18 Jun 2021

1/11/21: Pemphigus Vulgaris

PGY4 Tiffany Pan presented a case of a 38 year old female with  history of drug abuse who presented with skins lesions x4 weeks.  The lesions initially started on her R forearm as a blister that then popped and spread to involve her chest, back, upper extremities, lower extremities and mouth which also blistered and then popped.  Drainage described as both serous and pus.  Denies any vaginal mucosal involvement.  She had presented to OSH ~15 days prior for same concern.  She received IV antibiotics (unknown which) but left without completing the full treatment.  Rash became more painful, so she presented to LAC+USC for further care. 

ROS also revealed a R globe injury s/p repair with drainage x1 month, +b/l eye pain, history of genital ulcers and +pain and stiffness of the L hand.

Ancillary history revealed the patient was homeless, +unprotected sexual intercourse but denies history of STIs, 0.5 ppd x20 years, 1.5 bottles of hard liquor/day and crack/meth use (last used 1 day PTA) but denies IVDU.

Vitals: T 37.3,HR 90, RR 16, BP 129/89, O2 sat 99% on RA

Physical Exam significant for the patient appearing uncomfortable, R eye with mucoid discharge, upper and lower vermillion lips with erosions with yellow exudate but inner oral mucosa clear, scattered erosions of b/l nasolabial folds, normal cardiac and pulm exam, skin with scattered targetoid to annular eroded plaques in various stages of healing on chest, back abdomen, upper and lower extremities.

CBC: WBC 5.6> Hb 12.7/Hct 38.9 <Plt 470 (MCV 84, RDW 16.1%)

CMP:

Na 127/K 4.1/Cl 90/HCO3 24/BUN 10/Cr 0.81<Glucose 105

ALP 41>T.protein 5.3/Albumin 2.4/AST 23/ALT 11/Tbili 0.3/Dbili <0.2

Ca 7.9

Coags: PT 13.2, INR 1.01

Infectious workup initiated, with the following findings:

  • BCx negative, UA negative
  • Wound culture with 2+ staph aureus – Methicillin resistant
  • HSV1 IgG and HSV2 IgG elevated but IgM negative
  • HIV, RPR, GC/CT negative
  • Hepatitis panel negative

Derm workup:

  • ANA, ANCA negative
  • Complements wnl
  • Desmoglein-1 Ab: 178 (neg <14)
  • Desmoglein-2 Ab: 155 (neg <9)
  • BP180 Ab and BP230 Ab negative
  • Anti-BMZ negative

Skin biopsy was performed with pathology consistent with pemphigus vulgaris.

Treatment: Patient was started on methylprednisolone 250mg IV qday x3 days and tapered to 125mg IV qday x2 days.  She was also given rituximab 600mg IV qweekly x4 weeks and received the first dose while hospitalized.  She was discharged on PO doxycycline x10 days for MRSA from wound cultures along with prednisone 120mg qday until next dermatology appointment.

Teaching points:

  1. Differential for skin lesions can be narrowed based on the appearance of the lesions (just to list a few):
    1. Drug eruptions/DRESS: tend to be morbilliform in appearance (maculopapular eruption)
    1. TEN: appear in sheets instead of individualized areas
    1. Infections
      1. Fungal: retiform purpura (net like in appearance, angioinvasive)
      1. HSV: vesicles
    1. Levamisole vasculitis: retiform purpura with acral distribution
    1. Mycosis fungoides: erythrodermic (red) skin in the background with ulcers in chronic stages of the disease
    1. Pemphigus vulgaris: affects the younger population with more mucosal surfaces involved
    1. Pemphigus foliaceus: affects just the skin, no mucosal involvement
    1. Bullous pemphigoid: affects more the older population, with more bullae seen, can be treated with just high intensity topical corticosteroids
    1. Paraneoplastic pemphigus: lip dermatitis recalcitrant to therapy
  2. Pemphigus vulgaris is caused by humoral autoimmune response.  Oral lesions are the first manifestation in 50-70% of cases, develop flaccid blisters of clear content that can break easily resulting in painful erosions.  Palms and soles are usually spared.
  3. Treatment of PV:
    1. First line: Corticosteroids (oral or IV), Rituximab
    1. Nonsteroidal Adjuvant therapies: Azathioprine, Mycophenolate mofetil, Dapsone, Methotrexate, Cyclosporine
  4. Mortality rate is around 10%, with main cause of death 2/2 sepsis.

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