Paraneoplastic pemphigus and Castleman's disease in the setting of herpes simplex virus infection.
A 14-year-old girl presented with a 3-week history of mucosal erosions, injected conjunctiva, dehydration, and respiratory distress. She had been treated with intravenous acyclovir for herpes simplex infection with positive herpes simplex virus immunoglobulin M and immunoglobulin G. Physical examination and imaging revealed a large abdominal mass. Incisional biopsy was obtained, and pathology demonstrated angiofollicular hyperplasia with hyalinized germinal centers and Castleman's syndrome-like features. Based on the mucosal erosions, herpes simplex virus serology and positive herpes simplex virus-1 direct fluorescent antibody, Castleman's disease secondary to overwhelming herpes simplex virus infection was the initial impression. The poor response to antivirals and subsequent development of a bullous eruption on the hands resulted in dermatology consultation. Skin biopsy was obtained from a bullae and revealed suprabasilar acantholysis with necrosis as well as upper dermal, perivascular, and interface infiltrate of lymphocytes and eosinophils. No viropathic changes were present. Direct immunofluorescence was significant for immunoglobulin G deposition intercellularly and along the dermoepidermal junction and focal trace C3 deposition along the dermoepidermal junction consistent with paraneoplastic pemphigus, later confirmed by indirect immunofluorescence. We report this case of paraneoplastic pemphigus secondary to Castleman's syndrome confounded by herpes simplex virus-1 positive mucosal erosions.
Department of Dermatology, Tufts Medical Center, Boston, Massachusetts 02111, USA.
Laine H
LH
Koch
Christle J
CJ
Layton
Monika
M
Pilichowska
Miguel J
MJ
Stadecker
Orr
O
Barak
D000051:Acantholysis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology / Q000821:virology; D000212:Acyclovir / Q000627:therapeutic use; D000293:Adolescent; D000998:Antiviral Agents / Q000627:therapeutic use; D001706:Biopsy; D005871:Castleman Disease / Q000188:drug therapy / Q000276:immunology / Q000473:pathology / Q000821:virology*; D003176:Complement C3 / Q000032:analysis / Q000276:immunology; D004804:Eosinophils / Q000276:immunology; D005260:Female; D005455:Fluorescent Antibody Technique; D006561:Herpes Simplex / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D006801:Humans; D007075:Immunoglobulin M / Q000032:analysis / Q000276:immunology; D008214:Lymphocytes / Q000276:immunology; D010257:Paraneoplastic Syndromes / Q000188:drug therapy / Q000276:immunology / Q000473:pathology / Q000821:virology*; D010392:Pemphigus / Q000188:drug therapy / Q000276:immunology / Q000473:pathology / Q000821:virology*; D016896:Treatment Outcome
D000998:Antiviral Agents; D003176:Complement C3; D007075:Immunoglobulin M; D000212:Acyclovir