscle disease. Preclinical studies have implicated abnormal stretch receptor signaling and external trauma as necessary components for the expression of certain cutaneous manifestations.1,2 These studies correlate with clinical findings, as DM preferentially affects UV-irradiated skin and frequently traumatized sites, such as over bony prominences on the hands and elbows. We evaluated a patient with residual hemiparesis from a stroke who developed cutaneous DM with a strikingly unilateral pattern on the hands: prominent disease on her functional side with sparing of her immobilized side. Our observation provides clinical evidence supporting the idea that external trauma, movement, and stretch may be requisite for the genesis of certain skin lesions in DM.

Report of a Case

A woman in her 60s presented for evaluation of a persistent pruritic eruption on her left hand. She had experienced a stroke 2 years before, which left her with residual right-sided hemiparesis. Her medical history was otherwise unremarkable.

On examination, discrete hyperkeratotic, lichenoid, erythematous papules were present on her left proximal and distal interphalangeal joints (Figure, A). Prominent nailfold capillary dilatation and dropout as well as cuticular dystrophy were also present on the left hand. Her right hand, by comparison, was unaffected (Figure, B). The remainder of her examination demonstrated bilateral violet periorbital discoloration, diffuse scalp erythema, and thinning of her scalp hair density. A biopsy was performed on her left index finger, which showed hyperkeratosis and interface dermatitis compatible with connective tissue disease. Together, her clinical and histopathologic findings were consistent with DM.

Figure.
Unilateral Gottron Papules in a Patient Following a Stroke
Unilateral Gottron Papules in a Patient Following a Stroke

A, Hyperkeratotic erythematous papules (Gottron papules) on the proximal and distal interphalangeal joints of the functional left hand. B, The paralyzed right hand was unaffected.

Apart from residual right-sided weakness related to her stroke, she had no other strength deficits. Her serum muscle enzymes and pulmonary function tests were normal, and a comprehensive malignancy screening was negative. She was treated with hydroxychloroquine and methotrexate, and within 3 months had improvement of her left hand lesions as well as her periorbital and scalp erythema.

Discussion

Recently, novel insights explaining the characteristic localization of cutaneous DM have emerged. Specifically, CD44v7, a cell adhesion molecule that plays a role in autoimmunity, was found to be inducible by mechanical stretching and appears to act together with osteopontin, a protein upregulated in DM skin, to produce Gottron papules.1 Increased osteopontin expression in response to mechanical trauma has also been shown.2 These preclinical studies suggest that a key component to the expression of Gottron papules and possibly other DM skin lesions may relate to stretch and mechanical trauma, thereby accounting for our patient’s unilateral presentation, as the lack of movement on her paralyzed limb prevented the development of cutaneous lesions.

Unilateral presentations of psoriasis have also been reported in the setting of hemiparesis.3,4 The observed unilaterality of these cases has been attributed to the absence of the isomorphic response on the immobilized limb. Furthermore, neurogenic inflammation might contribute to the development of psoriatic lesions, and after nerve injury has occurred, this inflammatory trigger is removed. Whether neurogenic inflammation also plays a role in cutaneous DM remains unknown.

Our case report serves as a clinical example that substantiates the idea that stretch and movement may be key pathogenetic factors for the genesis of some cutaneous DM lesions. While classic Gottron papules were seen on our patient’s functional side, these lesions were remarkably absent on her immobilized side. The lack of movement and stretch appeared to preclude the development of Gottron papules on her paralyzed hand. Therefore, an autoimmune diathesis alone may be insufficient for the development of certain cutaneous DM lesions. Stretch, movement, trauma, and possibly neurogenic inflammation may play a requisite role. Hence, the strikingly unilateral pattern seen in this patient who had experienced a stroke provides clinical insight into the pathophysiologic mechanisms responsible for cutaneous DM.

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Article Information

Corresponding Author: Ruth Ann Vleugels, MD, MPH, Autoimmune Skin Disease Program, Department of Dermatology, Brigham and Women’s Hospital, Harvard Medical School, 221 Longwood Ave, Boston, MA 02115 (rvleugels@partners.org).

Published Online: June 8, 2016. doi:10.1001/jamadermatol.2016.1537.

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank Andrea Moy, MD, and Alvaro Laga, MD, Brigham and Women’s Hospital, for providing dermatopathology consultation. They received no compensation for their contributions.

References
1.
Kim  JS, Bashir  MM, Werth  VP.  Gottron’s papules exhibit dermal accumulation of CD44 variant 7 (CD44v7) and its binding partner osteopontin: a unique molecular signature.  J Invest Dermatol. 2012;132(7):1825-1832.PubMedGoogle ScholarCrossref
2.
Denhardt  DT, Noda  M, O’Regan  AW, Pavlin  D, Berman  JS.  Osteopontin as a means to cope with environmental insults: regulation of inflammation, tissue remodeling, and cell survival.  J Clin Invest. 2001;107(9):1055-1061.PubMedGoogle ScholarCrossref
3.
Joseph  T, Kurian  J, Warwick  DJ, Friedmann  PS.  Unilateral remission of psoriasis following traumatic nerve palsy.  Br J Dermatol. 2005;152(1):185-186.PubMedGoogle ScholarCrossref
4.
Veale  D, Farrell  M, Fitzgerald  O.  Mechanism of joint sparing in a patient with unilateral psoriatic arthritis and a longstanding hemiplegia.  Br J Rheumatol. 1993;32(5):413-416.PubMedGoogle ScholarCrossref
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