Rowell's syndrome L93.1

Rowell et al. 1963; Scholz M 1922

Clinical variant of acute or subacute cutaneous lupus erythematosus characterized by the appearance of anular, or multiforme, succulent, possibly bullous lesions on the trunk, face, neck (see below integumentary lupus er ythematosus or systemic lupus erythematosus). The clinical picture may resemble erythema exsudativum multiforme.

See also the broad overlap with the "acute syndrome of apoptotic pan-epidermolysis". The independence of Rowell's syndrome is disputed by numerous authors (Antiga E et al. 2012), especially since multiforme aspects are not uncommon in subacute cutaneous lupus er ythematosus.

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.

The diagnosis of Rowell syndrome can be made according to the criteria established by Zeitouni NC et al as follows:

Major criteria:

• Skin lesions of subacute cutaneous lupus erythematosus

and

• of erythema multiforme
• Laboratory: positive ANA with speckled pattern.

Minor criteria:

• Chilblain-like skin lesions

• Laboratory: positive SS-A and SS-B antibodies or reactive rheumatoid factor

• Diagnosis must have all major criteria and at least one minor criterion.

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

Initial systemic and topical glucocorticoids to adequately address the multiforme acute situation.

Avoidance of possible triggers - light protection.

If not successful, combination of steroid therapy with systemic immunosuppressants.

Described as long-term therapy: hydroxychloroquine, dapsone, methotrexate, azathioprine or ciclosporin.

1. Antiga E et al (2012) The last word on the so-called 'Rowell's syndrome'? Lupus 21:577-585.
2. Challa M et al (2022) Rowell syndrome with good response to methotrexate. Mediterr J Rheumatol. 33:92-93.
3. Lent M et al (2011) Rowell's syndrome: a distinct clinical entity or a coincidence of diagnoses? Int J Dermatol 51:718-721.
4. Nonhoff J et al (2021) Rowell's syndrome in subacute cutaneous lupus erythematosus. Act Dermatol 47: 106-110
5. Pozharashka J et al (2019) Rowell's syndrome triggered by omeprazole. Acta Dermatovenerol Croat. 27:124-126.
6. Rowell NR et al (1963) Lupus erythematosus et erythema multiforme-like lesions. Arch Dermatol 88: 176-180
7. Roy M et al.(2010) Rowell's syndrome: presenting features of systemic lupus erythematosus. Rheumatol Int 33:1075-1077
8. Scholz M (1922) Lupus erythematosus acutus disseminatus hemorrhagicus. Arch dermatol Syph 6:464-475.
9. Zeitouni NC et al (2000) Redefining Rowell's syndrome. Br J Dermatol142:343-346.