|Year : 2000 | Volume
| Issue : 6 | Page : 324-325
Alka Dogra, YC Minocha, Monish Gupta, Pun Capalash
Source of Support: None, Conflict of Interest: None
A 65-year old woman presented with long standing history of lupus erythematosus with episodic eruption of erythema mutiforme - like lesions. Immunological investigations revealed positive rheumatoid factor, ANF and histology was consistent with the clinical findings.
Keywords: Rowell′s syndrome, Lupus erythematosus, Erythema multiforme
|How to cite this article:|
Dogra A, Minocha Y C, Gupta M, Capalash P. Rowell's Syndrome. Indian J Dermatol Venereol Leprol 2000;66:324-5
| Introduction|| |
Rowell's syndrome is a distinctive syndrome in which a subset of patients with lupus erythematosus develop erythema multiforme- like skin lesions with positive serum rheumatoid factor , a speckled pattern of ANF and precipitating antibody to saline extract of human tissue anti La (SS-B).  We are describing a patient of systemic lupus erythematosus who developed episodes of erythema multiforme- like lesions and fulfills the criteria described by Rowell.
| Case Report|| |
A 65- year- old woman presented with skin lesions of five years duration consisting of irregular erythematous scaly plaques over the malar areas of face, ears, neck, trunk and limbs. She had frequent episodes of erythematous annular lesions occurring on the extensors of the arms and dorsal aspect of hand which on examination were all circumscribed, erythematous, annular lesions with central necrotic zone. She also developed progressive photosensitivity, and blanching of fingers on exposure to cold. There was history of intermittent oral ulcers, mild-moderate fever and progressive diffuse loss of hair. The patient denied any history of previous drug intake. Routine investigations revealed leukopenia and anaemia. Rheumatoid factor and ANF were positive. LE cell phenomenon was negative. Histological examination revealed hyperkeratosis without parakeratosis; liquefacitve degeneration of the basal cell layer and prominent dermal edema consistent with SLE. Another biopsy taken from the annular lesions showed mild spongiosis and exocytosis, necrosis of individual keratinocytes in stratum malpighii with vacuolization of basal layer and a lymphocytic infiltrate at the dermo- epidermal junction consistent with erythema multiforme.
| Discussion|| |
Erythema multiforme is most often found in association with drug ingestion, Mycoplasma pneumoniae nd Herpes simplex infections, although other causes including infectious agents, malignant conditions, contactants and connective tissue disease have been implicated.
The early EM- like lesions in Rowell's syndrome begin as erythematous papules and progress to ring shaped lesions with a vesicular edge; bullae and necrosis occur in severe cases. Histopathologically these lesions show changes characteristic of erythema multiforme. Patients with this syndrome also frequently have perniotic lesions.
The syndrome has been reported in identical twin sisters, one of whom had DLE and the other, SLE. When this syndrome occurs in DLE, dermoepidermal band test is positive in the discoid lesions and negative in the EM lesions. If LE is systemic the EM lesions show positive findings comparable with those seen in the uninvolved skin of patients with SLE.
| References|| |
|1.||Fitzgerald EA, Percell SM, Gary R, et al. Rowell's syndrome: report of a case. J Am Acad Dermatol 1996; 35: 801-803. |
|2.||Huff JC, Weston WL, Tonnesen MG. Erythema multiforme: a critical review of characteristics, diagnostic criteria and causes. J Am Acad Dermatol1998; 8: 763- 765. |
|3.||Millard LG, Rowell NR. Chilblain lupus erythematosus Hutchinson. Br J Dermatol 1978; 98: 497- 506. |
|4.||Lawrence CM, Marshall TL, Byrne JPH. Lupus erythematosus associated with erythema multiforme- like lesions in identical twins. Br J Dermatol 1982; 107: 349- 356. |