|Year : 2000 | Volume
| Issue : 5 | Page : 262-263
M L Khatri
Source of Support: None, Conflict of Interest: None
A 22-year old female patient presented with recurrent episodes of erythema multiforme-like eruption in association with systemic lupus erythematosus. Laboratory findings included leucopenia, anaemia, positive LE cell phenomenon, positive rheumatoid factor and mild proteinuria. The features included most of the criteria of Rowell's syndrome.
Keywords: Rowell′s syndrome, Erythema multiforme, Systemic lupus erythematosus
|How to cite this article:|
Khatri M L. Rowell's syndrome. Indian J Dermatol Venereol Leprol 2000;66:262-3
| Introduction|| |
Rowell et al  first described a subset of patients with lupus erythematosus, clinically resembling erythema multiforme, having positive rheumatoid factor and a speckled pattern of aninuclear antibody to saline extract of human tissue (anti-SjT). We describe a case of systemic LE presenting as recurrent episodes of erythema multiforme with diagnostic criteria as described by Rowell et al.
| Case Report|| |
A 22-year-old unmarried Yemeni female was admitted with complaints of fever, skin rash and arthralgia for 3 weeks. She had similar three previous episodes in last one year. There was no history of herpes simplex infection, any respiratory infection or any drug intake prior to all episodes. At the time of assessment she had high fever and skin rash in the form of diffuse erythematous plaques on nose and cheeks and a few discrete target shaped thin plaques on face. She also had multiple erythematous target shaped plaques on dorsum of hands, forearms and palms and erythematous vesiculo- bullous lesions like chilblain on the toes of both feet. There were crusted erosions on lips and erosions all over oral and pharyngeal mucosa.[Figure - 1]
Laboratory test revealed erythrocyte sedimentation rate of 28 mm/1 st hour, leucopenia (total count 2200/mm 3 with differential count polymorphs 53%, lymphocytes 38%, monocytes 4%, eosinophils 2%, basophils 3%) anaemia (haemoglobin 8.8 gm%), positive rheumatoid factor, positive LE cell phenomenon and mild proteinuria (550 mg/24 hours of urine). Blood urea, electrolytes and serum creatinine were within normal range. VDRL was non reactive. Investigation of antinuclear factor was not available.
X-ray chest, ultrasound abdomen and ECG did not reveal any abnormality.[Figure - 2]
Histopathology of a target lesion from dorsum of the hand revealed epidermal necrosis, vacuolar degeneration of basal cells and papillary dermal oedema with perivascular lymphocytic infiltration in mid dermis.
The patient was treated with systemic steroids, initially with high dose of methyl prednisolone followed by oral prednisolone and supportive treatment. Oral and skin lesions healed well with residual mild activity in skin lesions within 3 weeks. The patient was discharged with prednisolone 30 mg/day. Later the patient was lost to follow-up.
| Discussion|| |
Common causes of recurrent erythema multiforme are herpes simplex infection and drugs. We could not elicit any evidence of these causes or other common causes of erythema multiforme in our patient.
An association of erythema multiforme and lupus erythematosus was reported first by Scholz.  Later Rowell et all described 4 cases (among their 120 cases of DLE) with distinctive clinical and immunologic findings that included erythema multiformelike lesions, chilblain-like lesions, speckled pattern of antinuclear antibodies, positive serum rheumatoid factor and antibodies to saline extract of human tissues (anti SjT). Although our patient had most of the criteria as described by Rowell et al but unlike their cases our patient had systemic lupus erythematosus. Similar association has been described before. , Our patient also had chilblainlike lesions as reported earlier.  We conclude that Rowell's syndrome is a distinct clinical and immunological entity in which association of erythema multiforme can occur either with DLE or SLE.
| References|| |
|1.||Rowell NR, Becks SJ, Anderson JR. Lupus erythematosus and erythema multiforme-like lesions. Arch Dermatol 1963;88:176-80. |
|2.||Scholtz M. Lupus erythematosus acutus disseminatus haemorrhagicus. Arch Dermatol Syph 1922;6:466. |
|3.||Fitzgerald EA, Purcell SM, Kantor GR, et al. Rowell's syndrome: report of a case. J Am Acad Dermatol 1996;35:801-3. [PUBMED] |
|4.||Lawrence CM, Marshall TL, Byrne JPH. Lupus erythematosus associated with erythema multiforme-like lesions in identical twins. Br J Dermatol 1982;107:349-56. |
[Figure - 1], [Figure - 2]