Unusual Presentation of Idiopathic Sweet's Syndrome in a Photodistributed Pattern
A 40-year-old lady with unknown co-morbidities presented with a 10 days history of multiple red raised painful lesions over body. Lesions were initially noted over forearms and in the next 2-3 days, new lesions appeared over arms and back. Patient complained of increased pain and burning sensation in the lesions while working in sunlight. Associated mild to moderate grade fever, intermittent in nature was present for the same duration. There was a history of malaise, pain over large joints, decreased appetite and redness of eyes. No history of oral ulcers, weight loss or any drug intake prior to her symptoms was present.\nGeneral examination revealed that she was febrile with an axillary temperature of 102°F. She also had pallor. Her systemic examination was essentially within normal limits. Dermatological examination revealed erythematous papules coalescing to form plaques with a pseudovesicular appearance over the extensor aspect of forearms and photo-exposed areas on back [Figures and ]. There was a sharp cut-off between the lesions and the photo-protected areas, where there were absolutely no lesions []. The plaques were tender. Conjunctival congestion was also present.\nInvestigations revealed anemia with haemoglobin of 9.5 gms/dl, and neutrophilic leukocytosis with 86% neutrophils out of a total of 13,000/mm3. She had an ESR of 36 mm fall in the first hour by the Westergren's technique. C reactive protein was positive. Antinuclear antibodies ANA and ELISA for HIV were negative. Extensive radiological, biochemical and hematological investigations did not reveal any underlying malignancy or any systemic association. Skin biopsy revealed edema of the papillary dermis along with a heavy neutrophilic infiltrate in the upper dermis. Furthermore, coalescing infiltrate of neutrophils in the lower dermis especially around blood vessels with absence of vasculitis was seen []. There was evidence of endothelial swelling with few fragmented neutrophilic nuclei but no fibrinoid necrosis []. She responded dramatically to a course of oral steroids in the form of Prednisolone 40 mg/day and topical broad spectrum photo-protection. Within seven days, her fever had subsided and within the next two weeks most of her skin lesions regressed. The oral steroids were tapered off in another 4 weeks. There was no recurrence of lesions after three months of follow-up.