Rowell's syndrome L93.1

Clinical variant of acute or subacute cutaneous lupus erythematosus characterized by the appearance of anular or multiforme, succulent, possibly bullous foci on the trunk, face, neck (see below integumentary lupus erythematosus 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".

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.

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.

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

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.

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