B-10 / B-11 / B-12 Papulosquamous/lp/erythroderma

Front 1

What type of T-cell infiltrates are seen in PLC vs PLEVA?

Back 1

PLC = CD4+
PLEVA = CD8+
(CD30-)

Front 2

Eponym for PRP?

Back 2

Devergie's Disease
aka Lichen ruber acuminatus
aka Lichen ruber pilaris

Front 3

Name the 5 types of PRP

Back 3

Type I = classical adult, 55% (most common adult type)
Type II = atypical adult, 5%
Type III = classic juvenile, 10%
Type IV = circumscribed juvenile, 25% (m/c juvenile type)
Type V = atypical juvenile, 5%

Front 4

Are men or women more affected with granular parakeratosis?

Back 4

Primarily women

Front 5

What is the defect in axillary granular parakeratosis?

Back 5

defect in processing profilaggrin to filaggrin, leaving keratohyalin retention in the stratum corneum

Front 6

What percentage of clonal dermatitis progress to CTCL over 5 years?

Back 6

20%

Front 7

How does the size of the lesions in Small Plaque Parapsoraisis differ from those in Large Plaque Parapsoriasis?

Back 7

Lesions of SPP < 5cm in diameter
Lesions of LPP > 5cm in diameter

Front 8

Name the three things that compose the clinical triad of Poikiloderma vasculare atrophicans

Back 8

1) Atrophy
2) Telangiectasia
3) Hyper/Hypopigmentation

Front 9

Which type of PRP has an Ichthyosiform scale on LE,
PPK with a coarse and lamellated scale,
occasional alopecia?

Back 9

Type II PRP (atypical adult form)

Front 10

Which type of PRP is in children and assoc with ichthyosiform scaling and a more chronic course which may include scleroderma-like changes?

Back 10

PRP -- Type V (Atypical Juvenile form)

Front 11

Which PRP is seen in HIV patient?

Back 11

Type VI
-- recalcitrant
-- may respond to HAART
-- patients may also have Acne conglobata, Hidradenitis suppurativa, Elongated follicular spines

Front 12

Name some drugs that can cause a PR-like reaction?

Back 12

Gold, ACE inhibitors, Flagyl, isotretinoin, arsenic, B- blocker, barbiturates, sulfasalazine, bismuth, clonidine

Front 13

Name the types of Pityriasis Rotunda

Back 13

Type I -- blacks & asians
-- hyperpigmented lesions, no family hx, possible assoc with internal malignancy

Type II - Caucasians
numerous lesions (>30); no assoc internal malignancy

Front 14

What are some associated disorders with Pityriasis Rotunda?

Back 14

Malnutrition
Mycobacterial diseases (TB, leprosy)
Malignancy (hepatocellular CA, gastric CA, multiple myeloma)
Hepatic cirrhosis

Front 15

What is the histopath of Pityriasis Rotunda similar to?

Back 15

Ichythosis vulgaris-like features
(absent or diminished granular cell layer, moderate hyperkeratosis, no parakeratosis);
May see increased basal cell pigment or pigment incontinence, epidermal atrophy, minimal infiltrate, and occasional follicular plugging

Front 16

What is the most common cause of erythroderma?

Back 16

dermatitis - 24%
psoriasis - 20%
drug reactions - 19%
CTCL - 8%
(25-30% are idiopathic)

Front 17

What is the most frequent complaint by patients with erythroderma?

Back 17

Pruritus in 90% (most severe in dermatitis or Sezary’s syndrome)

Front 18

What % of erythrodermic patients have Palmoplantar keratoderma?

Back 18

30%

Front 19

What % of erythrodermic patients have nail changes?

Back 19

40%
- often 'shiny nails'

Front 20

What % of erythrodermic patients have alopecia? which type is m/c?

Back 20

20%
M/C diffuse, non-scarring alopecia

Front 21

What is the duration of a drug-induced erythroderma?

Back 21

Usu will resolve in 2-6 weeks after withdrawal of the responsible drug

Front 22

How do drug induced erythrodermas present?

Back 22

If due to topical medications, usually will start as dermatitis.

If due to systemic medication, will start as morbilliform or scarlatiniform exanthem.

Front 23

What is the triad of Sezary syndrome?

Back 23

Erythroderma
Circulating malignant T cells
Generalized LAD

Front 24

Which of the bullous dermatoses most commonly presents as erythroderma?

Back 24

Pemphigus foliaceus

Impetigo-like blisters & erosions followed by collarettes of scale and scale-like crusts

Erythroderma usually preceded by localized lesions on face and upper trunk

Front 25

What % of patients presenting with cutaneous LP will have mucosal involvement?

Back 25

75%
(Oral LP is the only manifestation in 25% of cases)

Front 26

What contact allergens have been associated with LP?

Back 26

Dental restoratives: amalgam (mercury), copper & gold

Front 27

What vaccinations have been associated with LP?

Back 27

HBV

Front 28

Difference between Bullous LP and LP pemphigoides?

Back 28

Bullous LP -- bullae develop in pre-existing LP lesions (exaggerated Max-Joseph spaces)

LP pemphigoides -- bullae develop in uninvolved skin (coexistence of LP with BP); IgG against BPAg2

Front 29

What is main risk factor for hypertrophic LP, and what are they are risk for?

Back 29

Main risk factor is chronic venous stasis;
Risk of developing SCC.

Front 30

What is Graham Little-Piccardi-Lassueur syndrome?

Back 30

Variant of LPP with
Spinous or acuminated follicular lesions
- Cutaneous or mucosal LP
- Alopecia of scalp +/- atrophy

Front 31

What % of patients with LP have nail LP?

Back 31

10%

Front 32

What is the most common (and characteristic) form of oral LP?

Back 32

Reticular form -- asymptomatic, symmetric, involving the buccal mucosa

(Other forms include:
Atrophic
Bullous
Erosive - chronic desquamative gingivitis
Papular
Pigmented
Plaque-like -- seen in smokers

Front 33

What age-group is most commonly affected by lichen striatus?

Back 33

Children (9 months to 9 years old)

Different from Linear LP:
Linear LP more violaceous & in young adults (20-30’s)

Front 34

In patients with lichen striatus, what % have a family history of atopy?

Back 34

85%

Front 35

What drugs can cause a Lichenoid Drug Eruption?

Back 35

"GAP ATAS"
G old
A ntimalarials (hydroxychloroquine, quinidine)
P enicillamine/photo color developer

A mphetamines
T hiazides (HCTZ)
A CE inhibitors (captopril, enalapril)
S tatins

Also, B-blockers (labetalol, propanolol)

Front 36

How often does Acute GvHD occur?

Back 36

Acute GVHD occurs in 25-40% of recipients of grafts from HLA-matched sibling donors

Front 37

What are the main target tissues in Acute GvHD?

Back 37

Target tissues:
Liver – cholestatic hepatitis
GI tract – diarrhea
Skin

Front 38

Where does Acute GvHD dermatosis first appear?

Back 38

Acral Skin

Front 39

What is the prognosis for a patient w/ moderate acute GVHD after PBSCT?

Back 39

Approximately 50% may die as a consequence of mod-severe acute GVHD

Front 40

What percent of allogeneic PBSCT or BMT recipients get chronic GVHD?

Back 40

30-50%

Risk is 11 times greater in those with prior h/o acute GVHD

Front 41

What is the main cause of death in patient’s with chronic GVHD?

Back 41

Infections due to immunosuppression, both from GVHD & medications

Front 42

Histologically -- what are the varying Grades of Acute GvHD?

Back 42

Grade I
Focal or diffuse vacuolar change

Grade II
Plus keratinocyte necrosis & lymphs

Grade III
Plus focal DEJ separation with vesiculation

Grade IV
Plus formation of bullae

Front 43

What race is more commonly affected with Annular LP? And, what part of the body?

Back 43

Blacks more common
Penis & Scrotum more common

Front 44

What drugs tend to cause a Drug-Induced LP reaction that is photodistributed?

Back 44

5-FU
Carbamazepine
Diazoxide
Ethambutol
Quinine/Quinidine
TCNs
Thiazides
Furosemide

Front 45

Name some causes of lichenoid contact dermatitis

Back 45

color film developer
dental restorative material
musck ambrette
nickel
aminoglycosides
gold

Front 46

What is the DIF for Lichen Planus Pemphigoides?

Back 46

Linear deposition of IgG and C3 at the DE junction
IgG reacts to the 180/200 kDa antigen within BM zone

Front 47

What is the eponym for Keratosis Lichenoides Chronica?

Back 47

Nekam Disease