|
Final diagnosis: Lichen Planus Pemphigoides
Discussion: Rare disorder with combination of lichen planus lesions and tense bullous eruptions on skin, both in involved and uninvolved areas of the lichenoid eruption. 1, 2 LP pemphigoides affects primarily adult men in middle age (40-50 years of age), but several cases have been reported in children. Postulated to be a blistering autoimmune response to hemidesmosomal-derived antigens exposed after lichenoid inflammation. Commonly associated with drug reaction e.g. ACE inhibitors, statins, cinnarizine, Chinese herbs and weight reduction products.1, 2, 3 It can be associated with Castleman’s disease and internal malignancies. Diagnostic features include DIF with fibrinogen deposition at the dermal-epidermal junction with cytoid bodies and linear IgG and C3 along the basement membrane zone with positive BP180 and/or BP230. Treatment is usually associated with high rate of remission after initial disease control and patients can generally be weaned off all treatments. 3, 4
List of References
1. Lichen Planus Pemphigoides. In: Patterson, eds. Tissue reaction patterns: Weedon’s Skin Pathology Text. 4th ed. London, UK: Churchill Livingstone Elsevier; 2016:165–166
2. T.Shiohara, Y.Kano. Lichen Planus Pemphigoides. In: Bolognia, Jorizzo, Rapini eds. Papulosquamous and eczematous dermatoses; Lichen planus and lichenoid dermatitis. Dermatology Text. 2nd ed. Maryland heights, MO: Mosby Elsevier; 2008:168–169
3. Zaraa I, Mahfoudh A, Sellami MK, Lichen planus pemphigoides: four new cases and a review of the literature. Int J Dermatol. 2013 Apr; 52(4):406-12.
4. Ken Washio, Atsuko Nakamura, A Case of Lichen Planus Pemphigoides Successfully Treated with a Combination of Cyclosporine A and Prednisolone. Case Rep Dermatol. 2013 Jan-Apr; 5(1): 84–87.