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Rheumatology Advance Access originally published online on August 25, 2006
Rheumatology 2007 46(1):80; doi:10.1093/rheumatology/kel264
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© The Author 2006. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Interstitial granulomatous dermatitis (Ackerman's syndrome) in SLE presenting with the ‘rope sign’

S. Dubey and P. Merry

Department of Rheumatology, Norfolk and Norwich University Hospital, Colney Lane, Norwich, NR4 7UY, UK

Correspondence to: S. Dubey, Department of Rheumatology, Norfolk and Norwich University Hospital, Colney Lane, Norwich, NR4 7UY, UK. E-mail: s.dubey{at}nhs.net

A 50-yr-old man presented with synovitis of the metacarpophalangeal joints, Raynaud's phenomenon, recurrent pericarditis and a linear palpable rash in both axillae. Investigations: erythrocyte sedimentation rate: 16 mm [normal range (nr) 1–12], C-reactive protein: 18 mg/dl (nr 0–10), rheumatoid factor (RF) and anti nuclear antibody (ANA) negative, double stranded DNA >300 IU/ml (nr <10). Echocardiogram revealed thickened pericardium. He was diagnosed as ANA negative systemic lupus erythematosus [although he did not fulfil the American College of Rheumatology (ACR) criteria]. He responded well to steroids, but not to hydroxychloroquine. The rash was biopsied and methotrexate was commenced.

The biopsy revealed palisading dermal infiltration with histiocytes, with foci of collagen necrosis/necrobiosis along with vasculitis and venulitis. Interstitial granulomatous dermatitis (IGD, Ackerman's syndrome) was diagnosed.

Ackerman's syndrome, first described in 1993 [1], is a rare disorder characterized by the combination of arthritis and a pathognomonic rash (linear strands or erythematous, palpable cords, Fig. 1)—‘the rope sign’. Its associations include connective tissue disease (particularly SLE), rheumatoid arthritis, autoimmune thyroiditis, carcinomas and drug reactions. Histology reveals an interstitial and palisading granulomatous dermatitis associated with piecemeal fragmentation of collagen and elastic fibres [2].


Figure 1
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  		FIG. 1. Left axillary rash depicting the ‘rope sign’.

 
Our patient's symptoms are well-controlled on methotrexate. This is a rare syndrome and recognition of the signs may enable early diagnosis.

The authors have declared no conflicts of interest.


   References
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 References
 

  1. Ackerman AB, Guo Y, Vitale P, et al. (1993) Clues to diagnosis in dermopathology(ASCB Press, Chicago, IL) pp. 309–12.
  2. Crowson AN and Magro C. (2004) Interstitial granulomatous dermatitis with arthritis. Human Pathology 35:779–80.[CrossRef][Web of Science][Medline]
Accepted 27 June 2006


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This Article
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