doi: 10.1111/1346-8138.12650
Journal of Dermatology 2014; 41: 1003–1005
CONCISE COMMUNICATION
Refractory bullous pemphigoid leaving numerous milia during recovery Shusuke UCHIDA,1 Naoki OISO,1 Hiroshi KOGA,2,3 Norito ISHII,2,3 Kazunori OKAHASHI,1 Hiromasa MATSUDA,1 Takashi HASHIMOTO,2,3 Akira KAWADA1 1
Department of Dermatology, Kinki University Faculty of Medicine, Osaka-Sayama, 2Department of Dermatology, Kurume University School of Medicine, and 3Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan
ABSTRACT Recovery with milia may occur in bullous pemphigoid (BP), mucous membrane pemphigoid (MMP) and epidermolysis bullosa acquisita (EBA). Scarring commonly occurs in MMP and EBA. Here, we report a 62-year-old man patient with BP, who was left with numerous milia during recovery. The patient had immunoglobulin (Ig)G autoantibodies to the recombinant protein of the BP180-NC16a domain and the soluble 120-kDa ectodomain of BP180 (linear IgA bullous dermatosis [LAD]-1). There are cases of BP with IgG autoantibodies to LAD-1 and/or the recombinant protein of BP180 C-terminal domain. Extensive milia formation during recovery may be associated with immunological predisposition and/or improper interaction between hemidesmosomes and the extracellular matrix components.
Key words: BP180-NC16a domain, bullous pemphigoid, epidermolysis bullosa immunoglobulin A bullous dermatosis-1, milium, mucous membrane pemphigoid.
INTRODUCTION Bullous pemphigoid (BP) is an autoimmune blistering disorder caused by immunoglobulin (Ig)G autoantibodies to two hemidesmosomal components, BP180 and BP230.1,2 Here, we report a 62-year-old man patient with refractory BP showing numerous milia during recovery. The patient had IgG autoantibodies which were reactive to the recombinant protein (RP) of the immunodominant NC16a domain of BP180 (BP180-NC16a) and the soluble 120-kDa ectodomain of BP180 (linear IgA bullous dermatosis [LAD]-1), as well as weak IgA reactivity with the BP180-NC16a RP.
CASE REPORT A 62-year-old Japanese man complaining of increasing skin lesions was referred to us in September 2013. At the first visit, physical examination revealed multiple erythemas with papules and vesicles on the entire body. Then, the erythemas spread and coalesced, forming many large erythemas with tense vesicles and bullae, pustules and erosions (Fig. 1a). Mucosal membranes were not involved. Laboratory examinations at the first visit showed slight increases of leukocytes (10 200/lL; normal, 3900–9300/lL) and eosinophils (4.4%; normal, 0.2–4.1%) and increased C-reacted protein (CRP; 1.111 mg/dL, normal
Journal of Dermatology 2014; 41: 1003–1005
CONCISE COMMUNICATION
Refractory bullous pemphigoid leaving numerous milia during recovery Shusuke UCHIDA,1 Naoki OISO,1 Hiroshi KOGA,2,3 Norito ISHII,2,3 Kazunori OKAHASHI,1 Hiromasa MATSUDA,1 Takashi HASHIMOTO,2,3 Akira KAWADA1 1
Department of Dermatology, Kinki University Faculty of Medicine, Osaka-Sayama, 2Department of Dermatology, Kurume University School of Medicine, and 3Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan
ABSTRACT Recovery with milia may occur in bullous pemphigoid (BP), mucous membrane pemphigoid (MMP) and epidermolysis bullosa acquisita (EBA). Scarring commonly occurs in MMP and EBA. Here, we report a 62-year-old man patient with BP, who was left with numerous milia during recovery. The patient had immunoglobulin (Ig)G autoantibodies to the recombinant protein of the BP180-NC16a domain and the soluble 120-kDa ectodomain of BP180 (linear IgA bullous dermatosis [LAD]-1). There are cases of BP with IgG autoantibodies to LAD-1 and/or the recombinant protein of BP180 C-terminal domain. Extensive milia formation during recovery may be associated with immunological predisposition and/or improper interaction between hemidesmosomes and the extracellular matrix components.
Key words: BP180-NC16a domain, bullous pemphigoid, epidermolysis bullosa immunoglobulin A bullous dermatosis-1, milium, mucous membrane pemphigoid.
INTRODUCTION Bullous pemphigoid (BP) is an autoimmune blistering disorder caused by immunoglobulin (Ig)G autoantibodies to two hemidesmosomal components, BP180 and BP230.1,2 Here, we report a 62-year-old man patient with refractory BP showing numerous milia during recovery. The patient had IgG autoantibodies which were reactive to the recombinant protein (RP) of the immunodominant NC16a domain of BP180 (BP180-NC16a) and the soluble 120-kDa ectodomain of BP180 (linear IgA bullous dermatosis [LAD]-1), as well as weak IgA reactivity with the BP180-NC16a RP.
CASE REPORT A 62-year-old Japanese man complaining of increasing skin lesions was referred to us in September 2013. At the first visit, physical examination revealed multiple erythemas with papules and vesicles on the entire body. Then, the erythemas spread and coalesced, forming many large erythemas with tense vesicles and bullae, pustules and erosions (Fig. 1a). Mucosal membranes were not involved. Laboratory examinations at the first visit showed slight increases of leukocytes (10 200/lL; normal, 3900–9300/lL) and eosinophils (4.4%; normal, 0.2–4.1%) and increased C-reacted protein (CRP; 1.111 mg/dL, normal
