93, 94, 95: Actinic Prurigo, Hydroa Vacciniforme, Actinic Dermatitis Flashcards
What is Actinic Prurigo (AP) and how is it characterized?
Actinic Prurigo (AP) is a chronic sunlight-induced pruritic eruption characterized by papules or nodules, many of which are excoriated.
What populations are particularly affected by Actinic Prurigo?
The indigenous populations of North and South America are particularly affected by Actinic Prurigo.
What are the common clinical presentations of Actinic Prurigo?
Common clinical presentations include pruritus, pain or tingling sensations, conjunctivitis, and pruritic papules or nodules.
What are the noncutaneous findings associated with Actinic Prurigo?
Noncutaneous findings include mucosal involvement, cheilitis, and conjunctivitis.
What are the complications associated with Actinic Prurigo?
Complications include mild scarring, hypopigmentation from excoriations, and primary cutaneous B-cell lymphoma.
What is the etiology and pathogenesis of Actinic Prurigo?
Etiology includes induction by ultraviolet radiation, with TNF-α overexpressed by keratinocytes.
What is the presumed immunologic mechanism behind worsening symptoms in winter for Actinic Prurigo?
Immunologic tolerance presumably develops during the summer, leading to worsening symptoms in winter.
What is the typical onset age for Actinic Prurigo?
Usually by age 10 years, with the earliest onset in Native American populations at 4 to 5 years of age.
What environmental factor is primarily responsible for Actinic Prurigo?
Ultraviolet radiation.
What are the clinical features that suggest Actinic Prurigo rather than Polymorphous Light Eruption (PMLE)?
Features include disease onset in childhood, lesions on both exposed and sun-protected skin, and persistence of lesions beyond 4 weeks.
What laboratory tests support the diagnosis of Actinic Prurigo?
Laboratory tests include ANA and ENA to exclude other forms, and HLA DR4 typing.
What are the common management options for Actinic Prurigo?
Management options include higher-potency topical corticosteroids, topical calcineurin inhibitors, and oral antihistamines.
What is the treatment of choice for more severe or recalcitrant cases of Actinic Prurigo?
Thalidomide is the treatment of choice for severe or recalcitrant cases.
What are the potential adverse effects of Thalidomide when used for Actinic Prurigo?
Potential adverse effects include drowsiness, headache, constipation, weight gain, and increased risk of thromboembolism.
What are the cornerstone strategies for managing Actinic Prurigo?
The cornerstone strategies include sun protection and avoidance strategies.
What is Hydroa Vacciniforme and how does it typically present in patients?
Hydroa Vacciniforme (HV) is a rare, chronic photodermatosis characterized by photoinduced papules and vesicles that invariably scar after healing.
What are the common cutaneous findings associated with Hydroa Vacciniforme?
Cutaneous findings include initial erythema and swelling, eruption of tender papules and vesicles, and development of permanent scars.
What noncutaneous findings are associated with Hydroa Vacciniforme?
Noncutaneous findings include oral ulcers and eye involvement.
What is Hydroa Vacciniforme (HV)?
A rare, chronic photodermatosis characterized by photoinduced papules and vesicles that invariably scar after healing.
What is the typical onset age for Hydroa Vacciniforme?
Typically occurs in childhood, often before the age of 8.
What are the common clinical presentations of Hydroa Vacciniforme?
Intense burning or stinging sensation followed by papules and vesicles after sunlight exposure, leading to scarring.
What are the noncutaneous findings associated with Hydroa Vacciniforme?
Oral ulcers and eye involvement, including conjunctival hyperaemia and corneal erosions.
What complications can arise from Hydroa Vacciniforme?
Scarring, embarrassment among children, and potential development of HV-like lymphoma in severe cases.
What role does Epstein-Barr virus (EBV) play in Hydroa Vacciniforme?
EBV may play a role in the pathogenesis of HV, with EBV nucleic acids found in 85% to 95% of HV lesions.
How does sunlight exposure affect Hydroa Vacciniforme?
Sunlight exposure leads to the eruption of lesions within 24 hours, with a symmetrical distribution on the face and hands.
What is the typical demographic affected by Hydroa Vacciniforme?
Patients with light pigmentation are affected preferentially, and it is rare with an incidence of 0.34 cases per 100,000 individuals.
What laboratory tests are used to support the diagnosis of Hydroa Vacciniforme (HV)?
Laboratory tests include blood, urine, and stool porphyrin to exclude cutaneous porphyria; ANA & ENA to exclude cutaneous lupus erythematosus; evidence of EBV viremia may support the diagnosis or indicate disease activity.
What are the early histologic changes observed in Hydroa Vacciniforme (HV)?
Early histologic changes include intraepidermal vesicle formation with spongiosis, focal epidermal keratinocyte necrosis, and dermal perivascular neutrophil and lymphocyte infiltrate.
What is the differential diagnosis for Hydroa Vacciniforme (HV)?
The differential diagnosis includes photoexacerbated viral dermatoses, erythropoietic protoporphyria, polymorphic light eruption, actinic prurigo, subacute cutaneous lupus, xeroderma pigmentosum, and Hydroa vacciniforme-like lymphoma (HVLL).
What management options are available for Hydroa Vacciniforme (HV)?
Management options include medications such as antiviral therapy with acyclovir and valacyclovir, dietary fish oil, prophylactic phototherapy, and counseling on strict sun protection.
What is the clinical course and prognosis for Hydroa Vacciniforme?
The clinical course often resolves in adolescence but may persist into adult life. Males tend to have later onset and longer duration.
What is the significance of EBV DNA levels in patients with Hydroa Vacciniforme?
Higher levels correlate with disease activity and severity.
What characterizes Hydroa Vacciniforme initially after sun exposure?
It is characterized initially by erythema, sometimes with swelling, followed by the eruption of tender papules and vesicles within hours of sun exposure.
What are the permanent scars left by lesions in HV called?
Permanent, depressed, hypopigmented scars, also called atrophic scars.
What is Chronic Actinic Dermatitis (CAD) and its common features?
Chronic Actinic Dermatitis (CAD) is a rare, acquired, persistent eczematous eruption of exposed skin, often with pseudolymphomatous features.
What are the commonly implicated allergens in Chronic Actinic Dermatitis?
Commonly implicated allergens include sesquiterpene lactones from the Compositae family and various chemical components in sunscreens.
What is the significance of photopatch testing in diagnosing Chronic Actinic Dermatitis?
Photopatch testing helps determine the safe dose of UVA for testing, whether there is a photoallergy present, and identifies relevant allergens.
How does Chronic Actinic Dermatitis differ in its presentation among various skin types?
It is more common in individuals with darker skin phototypes but can occur in all skin types.
What are the clinical features and history associated with Chronic Actinic Dermatitis?
Development in apparently normal skin or in skin with previous eczema, often affecting middle-aged and elderly men.
What is the presumed pathogenesis of CAD in a patient with a history of atopic eczema?
CAD may result from an allergic reaction to UVR-altered DNA or associated molecules.
What allergens should be considered in a patient with Chronic Actinic Dermatitis who has a history of using sunscreens?
Sunscreens are commonly implicated allergens.
What allergens should be tested in a patient with Chronic Actinic Dermatitis who has a history of airborne contact dermatitis?
Allergens such as sesquiterpene lactones from Compositae plants and methylisothiazolinone should be tested.
What is the relationship between Chronic Actinic Dermatitis and allergic contact dermatitis?
Patients with CAD often have concomitant allergic contact dermatitis to airborne and other ubiquitous allergens.
What factors may influence the prevalence of allergens in different regions for CAD?
Relevant allergens will differ in different parts of the world.
What is the role of UVR in Chronic Actinic Dermatitis?
Chronic photodamage from UVR may impair normal skin immunosuppression.
What is the clinical feature of the eruption in CAD?
The eruption is eczematous, requiring lower doses of UVR to evoke CAD than to produce erythema.
What demographic is classically affected by Chronic Actinic Dermatitis?
Middle-aged and elderly men.
What are the common characteristics of cutaneous lesions in Chronic Actinic Dermatitis (CAD)?
Eczematous, patchy, or confluent lesions that can be acute, subacute, or chronic.
What are the common characteristics of cutaneous lesions in Chronic Actinic Dermatitis (CAD)?
- Eczematous, patchy, or confluent lesions
- Can be acute, subacute, or chronic
- Severe cases may show lichenification
- Commonly affects habitually exposed areas with sharp cutoff at clothing lines
- May present with pseudolymphomatous papules or plaques
- Sparing of deep skin creases, upper eyelids, and finger webs
- Irregular hyperpigmentation and hypopigmentation, sometimes vitiligo-like.
What laboratory tests are important for diagnosing Chronic Actinic Dermatitis (CAD)?
- Histology: Look for epidermal spongiosis, acanthosis, and perivascular lymphocytic infiltrate.
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Blood Tests:
- Lupus autoantibodies to exclude cutaneous lupus erythematosus.
- Circulating CD8+ Sézary cells in severe or erythrodermic CAD.
- Assess HIV status if suspected as a predisposing factor.
- Elevated Serum IgE levels correlate with more severe disease.
What is the significance of phototesting in the diagnosis of Chronic Actinic Dermatitis (CAD)?
- Phototesting is essential for diagnosing CAD as it assesses erythemal thresholds and responses to UVB and UVA.
- It helps identify drug reactions if only UVA sensitivity is present.
- Testing should be performed on uninvolved skin without prior topical or systemic steroid therapy to avoid false negatives.
What are the potential complications associated with Chronic Actinic Dermatitis (CAD)?
- Chronic Actinic Dermatitis (CAD) may coexist with cutaneous T-cell lymphoma (CTCL), but this is not expected by chance.
- CTCL can rarely present with severe CAD-like photosensitivity, necessitating careful investigation to exclude CTCL when suspected.
A middle-aged man presents with eczematous lesions on sun-exposed areas that worsen in summer. What is the most likely diagnosis?
Chronic Actinic Dermatitis (CAD), which commonly affects middle-aged and elderly men and worsens in summer.
A patient with suspected Chronic Actinic Dermatitis (CAD) undergoes phototesting. What findings would confirm the diagnosis?
Phototesting shows decreased erythemal thresholds and eczematous or pseudolymphomatous responses after UVB, UVA, or visible light irradiation.
A patient with suspected photoallergy has a history of using sunscreens. What secondary complication should be considered?
Secondary contact or photocontact sensitivity to sunscreens may complicate the clinical picture.
A patient with suspected photoallergy has a history of using methylisothiazolinone. What is the relevance of this allergen?
Methylisothiazolinone is an airborne allergen that can be photoaggravated, resembling CAD.
A patient with suspected photoallergy has a history of using window glass for sunlight exposure. What is the relevance of this method?
Window glass filters out UVB, enabling testing with whole-spectrum sunlight minus UVB.
A patient with suspected photoallergy has a history of using a slide projector. What is the purpose of this device?
A slide projector is used for phototesting with visible light.
What is the typical presentation of cutaneous lesions in CAD?
Eczematous, patchy or confluent, and can be acute, subacute, or chronic.