Lecture 8 Part 2

Rosacea

• Very common in individuals with light skin, especially those of Irish or Scottish descent
• Often begins with intermittent flushing triggered by hot drinks, alcohol, caffeine, spicy food, wind, or sunlight, leading to facial warmth and redness.
• Redness can become permanent, and facial telangiectasias may develop.
• Next phase includes red papules and pustules, mainly on cheeks, nose, and forehead.
• Third phase involves thickening of the nose (rhinophyma) often after years of other symptoms
• Poorly understood, likely a component of facial cutaneous vasculature hyperresponsiveness.
• One theory suggests excess vasodilation causing skin edema and inflammation
• Primarily treated with topical antibiotics (e.g., metronidazole, clindamycin) to treat papules and pustules, though not general redness or rhinophyma, due to their supposed anti-inflammatory effects rather than microbial killing.
• Oral antibiotics (e.g., doxycycline) for severe cases, also work as anti-inflammatory agents
• Flushing triggers should be avoided
• For erythema and telangiectasias, pulsed dye laser treatment is often most effective due to its focus on hemoglobin and blood vessel destruction.
• Rhinophyma surgically treated for excess tissue.

Livedo Reticularis (LR)

• A bluish/violet, net-like pattern on the skin, usually on the legs.
• Usually no palpable component or symptoms.
• Mostly caused by skin exposure to cold temperature; resolves with warming
• Can be persistent and unrelated to cold exposure, presenting with skin "aching" if unrelated to cold, which warrants a review of systems.
• Represents increased deoxygenated blood in cutaneous venules, visible through skin.
• Any process increasing blood or reducing oxygen in venules can cause it:
  • Cold-related: arterial vasoconstriction and slower blood flow causing more deoxygenation.
  • Persistent: Increased viscosity (e.g., lupus anticoagulant, cryoglobulinemia) or vasodilation causes (e.g., neurologic or endocrine disorders).
• Usually does not require treatment unless persistent; assess for underlying causes if not resolving.
• Associated with lupus anticoagulant in women.

Raynaud's Phenomenon

• Relatively common in young women.
• Vasospastic disorder affecting the hands.
• Triggers (e.g., cold exposures):
  • Skin color change (white, then blue, then red)
  • Numbness or tingling
  • Swelling and burning/aching.
• Severe cases may cause gangrene or ulcers.
• Possible causes:
  • Sympathetic nervous system control of digital vessels issues
  • Blood hyperviscosity
  • Abnormalities or blockages in digital vessels, or local overproduction of vasoconstrictors/underproduction of vasodilators
• Primary Raynaud's (80-90%) has no underlying medical issues, while secondary Raynaud's is associated with an underlying disease.
• Diagnosis requires a review of systems and general examination. Checking for ANA and ESR in patients with Raynaud's could be useful; abnormalities of capillaries on the proximal nail folds might indicate systemic sclerosis as possible underlying disease
• Treatments:
  • Maintain warm extremities
  • Calcium channel blockers (e.g., nifedipine or diltiazem)

Pemphigus Vulgaris (PV) and Pemphigus Foliaceous (PF)

• Rare, autoimmune blistering diseases usually impacting 30s, 40s, or 50s

• More common in Jewish or Mediterranean people

• Often involves oral and cutaneous erosions/blisters (potentially severe, life-threatening)

• Differentiated based on affected desmoglein:

  • PV involves desmoglein 1 and 3, leading to oral and cutaneous involvement
  • PF involves only desmoglein 1 and hence primarily cutaneous involvement

• Treatment includes immunosuppressants (e.g. prednisone) and sometimes intravenous immunoglobulin or plasmapheresis in resistant cases.

Cutaneous Lupus

• Systemic autoimmune disorder, more common in women.
• Presents in three forms:
  • Acute Cutaneous Lupus Erythematosus (ACLE): Malar rash (cheeks and nasal bridge), erythema and edema.
  • Subacute Cutaneous Lupus Erythematosus (SCLE): Wide spread, erythematous, scaly, photo-distributed rash which avoids the face
  • Chronic Cutaneous Lupus Erythematosus (CCLE) or discoid lupus: 1-3 cm lesions (atropy, dilated pores), scaling, and pigmentary changes (hypo- or hyper-pigmentation). Primarily affects the conchal bowl of the ear
• Photo-related, and related to sun exposure damages to cells.
• Treatment emphasizes sun protection and sometimes systemic steroids, antimalarials (e.g. hydroxychloroquine and quinacrine) if severe, or other immunosuppressants

Polymorphous Light Eruption (PMLE)

• Often affects upper chest, arms, upper back (temporarily).
• Symptoms often appear in spring.
• Papules/plaques, erythematous and edematous, without scales.
• Mild, self-resolving, & rarely seeking medical attention
• Treatment generally combines sun protection and antimalarial medications, potentially low-intensity UV exposures to reduce symptoms

Porphyria Cutanea Tarda (PCT)

• Uncommon. More often found in men in their 40s, typically.
• Skin lesions (e.g. fragility, blisters, scarring, milia) affecting sun-exposed skin
• Deficiency in uroporphyrinogen decarboxylase (UROD) enzyme causes build-up of uroporphyrins that cause damage. This damage also is related to sun exposure.
• Often associated with liver disease (hemochromatosis, alcoholism, hepatitis C).
• Treated with phlebotomy (blood removal) and, if necessary, other immunosuppresants

Erythema Nodosum (EN)

• Inflammation in subcutaneous fat, producing painful red nodules on shins.
• Common in 20s, 30s, or 40s.
• Often associated with systemic disorders like sarcoidosis, infections, and medications.
• Treatment typically includes non-steroidal anti-inflammatory drugs (NSAIDs), elevation, rest. Systemic steroids may be used if symptoms persist.