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The “rope” sign is knot pathognomonic: Autoimmunity ties the etiologies together

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By Warren R. Heymann, MD
July 8, 2016

The “rope” sign is a dramatic finding presenting as a linear to curvilinear cords (“cordonifom”) on the lateral chest. It was originally described as an uncommon, but specific feature of Interstitial Granulomatous Dermatitis with Arthritis (IGDA), and has been considered pathognomonic for that disorder. That perspective is no longer tenable.

A characteristic case of IGDA was recently reported in a 36 year-old man who developed bilateral tender cords from his arms to his flanks a week after lifting a heavy box. He also had a history of multi-joint stiffness for several months that proved to be rheumatoid arthritis (RA). The skin biopsy demonstrated a dense, diffuse interstitial histiocytic infiltrate, with neutrophils, plasma cells, and eosinophils (1).

Gulati et al reported the case of a 42 year-old woman with SLE who developed erythematous, hot, tender, linear, indurated subcutaneous cords extending from the posterior axillary folds across the chest wall. Although a superficial thrombophlebitis was considered clinically, a biopsy revealed a dermal mixed inflammatory infiltrate and hyalinized and degenerated collagen bundles. Palisading of neutrophils, leukocytoclastic debris, and flame figures caused by the accumulation of degranulated neutrophilic contents were observed, but there was no frank vasculitis or granuloma formation. These features were consistent with SLE-associated palisaded neutrophilic granulomatous dermatitis (PNGD). Subsequently, she was treated with mycophenolate mofetil which prevented any further acute episodes. (2). (I consider IGDA and PNGD part of a spectrum of the same disease, but I will save that discussion for another commentary.)

Mondor disease (MD) is a superficial thrombophlebitis involving the lateral thoracic, thoraco-epigastric, and superficial epigastric veins, more commonly than the dorsal vein of penis and antecubital veins, that may be considered in the clinical differential diagnosis. The etiology is unknown in most cases, although it has been associated with breast cancer. It is usually assessed by ultrasound, resolves spontaneously in a few weeks, and may be treated with NSAIDs (3). Superficial migratory thrombophlebitis, due to a hypercoagulable state in patients with cancer (Trousseau syndrome) (4), may present similarly.

Tomasini described two adults, a 53 year-old woman and a 53 year-old man, with linear or curvilinear indurations of the chest wall that were reminiscent of the rope sign of IGDA. The woman also had lesions of discoid lupus on her forehead and elbows. Biopsies of the “cordoniform” lesions from both patients were diagnostic of morphea with a lymphoplasmacytic infiltrate at the dermal-subcutaneous border. Both patients were found to have Borrelia in their specimens by immunohistochemistry and focus floating microscopy; the man had Borrelia afzelii identified by polymerase chain reaction. The woman was treated was chloroquine and prednisone, with the lupus lesions resolving within 2 months, and the cords almost completely regressing by 6 months. The man, whose medical history was only remarkable for a monoclonal gammopathy of underdetermined significance, was not treated; his lesions regressed spontaneously within a year (5). Although the authors speculate about the role of Borrelia as potentially pathogenic in this unusual variant of morphea, this theory remains controversial (6).

Tying this all together, it is clear that the rope sign is not pathognomonic for IGDA and may be associated with other autoimmune diseases such as RA or lupus, or it may be morphea itself.

1. Worsnop FS, Ostlere L. Interstitial granulomatous dermatitis with arthritis presenting with the rope sign. Clin Exp Dermatol 2013; 38: 564-5.
2. Gulati A, et al. Palisaded neutrophilic granulomatous dermatitis associated with systemic lupus erythematosus presenting with the burning rope sign. J Am Acad Dermatol 2009; 61: 711-4.
3. Bhattacharjee S, et al. Mondor’s disease. Indian J Dermatol Venereol Leprol 2014; 80: 173-5.
4. Ikushima S, et al. Trousseau’s syndrome: cancer-associated thrombosis. Jpn J Clin Oncol 2016; 46: 204-8.
5. Tomasini C. Cordoniform morphea: A clinicopathologic study of two cases presenting with the rope sign. J Cutan Pathol 2016; 43: 613-22.
6. Heymann WR, Ellis DL. Borrelia burgdorferi infections in the United States. J Clin Aesthet Dermatol 2012; 5: 18-28.

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