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Critical Situations |

Aggressive Immunosuppressive Therapy for a Refractory Case of IgA Pemphigus

Kendall Sibley Hash, MD; Adrienne Rencic, MD, PhD; Maria Ines Hernandez, MD; Takashi Hashimoto, MD; H. Carlos Nousari, MD
Arch Dermatol. 2002;138(6):744-746. doi:10.1001/archderm.138.6.744.
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Hashimoto  TKiyokawa  CMori  O  et al.  Human desmocollin 1 (Dsc1) is an autoantigen for the subcorneal pustular dermatosis type of IgA pemphigus. J Invest Dermatol. 1997;109127- 131
Link to Article[[XSLOpenURL/10.1111/jid.1997.109.issue-2]]
Hashimoto  TKomai  AFutei  YNishikawa  TAmagai  M Detection of IgA autoantibodies to desmogleins by an enzyme-linked immunosorbent assay: the presence of new minor subtypes of IgA pemphigus. Arch Dermatol. 2001;137735- 738
Robinson  NDHashimoto  TAmagai  MChan  LS The new pemphigus variants. J Am Acad Dermatol. 1999;40649- 673
Link to Article[[XSLOpenURL/10.1016/S0190-9622(99)70145-3]]
Harman  KEHolmes  GBhogal  BSMcFadden  JBlack  MM Intercellular IgA dermatosis (IgA pemphigus): two cases illustrating the clinical heterogeneity of this disorder. Clin Exp Dermatol. 1999;24464- 466
Link to Article[[XSLOpenURL/10.1046/j.1365-2230.1999.00534.x]]
Karpati  SAmagai  MLiu  WLDmochowski  MHashimoto  THorvath  A Identification of desmoglein 1 as autoantigen in a patient with intraepidermal neutrophilic IgA dermatosis type of IgA pemphigus. Exp Dermatol. 2000;9224- 228
Link to Article[[XSLOpenURL/10.1034/j.1600-0625.2000.009003224.x]]
Niimi  YKawana  SKusunoki  T IgA pemphigus: a case report and its characteristic clinical features compared with subcorneal pustular dermatosis. J Am Acad Dermatol. 2000;43546- 549
Link to Article[[XSLOpenURL/10.1067/mjd.2000.107478]]
Ongenae  KCTemmerman  LJVermander  FNaeyaert  JM Intercellular IgA dermatosis. Eur J Dermatol. 1999;985- 94
Hashimoto  TEbihara  TNishikawa  T Studies of autoantigens recognized by IgA anti-keratinocyte cell surface antibodies. J Dermatol Sci. 1996;1210- 17
Link to Article[[XSLOpenURL/10.1016/0923-1811(95)00449-1]]
Karpati  SAmagai  MLiu  WLDmochowski  MHashimoto  THorvath  A Identification of desmoglein 1 as autoantigen in a patient with intraepidermal neutrophilic IgA dermatosis type of IgA pemphigus. Exp Dermatol. 2000;9224- 228
Link to Article[[XSLOpenURL/10.1034/j.1600-0625.2000.009003224.x]]
Yasuda  HKobayashi  HHashimoto  TItoh  KYamane  MNakamura  J Subcorneal pustular dermatosis type of IgA pemphigus: demonstration of autoantibodies to desmocollin-1 and clinical review. Br J Dermatol. 2000;143144- 148
Link to Article[[XSLOpenURL/10.1046/j.1365-2133.2000.03604.x]]
Harman  KEGratian  MJSeed  PTBhogal  BSChallacombe  SJBlack  MM Diagnosis of pemphigus by ELISA: a critical evaluation of two ELISAs for the detection of antibodies to the major pemphigus antigens, desmoglein 1 and 3. Clin Exp Dermatol. 2000;25236- 240
Link to Article[[XSLOpenURL/10.1046/j.1365-2230.2000.00624.x]]
Ohno  HMiyagawa  SHashimoto  T  et al.  Atypical pemphigus with concomitant IgG and IgA anti-intercellular autoantibodies associated with monoclonal IgA gammopathy. Dermatology. 1994;189 (suppl 1) 115- 116
Link to Article[[XSLOpenURL/10.1159/000246948]]
Hodak  ELapidoth  MDavid  M Effect of colchicine in the subcorneal pustular dermatosis type of IgA pemphigus. J Am Acad Dermatol. 1999;4091- 94
Link to Article[[XSLOpenURL/10.1016/S0190-9622(99)70533-5]]
Gruss  CZillikens  DHashimoto  T  et al.  Rapid response of IgA pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin. J Am Acad Dermatol. 2000;43923- 926
Link to Article[[XSLOpenURL/10.1067/mjd.2000.104002]]
Nousari  HCKimyai-Asadi  AKetabchi  NHughes  MAnhalt  GJ A patient with a refractory cutaneous bullous eruption. J Cutan Med Surg. 1999;3201- 204
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Figure 1.

Chest and inframammary area of a patient with aggressive IgA pemphigus shown before (A) and 8 months after (B) treatment with plasmapheresis and cyclophosphamide.

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Figure 2.

Histologic examination of a biopsy specimen from a patient with IgA pemphigus revealed an acantholytic neutrophil-rich intraepidermal blister (hematoxylin-eosin, original magnification ×40).

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Figure 3.

Direct immunofluorescence of a perilesional skin biopsy specimen from a patient with IgA pemphigus revealed patchy granular and linear IgA deposits on the keratinocyte cell surfaces (original magnification ×80).

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