Endgames Case Review

Blisters in disguise

BMJ 2018; 360 doi: https://doi.org/10.1136/bmj.j5364 (Published 04 January 2018) Cite this as: BMJ 2018;360:j5364
  1. EA Dowlatshahi, dermatologist1,
  2. GFH Diercks, pathologist, head of immunodermatology laboratory1 2,
  3. MBA van Doorn, dermatologist, clinical pharmacologist1
  1. 1Erasmus Medical Centre, Rotterdam, Netherlands
  2. 2University Medical Centre, Groningen, Netherlands
  1. Correspondence to EA Dowlatshahi emmilia.dowlat{at}gmail.com

A 77 year old man with a history of hypertension, angina pectoris, chronic obstructive pulmonary disease, and prostate hyperplasia presented to the dermatology department with a 6 month history of thick, oozing, foul smelling skin lesions in the groin and around the umbilicus (fig 1), and erosions on the lips. He was mostly concerned about the smell from the lesions and the painful lip erosions, which made it difficult to eat. He had lost 7 kg in the past six months.

Fig 1

Vegetating periumbilical and inguinal plaques

His medication included metoprolol, isosorbide mononitrate, seretide, flixonase, tamsulosine, and desloratadine.

Physical examination revealed swollen, erosive lips with rhagades and crusts. He had thick oozing inguinal and periumbilical papillomatous plaques.

Laboratory studies showed erythrocyte sedimentation rate of 47 mm/h (normal range <20 mm/h) and mild eosinophilia (12.8% of leucocytes, normal range 1.4-6.2% of leucocytes) with a normal leucocyte count (normal range 3.5-10.0×109/L).

Histopathologic examination of skin biopsy from the groin showed a broadened epidermis with elongation of the rete ridges with focal hyperkeratosis and abscesses in the hair follicles, and a dense plasma cell infiltrate. Immunofluorescence of perilesional skin showed intercellular depositions of IgG, IgA, and C3 complement, and enzyme linked immunosorbent assay showed IgG antibodies directed against desmoglein 3.

Questions

  • 1. What is the most likely diagnosis and how is the condition diagnosed?

  • 2. How is this condition treated?

  • 3. What are the complications of this condition?

Answers

1. What is the most likely diagnosis and how is the condition diagnosed?

Short answer

Diagnosis of pemphigus vegetans, a subtype of pemphigus vulgaris, is based on the typical clinical appearance, immunofluorescence, and enzyme linked immunosorbent assay. Direct immunofluorescence on perilesional skin shows intercellular depositions …

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