Rowell's syndrome L93.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 09.05.2021

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Synonym(s)

Lupus erythematosus and erythema multiforme-like syndrome; Rowell`s syndromes; Rowell syndrome

Definition
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Clinical variant of acute or subacute cutaneous lupus erythematosus characterized by the appearance of anular, or multiform, succulent, possibly bullous foci on the trunk, face, neck and nape of the neck (see below lupus erythematosus integumentalis or systemic lupus erythematosus). The clinical picture may resemble an erythema exsudativum multiforme.

Clinical features
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Erythema exsudativum multiforme-like skin lesions in combination with skin lesions of lupus erythematosus (systemic, discoid or subacute-cutaneous). Chilblain-like skin changes on the hands and feet.

Diagnostics
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Major criteria: skin lesions of lupus erythematosus and erythema exsudativum, positive ANA with speckled pattern.

Minor criteria: positive SS-A and SS-B antibodies, reactive rheumatoid factor, chilblain-like skin lesions.

For diagnosis, all major criteria and at least one minor criterion must be present.

Laboratory
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ANA- fine speckled pattern, positive SS A and SS B autoantibodies, rheumatoid factor

Therapy
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Initial systemic and topical glucocorticoids. Avoidance of possible triggers - light protection.

If not successful, combination of steroid therapy with systemic immunosuppressants. Hydroxychloroquine, dapsone, methotrexate, azathioprine or ciclosporin have been described.

Literature
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  1. Rowell NR et al (1963) Lupus erythematosus et erythema multiforme-like lesions. Arch Dermatol 88: 176-180
  2. Lent M et al (2011) Rowell's syndrome: a distinct clinical entity or a coincidence of diagnoses? Int J Dermatol 51:718-721
  3. Roy M et al.(2010) Rowell's syndrome: presenting features of systemic lupus erythematosus. Rheumatol Int 33:1075-1077
  4. Nonhoff J et al (2021) Rowell's syndrome in subacute cutaneous lupus erythematosus. Act Dermatol 47: 106-110

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 09.05.2021