Benign Skin Lesions

Question 1

Name 4 benign skin appendage tumors

Answer 1

1. Syringoma: intraepidermal eccrine duct adenomas
2. Trichilemmoma: Seen in Cowden’s disease, tumor of outer hair root sheath
3. Sebaceous hyperplasia, rosacea, rhinophyma
4. Pilomatrixoma: Benign skin tumor derived from hair matrix; associated with Gardener, Turner, Trisomy 9, Kabuki, and Rubinstein-taybi syndrome

Vancouver notes page 9

Question 2

Regarding Xanthomas:
1. What are they?
2. Where do they occur?
3. What is the management?

Answer 2

WHAT:
- Localized deposits of lipids
- 50% occur in the setting of lipid abnormality

LOCATION:
- Most common upper eyelid and cheek

MANAGEMENT:
- Check lipids: if abnormal, treat lipid disorder and most lesions will regress
- No lipid abnormality, or failure to regress with normalized lipids = surgery

Vancouver page 9

Question 3

What is a Fibrous histiocytoma?
What is their histology?
What is the treatment?

Answer 3

WHAT:
- Benign painless tumor of mesenchymal origin
- Most common location: Sun exposed skin, orbital tissue

HISTOLOGY:
1. Biphasic cell population of fibroblasts (long thin cells) and histiocytes (round foamy looking cells)
2. Cartwheel pattern / whorl pattern

TREATMENT: Excision

https://www.pathologyoutlines.com/topic/softtissuebfh.html

Question 4

Regarding Rhinophyma, discuss:
1. What is it?
2. Epidemiology
3. Etiology
4. Histologic variants and features
5. Treatment

Answer 4

RHINOPHYMA:
- Phymatous type of rosacea, sebaceous hyperplasia characterized by marked skin thickenings and irregular surface nodularity of the nose, chin, forehead, one or both ears, and/or the eyelids

EPIDEMIOLOGY:
- ~50 year old males
- May harbour occult BCC

ETIOLOGY:
- Associated with Demodex Folliculorum mites (inhabit hair follicles)
- Possibly H. Pylori assocation
- Risk factors: Men > 50, Rosacea
- EtOH is NOT associated

CLINICAL PRESENTATION - May harbour occult BCC

HISTOLOGY:
1. Histologic variants:
- Glandular
- Fibrous
- Fibroangiomatous
- Actinic

2. Histological features:
   - Sebaceous gland hyperplasia
   - Connective tissue hyperplasia
   - Signs of chronic inflammation, perivascular infiltration of lymphocytes/inflammatory cells
   - Dilated blood vessels

TREATMENT:
1. Preventative: Defatting detergents
2. Medical (usually responds poorly, unless Rosacea alone): anti-inflammatories, antibiotics (e.g. topical flagyl, PO Doxycycline/Erythromycin), benzoyl peroxide, isotretinoin topical application, Ivermectin 1% gel (kills Demodex mites)
3. AVOID topical steroids
4. Surgical correction:
- Electrosurgery for serial shaves (must leave enough glands/follicles to allow for reepithelialization)
- Dermabrasion
- Skin flap elevation with dermal excision
- Subdermal defatting
- Lasers: Argon ND-Yag, Erb-Yag, or CO2 laser
- ± STSG following debridement

Question 5

What is the difference between Anaphylaxis and Angioedema?

Answer 5

ANGIOEDEMA: Angioedema Is swelling at deeper layers of skin (subcutaneous tissue) or submucosal tissue occurring either as an isolated form or as a component of anaphylaxis.
- Also can affect GI tract

ANAPHYLAXIS:
- Severe life-threatening allergic reaction affecting > 1 organ system
- IgE mediated (Gel & Coombs type 1 hypersensitivity reaction)

Question 6

What is the differential diagnosis of acute angioedema < 6 weeks?
What is the treatment?

Answer 6

1. Allergy (food, drug, contact, venom)
2. Infection (most common)
3. Drug (ACE inhibitor)

Treatment:
1. Oxygenation
2. Epinephrine
3. Steroids
4. Antihistamines
5. Aminophylline (bronchodilator)
6. Later avoidance and discontinuing offending agents

Question 7

What is the differential diagnosis of chronic angioedema > 6 weeks?

Answer 7

1. Systemic disease related
2. Idiopathic
3. Angioneurotic - Quincke’s edema (hereditary) (acute, recurrent mucosal edema, uvula example)
4. Estrogen dependent
5. Acquired C1-esterase inhibitor deficiency
6. Autoimmune
7. Thyroid disease

SIQE CAT

Question 8

Regarding Hereditary Angioedema, discuss:
1. Inheritance/Genetics
2. Pathophysiology
3. Clinical Presentation
4. Types
5. Diagnosis
6. Treatment

Answer 8

GENETICS:
- Gene: SERPING 1 Gene
- Inheritance: Autosomal dominance

PATHOPHYSIOLOGY:
1. Deficiency or dysfunction of C1 inhibitor
2. C1 cleaves C4 to cause the complement cascade (absence of C1INH leads to increased C1 and cleavage of C4 goes unchecked, so low C4)
3. C1 regulates early compliment pathway - decreases inflammation, prevents immune complex formation
4. Mediated by Bradykinin (can occur at any time when using medications ie. ACEi)
5. The exact mechanism leading to angioedema is unknown

CLINICAL PRESENTATION:
1. Recurrent episodes of angioedema (most often affects skin and mucosa of upper GI tract), without urticaria, typically asymmetric, abdominal pain common, unresponsive to steroids
2. Triggers = stress, infection, oral/dental manipulation

TYPES:
1. Type 1 (85%)
- Deficiency in C1 esterase inhibitor
- Decreased C1INH, elevated C1, decreased C4

2. Type 2 (15%)
   - Dysfunction of C1 esterase inhibitor
   - C1INH normal or increased, but decreased function
   - Elevated C1, decreased C4
3. Other types
   - Normal C1INH level and function, normal complement studies
   - At least 4 other disorders are known

DIAGNOSIS:
1. Clinical - Angioedema without urticaria, unexplained recurrent abdominal pain, family history unexplained laryngeal edema
2. Check C4 (low) and C1 Inhibitor levels (low or elevated) and C1 levels (high)

TREATMENT:
1. Acute: ABCs, C1 esterase inhibitor replacement (Berinert P or FFP)
2. Chronic:
- Tranexamic acid and Danazol for long-term prevention (raises C1 esterase inhibitor level)
- Use for procedural prophylaxis
- Icatibant (new, bradykinin B2 receptor antagonist)