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Reports of Two Cases of Atypical Linear IgA Bullous Dermatosis

Author: FengChao
Tutor: YangGuoLing
School: Dalian Medical University
Course: Dermatology and Venereology
Keywords: LABD rash direct immunofluorescence microscopy dapsone
CLC: R758.66
Type: Master's thesis
Year: 2013
Downloads: 8
Quote: 0
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Abstract


Background: Linear IgA bullous dermatosis (LABD) is a rare acquiredsubepidermal blistering disease. The clinical features of LABD may resemble those ofbullous pemphigoid and dermatitis herpetiformis. The disease commonly presents as anerythematous maculopapular rash or urticated plaques with sporadic vesicles. LABDhas the tendency of spontaneous Remission and can be cured by drug. The disease canbe induced by drug, viral infection, disorder of immune system, tumor and trauma. Itwas characterized by continuous linear IgA deposits along basement membrane zonewhen visualized with direct immunofluorescent microscopy.Methods：This text report two cases of atypical LABD accompanied with rashwhich was not annular dispersed and the mucosa showed no abnormal in our clinic.Case presentation:1. A48-year-old male was suffered erythematous plaques in his neck for3months,which rapidly progressed to spread to the whole body withthin2months. One weeklater, the patient admitted to our clinic when the disease aggravated. He had beendiagnosed as “Atopic dermatitis” and treatment with antihistaminics and topical steroidin the local hospital, but the rash increased and accompanied by severe itching andscabs one week ago.2. A38-year-old male was suffered erythema, blisters with itchingin the whole body. The patient was admitted to the department of dermatologyh whenthe symptom rapidly aggravated3days ago. The disease can heal after scratching andscabbing, but the condition recurring. Both patients were generally in good conditionwithout fever and mucosal damage. Vital signs and physical examination were normal.The patients were health before with no family disease history but allergic to penicillin.Specialist examination: The skin disease was accompanied by erythema, papules,papulovesicles, blisters, pigmentation spots in the trunk. The Nikolsky’s sign wasnegative. The most blisters were scratched with surface deposition and blood crust was visible.Laboratory examination: Skin biopsy and direct immunofluorescence showedsubepidermal blisters and linear IgA bullous dermatosis within the epithelialbasement membrane respectively for sample1. The Skin biopsy showedhyperkeratosis, skin mild edema and inflammatory cells were visible around the dermalblood vessels for sample2. The IgG and C3were both negative.Conclusion:1. The lesions of two cases showed dispersed but not ring-shaped compared withtypical skin lesions. But IgA antibody deposited was observed in basement membranezone when using direct immunofluorescent microscopy. The characters accord with thatof LABD.2. The gold standard for diagnosis of LABD is direct immunofluorescence.

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CLC: > Medicine, health > Dermatology and Venereology > Dermatology > The reason is unknown skin disease > Bullous skin diseases
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