XXXII International Academy of Pathology Congress Ahmed Alomari, MD - - PowerPoint PPT Presentation

▶
xxxii international academy of pathology congress
SMART_READER_LITE
LIVE PREVIEW

XXXII International Academy of Pathology Congress Ahmed Alomari, MD - - PowerPoint PPT Presentation

Mar 04, 2023 27 likes •410 views

XXXII International Academy of Pathology Congress Ahmed Alomari, MD Indiana University, School of Medicine Disclosures No relevant financial disclosures SL06 Inflammatory Dermatosis Case 005 Clinical Presentation 64yearold

slide-1
SLIDE 1

XXXII International Academy of Pathology Congress

Ahmed Alomari, MD Indiana University, School of Medicine

slide-2
SLIDE 2

Disclosures

  • No relevant financial disclosures
slide-3
SLIDE 3

SL‐06 Inflammatory Dermatosis Case 005

slide-4
SLIDE 4

Clinical Presentation

  • 64‐year‐old gentleman with one‐month

history of diffuse pruritic eruption involving trunk and extremities.

  • No associated constitutional symptoms
  • Medications: Metoprolol (HTN), Ibuprofen

(PRN headache), a daily multivitamin and fish

  • il
slide-5
SLIDE 5

Clinical Presentation

  • Initial physical examination: well‐demarcated

hyperpigmented and violaceous papules coalescing into larger plaques with some areas

  • f sparing.
  • Initial workup was negative for neoplasia,

infections and vitamin deficiencies

slide-6
SLIDE 6
slide-7
SLIDE 7

Initial Laboratory Results

  • HIV/HBV/HCV/RPR ‐ neg
  • ANA ‐ neg
  • CBC/CMP/Lipid/TSH ‐ wnl
  • Vitamin B1/B3/B6/B12 and zinc ‐ wnl
slide-8
SLIDE 8

Initial Pathology

  • Initial biopsy was read at an affiliated

institution and showed mild interface damage with a predominantly perivascular infiltrate and few eosinophils.

  • Working diagnosis: lichenoid drug eruption
slide-9
SLIDE 9

Initial Follow up

  • Despite intramuscular steroid injections, the

rash progressed with bullae formation predominantly on extremities.

  • Additional biopsies with direct

immunofluorescence were submitted for examination.

slide-10
SLIDE 10
slide-11
SLIDE 11
slide-12
SLIDE 12
slide-13
SLIDE 13
slide-14
SLIDE 14
slide-15
SLIDE 15

IgG

slide-16
SLIDE 16

C3

slide-17
SLIDE 17

Fibrinogen

slide-18
SLIDE 18

Final Diagnosis

  • Lichen Planus Pemphigoides
slide-19
SLIDE 19

Additional Laboratory Data

  • BP180 ‐ positive
  • BP 230 ‐ neg
slide-20
SLIDE 20

Lichen Planus Pemphigoides

  • Very rare disorder
  • Combination of lichen planus lesions and

tense bullous eruptions on skin both in involved and in not involved areas of the lichenoid eruption.

  • Postulated to be a blistering autoimmune

response to hemidesmosomal derived antigens exposed after lichenoid inflammation

slide-21
SLIDE 21

Causes

  • Drug reaction – ACE inhibitors, statins,

cinnarizine, Chinese herbs and weight reduction products

  • Complication of PUVA
  • Associated with Castleman’s disease
  • Related to internal malignancies
slide-22
SLIDE 22

Diagnosis

  • DIF with fibrinogen deposition at the dermal‐

epidermal junction with cytoid bodies and linear IgG and C3 along the basement membrane zone

  • Positive BP180 and/or BP230
slide-23
SLIDE 23

Treatment

  • Limited disease ‐ topical steroids
  • Initial treatment for extensive disease – oral

steroids or cyclosporine

  • Steroid sparing agents including tetracyclines,

niacinamide, isotretinoin, Dapsone, CellCept

  • High rate of remission after initial disease

control and patients can generally be weaned

  • ff all treatments
slide-24
SLIDE 24

Our Patient

slide-25
SLIDE 25

SL‐06 Inflammatory Dermatosis Case 006

slide-26
SLIDE 26

Clinical Presentation

  • A 44‐year‐old lady with a PMH of moderate to

severe hidradenitis suppurativa, relatively well controlled on adalimumab for the last 3 months presented with a new rash for the past 1‐2 weeks.

slide-27
SLIDE 27

Clinical Presentation

  • Red spots primarily on her legs, with few on

her upper extremities

  • They were not itchy, painful or otherwise

symptomatic

  • She denied any new medications (other than

Adalimumab). No recent illnesses or immunizations and review of systems was unremarkable.

slide-28
SLIDE 28

Clinical Presentation

  • Palpable purpuric to erythematous slightly

keratotic papules scattered on the bilateral anterior and medial lower legs primarily.

  • Additionally, she has scarred sinus tracts in the

bilateral axillae, inframammary and inguinal folds

slide-29
SLIDE 29
slide-30
SLIDE 30
slide-31
SLIDE 31

Labs

  • Overall unremarkable
  • WBC 9.3

– Mild lymphocytosis (3.6)

  • Punch biopsy of the right medial lower leg

– Specimens were submitted for both H&E and DIF given the question of palpable purpura

slide-32
SLIDE 32
slide-33
SLIDE 33
slide-34
SLIDE 34

Adalimumab‐induced Pityriasis Lichenoides Chronica (PLC)

  • Reported with all TNF‐ inhibitors
  • Time course is highly variable

– Onset of the eruption few weeks to a few months with the maximum latent period being 12 months

slide-35
SLIDE 35

Adalimumab‐induced Pityriasis Lichenoides Chronica (PLC)

  • Mechanism unclear

– Close temporal association with drug exposure – Role of drug as an antigenic trigger – Inherent drug immune dysregulating properties

slide-36
SLIDE 36

Adalimumab‐induced Pityriasis Lichenoides Chronica (PLC)

  • Etiology most likely form of T‐cell dyscrasia

– Reversible dependent on drug withdrawal – Adding to the spectrum of lymphomatoid drug reactions – T cell subset CD4+ > CD8+ – In two cases, T‐cell receptor gene rearrangement was negative

slide-37
SLIDE 37

Treatment

  • Started on low dose methotrexate (7.5 mg

weekly)

– Improvement within 6 weeks – New lesions continued to develop over 6 months

  • Patient elected to switch to infliximab for her

hidradenitis with complete resolution

slide-38
SLIDE 38

Questions