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Rowell syndrome

Authors: Riyad NH Seervai, MD/PhD Student, Baylor College of Medicine, Houston, Texas, USA; Claire Jordan Wiggins, Medical Student, Baylor College of Medicine, Houston, Texas, USA. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. June 2020.


What is Rowell syndrome?

Rowell syndrome is a rare entity that involves erythema multiforme-like lesions coexisting with lupus erythematosus (LE) along with characteristic immunological findings.

The syndrome was first defined in 1963 by Rowell, Beck, and Anderson who reported four out of 120 patients with chronic discoid LE with erythema multiforme-like lesions on the arms, legs, face, and chest, coinciding with a speckled pattern of antinuclear antibody (ANA), positive rheumatoid factor, and a precipitating antibody to saline extract from human tissue (anti-Sj-T) [1].

Rowell syndrome is currently classified as a subtype of chronic cutaneous lupus erythematosus [2].

Who gets Rowell syndrome?

Rowell syndrome has been reported in patients with systemic and discoid lupus erythematosus. Of the 96 reported cases, 18 were from India [3].

  • The majority of cases are in women aged 20–70 years old [1].
  • The ratio of affected women to men is 8:1 [3].
  • Indian patients have a median age 23 compared to age 32 worldwide and are less likely to have discoid lupus (5.6% versus 21.1% worldwide) [3].
  • Rowell syndrome is often preceded by a history of LE, but it has been reported to be its first sign [4–6].

What causes Rowell syndrome?

The pathogenesis of LE involves genetics and environmental factors [7].

  • The higher incidence in females has been attributed to oestrogen signalling, the composition of the microbiome, and increased levels of toll-like receptor TLR7 in immune cells [8,9].
  • Rowell syndrome been induced by drugs including terbinafine [10–12], omeprazole [12–14], norfloxacin [15], and sodium valproate [16].
  • It has also been provoked by sun exposure [3,17,18].

What are the clinical features of Rowell syndrome?

Rowell syndrome is characterised by erythema multiforme-like lesions in a patient with LE.

  • The erythema multiforme-like lesions are painful, pruritic, annular or targetoid, erythematous, red-to-violet plaques or blisters.
  • They are found on the chest and back, arms and legs, hands and feet, and face.
  • Other features of LE include:

What are the complications of Rowell syndrome?

Complications are mostly due to systemic LE, such as haematological and renal involvement, or other conditions [3,19]. Stevens Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) has been reported [20].

Rowell syndrome has also been associated with:

How is Rowell syndrome diagnosed?

The specific features of Rowell syndrome have been controversial [21,25,26,29–35].

It is currently considered to be a distinct type of chronic cutaneous LE with the following criteria [2].

1. Major criteria (all required)

a. Presence of chronic cutaneous LE

b. Erythema multiforme-like lesions (typical or atypical target lesions)

c. ≥1 positive speckled ANA (present in >90% [27,28]), anti-Ro/SSA, or anti-La/SSB antibodies

d. Negative direct immunofluorescence.

2. Minor criteria (one required)

a. Absence of infectious or pharmacological triggers

b. Lesions are not in acral or mucosal areas (the typical sites of erythema multiforme)

c. Presence of at least one additional criterion for systemic LE.

Histology shows a periadnexal lymphocytic infiltrate and periadnexal CD123+ plasmacytoid dendritic cells [32].

What is the differential diagnosis for Rowell syndrome?

The differential diagnosis for Rowell syndrome includes [2,21,31,32,34]:

What is the treatment for Rowell syndrome?

The treatment of Rowell syndrome includes the range of medicines used to treat LE, including:

What is the outcome for Rowell syndrome?

Most patients with Rowell syndrome respond well to therapy.

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References

  1. Rowell NR, Beck JS, Anderson JR. Lupus Erythematosus and Erythema Multiforme-Like Lesions. A Syndrome with Characteristic Immunological Abnormalities. Arch Dermatol 1963; 88: 176–80. DOI: 10.1001/archderm.1963.01590200064012. PubMed Central
  2. Torchia D, Romanelli P, Kerdel FA. Erythema Multiforme and Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Associated with Lupus Erythematosus. J Am Acad Dermatol 2012; 67: 417–21. DOI: 10.1016/j.jaad.2011.10.012. PubMed
  3. Sharma YK, Chauhan S. Overlap Syndrome with Rowell's Syndrome, Antiphospholipid Syndrome, Primary Sterility, and Sensorineural Hearing Loss: A Case Report, Brief Review, and Analysis of Cases of Rowell's Syndrome Reported from India and Abroad. Indian J Dermatol 2018; 63: 418–23. DOI: 10.4103/ijd.IJD_437_17. PubMed
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  15. Baroni A, Piccolo V, Russo T, Cozzolino D, et al. Norfloxacin-induced Subacute Cutaneous Lupus with Erythema Multiforme-Like Lesions: The Enigma of the Rowell Syndrome. J Dtsch Dermatol Ges 2014; 12: 1039–42. DOI: 10.1111/ddg.12392. PubMed
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  17. Ahluwalia J, Marsch A. Photosensitivity and Photoprotection in Patients with Lupus Erythematosus. Lupus 2019; 28: 697–702. DOI: 10.1177/0961203319839486. PubMed
  18. Alkul S, Behrens E, Stetson C. Rowell Syndrome with Recurrence from Photoexacerbation: A Case Report. SAGE Open Med Case Rep 2019; 7: 2050313x19847337. DOI: 10.1177/2050313x19847337. PubMed
  19. Shahid S, Khan M, Qadar LT, Akmal M, Jamal A. The First Case of Rowell Syndrome with Lupus Nephritis and Lobar Pneumonia in a Male Child Reported in Pakistan. Cureus 2019; 11: e4604. DOI: 10.7759/cureus.4604. PubMed
  20. Mandelcorn R, Shear NH. Lupus-Associated Toxic Epidermal Necrolysis: A Novel Manifestation of Lupus? J Am Acad Dermatol 2003; 48: 525–9. DOI: 10.1067/mjd.2003.107. PubMed
  21. Aydogan K, Karadogan S, Balaban Adim S, Tunali S. Lupus Erythematosus Associated with Erythema Multiforme: Report of Two Cases and Review of the Literature. J Eur Acad Dermatol Venereol 2005; 19: 621–7. DOI: 10.1111/j.1468-3083.2005.01233.x. PubMed
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