Bullous Systemic Lupus Erythematosus
A 39-year-old lady, a homemaker and mother of three, presented with largely asymptomatic vesicobullous lesions (2 months duration), involving head and neck, upper limbs, and trunk over both photo-exposed and unexposed areas. The blisters would rupture over 2-3 days; yielding clear (sometimes hemorrhagic) fluid; heal over 2-3 weeks; leaving behind pigmentation and occasional scarring. Concurrent painful oral ulcers were reported; though, other mucosae were uninvolved. A 6-month history suggestive of Raynaud's phenomenon; arthralgias involving small joints of hands and feet; photosensitivity; and intermittent episodes of mild to moderate fever was present. The patient reported three previous, spontaneous abortions, (5months gestation) the last one being 6 years back.\nOn examination, there were multiple, tense, clear as well as hemorrhagic, vesicles and bullae, arising over an erythematous base and predominantly involving upper body []. Bulla spread sign and Nikolsky sign were positive. In addition, superficial erosions, areas of postinflammatory hyperpigmentation, and small superficial scars were noted. Superficial erosions were also noted over the buccal mucosa, hard palate, and lips. Her systemic examination was essentially normal.\nA provisional diagnosis of BSLE was considered and she was thoroughly investigated. She had a significant anemia (8.8 gm/dL) with evidence of iron deficiency on peripheral smear and iron studies. Other biochemical investigations were essentially normal, with no evidence of renal derangement. Extensive serological workup revealed antinuclear antibody positivity of 1:640 (using hep-2 cells); positive anti-dsDNA, anti-Ro, and anti-La antibodies; low levels of C3 and C4 with a normal CH50 component; negative antiphospholipid antibody, rheumatoid factor, and C-reactive protein. Russell's viper venom test revealed no lupus like anticoagulant. X-rays of chest and involved joints were normal.\nA lesional biopsy revealed subepidermal split with neutrophilic infiltrate in the papillary dermis []. Direct immunofluorescence (both perilesional and distant-covered skin) revealed IgM and C3 deposition along the basement membrane zone (BMZ) as well as perivascular location. Due to resource constraints, indirect immunofluorescence or salt-split skin could not be performed. Based on clinical, histopathological, and serological evidence, a diagnosis of BSLE was confirmed.\nWith a normal glucose 6-Phosphate dehydrogenase (G6PD) activity, we planned to start her on dapsone; however, her low hemoglobin prevented this. She was started on prednisolone (1 mg/kg/day as a single morning dose); hydroxychloroquine (200 mg twice a day); along with complete sunprotection and topical care. Hematinics were also initiated. There was only partial improvement with new lesions developing even after 4 weeks; wherein, she was started on dapsone. She responded quite favorably with no new lesions and satisfactory healing of pre-existing ones []. Over the next 6 months, steroids were completely withdrawn, and dapsone and hydroxychloroquine continued for further 1 year. The patient has remained in remission without any systemic manifestations as well, over the last 1 year.