DIAGNOSIS: Rowell's Syndrome

Discussion

In 1963 Rowell et al. described a syndrome characterized by lupus erythematosus with erythema multiforme-like lesions,speckled antinuclear antibodies, anti-SjT antibodies and rheumatoid factor.(1) Anti- SjT is now thought to be identical with anti-La (SS-B). Rowell originally reported this syndrome in DLE(1) but it also occurs in SLE.(2) Perniotic lesions frequently occur.(3)

Figure 7
Figure 7: Annular erythematous purpuric plaques on the arm.
This case fulfills all the criteria described initially by Rowell et al. and by subsequent reports.(4-7) Although a positive rheumatoid factor has also been shown in many (but not all) cases reported thus far, this test was not obtained for our patient. This report does differ from previous descriptions of Rowell's syndrome in that the first biopsy was interpreted as erythema multiforme.(8) However, the second biopsy was consistent with lupus erythematosus.

Some authors feel that Rowell syndrome may simply be coincidental occurrence of lupus erythematosus and erythema multiforme.(9) This patient was initially treated by her general practitioner as an erythema multiforme. He had even done Herpes titers. Both IgG and IgM were positive for type 1 and 2. These were not repeated. He therefore had put her on Famciclovir in January. I saw the patient for the first time 4 weeks later. The duration of the illness by this stage was ~10 weeks. This is much too long for erythema multiforme. Drug reaction to captopril or spironolactone was initially considered, however her disease did not improve on discontinuation of these medications.

Some of the cases of Rowell's syndrome have relapsed periodically over a period of 20 years. My patient has not had any relapse for over 1 year.

References

1. Rowell NR, Swanson-Beck J, Anderson JR. Lupus erythematosus and erythema multiforme-like lesions. Arch Dermatol 1963;88:176-80

2. Lawrence CM, Marshall TL, Byrne JPH. Lupus erythematosus associated with erythema multiforme-like lesions in identical twins. Br J Dermatol 1982; 107: 349-56.

3. Millard LG, Rowell NR. Chilblain lupus erythematosus, Hutchinson. Br J Dermatol 1978; 98: 497-506.

4. Fitzgerald EA; Purcell SM; Kantor GR; Goldman HM. Rowell's syndrome: report of a case. Journal of the American Academy of Dermatology, 1996 Nov, 35(5 Pt 2):801-3.

5. Chua SH; Giam YC; Sim CS. Systemic lupus erythematosus with erythema multiforme-like lesions and histiocytic necrotizing lymphadenitis--a case report. Annals of the Academy of Medicine, Singapore, 1996 Jul, 25(4):599-601.

6. Fiallo P; Tagliapietra AG; Santoro G; Venturino E. Rowell's syndrome. International Journal of Dermatology, 1995 Sep, 34(9):635-6.

7. Crickx B; Crickx L; Vissuzaine C; Grossin M; Belaich S. [Chronic lupus erythematosus with erythema multiforme-like lesions (Rowell's syndrome)]. Annales de Dermatologie et de Venereologie, 1985, 112(9):705-6.

8. Heyl T; Meyer T. Lupus erythematosus with erythema multiforme (Rowell's syndrome). A case report. South African Medical Journal, 1984 Apr 7, 65(14):568-9.

9. Parodi A; Drago EF; Varaldo G; Rebora A. Rowell's syndrome. Report of a case. Journal of the American Academy of Dermatology, 1989 Aug, 21(2 Pt 2):374-7.