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Bullous pemphigoid positive for anti-BP180 and anti-laminin 5 antibodies in a patient with graft-vs-host disease.
J Am Acad Dermatol. 2007 May; 56(5 Suppl):S94-7.JA

Abstract

We report the case of a 55-year-old female with bullous pemphigoid (BP) who was positive for anti-BP180 and anti-laminin 5 antibodies after development of graft-vs-host disease (GVHD) caused by a bone marrow transplant. She had tense blisters on her trunk and extremities. Histologic examination showed a subepidermal blister and marked lymphocytic infiltration, especially eosinophils. Direct immunofluorescence revealed a linear deposition of IgG on the base membrane zone. Indirect immunofluorescence on 1M NaCl split skin revealed a linear IgG deposition to both sides of the epidermal and the dermal layers. Immunoblot assays using human epidermal extracts and BP180 NC16a domain recombinant protein confirmed the presence of IgG antibodies against BP180 and recombinant BP180 NC16a domain protein. Furthermore, immunoblotting using laminin 5 purified from human keratinocyte extract as the substrate demonstrated reactivity against the gamma2 and beta3 subunits but not the alpha3 subunit of laminin 5. We diagnosed BP and treated her with prednisolone (40 mg/day). Both skin and oral lesions resolved without leaving scars on the bulla. Immune disturbance as well as destruction of basal epidermal cells and base membrane by GVHD may result in the induction of autoimmune blistering diseases with unusual clinical and laboratory manifestations.

Authors+Show Affiliations

Department of Dermatology, Kanazawa University Graduate School of Medical Science, Ishikawa, Japan.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

17434049

Citation

Izumi, Rieko, et al. "Bullous Pemphigoid Positive for anti-BP180 and Anti-laminin 5 Antibodies in a Patient With Graft-vs-host Disease." Journal of the American Academy of Dermatology, vol. 56, no. 5 Suppl, 2007, pp. S94-7.
Izumi R, Fujimoto M, Yazawa N, et al. Bullous pemphigoid positive for anti-BP180 and anti-laminin 5 antibodies in a patient with graft-vs-host disease. J Am Acad Dermatol. 2007;56(5 Suppl):S94-7.
Izumi, R., Fujimoto, M., Yazawa, N., Nakashima, H., Asashima, N., Watanabe, R., Kuwano, Y., Kurokawa, M., Hashimoto, T., & Tamaki, K. (2007). Bullous pemphigoid positive for anti-BP180 and anti-laminin 5 antibodies in a patient with graft-vs-host disease. Journal of the American Academy of Dermatology, 56(5 Suppl), S94-7.
Izumi R, et al. Bullous Pemphigoid Positive for anti-BP180 and Anti-laminin 5 Antibodies in a Patient With Graft-vs-host Disease. J Am Acad Dermatol. 2007;56(5 Suppl):S94-7. PubMed PMID: 17434049.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Bullous pemphigoid positive for anti-BP180 and anti-laminin 5 antibodies in a patient with graft-vs-host disease. AU - Izumi,Rieko, AU - Fujimoto,Manabu, AU - Yazawa,Norihito, AU - Nakashima,Hiroko, AU - Asashima,Nobuko, AU - Watanabe,Rei, AU - Kuwano,Yoshihoro, AU - Kurokawa,Mineo, AU - Hashimoto,Takashi, AU - Tamaki,Kunihiko, PY - 2006/06/20/received PY - 2006/10/09/revised PY - 2006/10/19/accepted PY - 2007/4/17/pubmed PY - 2007/6/5/medline PY - 2007/4/17/entrez SP - S94 EP - 7 JF - Journal of the American Academy of Dermatology JO - J Am Acad Dermatol VL - 56 IS - 5 Suppl N2 - We report the case of a 55-year-old female with bullous pemphigoid (BP) who was positive for anti-BP180 and anti-laminin 5 antibodies after development of graft-vs-host disease (GVHD) caused by a bone marrow transplant. She had tense blisters on her trunk and extremities. Histologic examination showed a subepidermal blister and marked lymphocytic infiltration, especially eosinophils. Direct immunofluorescence revealed a linear deposition of IgG on the base membrane zone. Indirect immunofluorescence on 1M NaCl split skin revealed a linear IgG deposition to both sides of the epidermal and the dermal layers. Immunoblot assays using human epidermal extracts and BP180 NC16a domain recombinant protein confirmed the presence of IgG antibodies against BP180 and recombinant BP180 NC16a domain protein. Furthermore, immunoblotting using laminin 5 purified from human keratinocyte extract as the substrate demonstrated reactivity against the gamma2 and beta3 subunits but not the alpha3 subunit of laminin 5. We diagnosed BP and treated her with prednisolone (40 mg/day). Both skin and oral lesions resolved without leaving scars on the bulla. Immune disturbance as well as destruction of basal epidermal cells and base membrane by GVHD may result in the induction of autoimmune blistering diseases with unusual clinical and laboratory manifestations. SN - 1097-6787 UR - https://www.unboundmedicine.com/medline/citation/17434049/Bullous_pemphigoid_positive_for_anti_BP180_and_anti_laminin_5_antibodies_in_a_patient_with_graft_vs_host_disease_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0190-9622(06)04086-2 DB - PRIME DP - Unbound Medicine ER -