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[Anti-p200 pemphigoid: Remission under mycophenolate mofetil (Cellcept®)].
Ann Dermatol Venereol. 2013 Dec; 140(12):784-7.AD

Abstract

BACKGROUND

Anti-p200 pemphigoid is a recently described autoimmune subepidermal bullous dermatosis characterized by its target antigen and the associated anatomoclinical picture. The treatment is not as yet well defined.

PATIENT AND METHODS

A 73-year-old man consulted for a pruritic bullous eruption with buccal involvement. Direct immunofluorescence revealed linear deposits of IgG and C3 at the dermal-epidermal junction. Elisa screening for circulating anti-BP180 and anti-BP230 antibodies was negative. A diagnosis of bullous pemphigoid was suspected. After an unfavourable clinical outcome under clobetasol and then prednisolone and methotrexate, other immunological tests were performed. Indirect immunofluorescence on NaCl-cleaved skin revealed a deposit of IgG4 antibodies on the dermal side. Immunoblotting showed antibodies directed against a 200-kDa antigen on a dermal extract. A diagnosis of anti-p200 pemphigoid was made. The patient was treated with dapsone combined with prednisolone. Seventy-two hours later, treatment was stopped due to hepatic cytolysis related to immunoallergic hepatitis. Treatment with mycophenolate mofetil was then initiated and resulted in complete remission, which persisted at seven months.

DISCUSSION

The diagnosis of anti-p200 pemphigoid was made on the basis of a set of clinical and immunological factors. Anti-p200 pemphigoid differs from standard bullous pemphigoid in terms of more frequent cephalic, acral and mucous membrane involvement, as well as a greater degree of miliary scarring. There was no eosinophilia. Elisa screening for anti-BP180 and anti-BP230 antibodies was negative. Immunoblotting showed antibodies directed against a 200kDa protein on dermal extract. The treatment is not well defined, even if dapsone appears to be the most effective therapy. To our knowledge, our patient is the first to be successfully treated with mycophenolate mofetil.

CONCLUSION

Treatment of anti-p200 pemphigoid is difficult. In our case, treatment by mycophenolate mofetil was effective and could offer an alternative to dapsone.

Authors+Show Affiliations

Service de dermatologie, hôpital Porte-Madeleine, CHR d'Orléans, 1, rue Porte-Madeleine, 45032 Orléans cedex 1, France.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

fre

PubMed ID

24315224

Citation

Raffin, D, et al. "[Anti-p200 Pemphigoid: Remission Under Mycophenolate Mofetil (Cellcept®)]." Annales De Dermatologie Et De Venereologie, vol. 140, no. 12, 2013, pp. 784-7.
Raffin D, Delaplace M, Roussel A, et al. [Anti-p200 pemphigoid: Remission under mycophenolate mofetil (Cellcept®)]. Ann Dermatol Venereol. 2013;140(12):784-7.
Raffin, D., Delaplace, M., Roussel, A., & Estève, E. (2013). [Anti-p200 pemphigoid: Remission under mycophenolate mofetil (Cellcept®)]. Annales De Dermatologie Et De Venereologie, 140(12), 784-7. https://doi.org/10.1016/j.annder.2013.07.005
Raffin D, et al. [Anti-p200 Pemphigoid: Remission Under Mycophenolate Mofetil (Cellcept®)]. Ann Dermatol Venereol. 2013;140(12):784-7. PubMed PMID: 24315224.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Anti-p200 pemphigoid: Remission under mycophenolate mofetil (Cellcept®)]. AU - Raffin,D, AU - Delaplace,M, AU - Roussel,A, AU - Estève,E, Y1 - 2013/08/13/ PY - 2013/02/11/received PY - 2013/06/02/revised PY - 2013/07/01/accepted PY - 2013/12/10/entrez PY - 2013/12/10/pubmed PY - 2014/8/15/medline KW - Anti-p200 pemphigoid KW - Bullous pemphigoid KW - Mycofenolate mofetil KW - Mycofénolate mofétil KW - Pemphigoïde anti-p200 KW - Pemphigoïde bulleuse KW - Traitement KW - Treatment SP - 784 EP - 7 JF - Annales de dermatologie et de venereologie JO - Ann Dermatol Venereol VL - 140 IS - 12 N2 - BACKGROUND: Anti-p200 pemphigoid is a recently described autoimmune subepidermal bullous dermatosis characterized by its target antigen and the associated anatomoclinical picture. The treatment is not as yet well defined. PATIENT AND METHODS: A 73-year-old man consulted for a pruritic bullous eruption with buccal involvement. Direct immunofluorescence revealed linear deposits of IgG and C3 at the dermal-epidermal junction. Elisa screening for circulating anti-BP180 and anti-BP230 antibodies was negative. A diagnosis of bullous pemphigoid was suspected. After an unfavourable clinical outcome under clobetasol and then prednisolone and methotrexate, other immunological tests were performed. Indirect immunofluorescence on NaCl-cleaved skin revealed a deposit of IgG4 antibodies on the dermal side. Immunoblotting showed antibodies directed against a 200-kDa antigen on a dermal extract. A diagnosis of anti-p200 pemphigoid was made. The patient was treated with dapsone combined with prednisolone. Seventy-two hours later, treatment was stopped due to hepatic cytolysis related to immunoallergic hepatitis. Treatment with mycophenolate mofetil was then initiated and resulted in complete remission, which persisted at seven months. DISCUSSION: The diagnosis of anti-p200 pemphigoid was made on the basis of a set of clinical and immunological factors. Anti-p200 pemphigoid differs from standard bullous pemphigoid in terms of more frequent cephalic, acral and mucous membrane involvement, as well as a greater degree of miliary scarring. There was no eosinophilia. Elisa screening for anti-BP180 and anti-BP230 antibodies was negative. Immunoblotting showed antibodies directed against a 200kDa protein on dermal extract. The treatment is not well defined, even if dapsone appears to be the most effective therapy. To our knowledge, our patient is the first to be successfully treated with mycophenolate mofetil. CONCLUSION: Treatment of anti-p200 pemphigoid is difficult. In our case, treatment by mycophenolate mofetil was effective and could offer an alternative to dapsone. SN - 0151-9638 UR - https://www.unboundmedicine.com/medline/citation/24315224/[Anti_p200_pemphigoid:_Remission_under_mycophenolate_mofetil__Cellcept®_]_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0151-9638(13)00846-6 DB - PRIME DP - Unbound Medicine ER -