Presentation
The pathogenesis of AP is unclear, although familial occurrence is often seen, with a strong association with HLA DR4 and in particular HLA DRB1*0407, which is present only rarely in the general population(7,8). [discovery.dundee.ac.uk]
Cheilitis may be the sole clinical presentation. Histologically, the presence of lymphoid follicles in mucosa is pathognomonic. [orpha.net]
Patients will present with intensely itchy, erythematous papulonodular lesions on predominantly sun-exposed areas of the skin. [ncbi.nlm.nih.gov]
Entire Body System
- Fatigue
He was started on methotrexate 15 mg orally once weekly, however, due to gastrointestinal symptoms, not only switched to subcutaneous administration but also discontinued due to extreme fatigue. [journals.sagepub.com]
- Surgical Procedure
Medical Treatment Surgical Procedures Physical Modalities Photoprotective measures (seeking shades, sunscreens, appropriate clothing, hats and sunglasses) Phototherapy or photochemotherapy Topical corticosteroids or calcineurin inhibitors. [dermatologyadvisor.com]
- Short Stature
Paper zz. short stature Fact sheets and practice questions will be posted They include polymorphic light eruption, actinic prurigo, chronic actinic dermatitis and solar urticaria. [ekometal.com.tr]
Eyes
- Conjunctival Disease
In severe cases, excoriations, cheilitis, conjunctival disease, and scarring may develop. The disease has a strong genetic component and is commonly seen in American Indians of North, Central, and South America. [ncbi.nlm.nih.gov]
Skin
- Papule
These were key to the diagnosis of actinic prurigo, along with the fact that his photosensitive rash consisted of itchy papules that developed 30 minutes after sun exposure. [mdedge.com]
It is triggered by exposure to ultraviolet (UV) radiation and consists of intensely pruritic papules, plaques, or nodules that are typically excoriated and may scar. Secondary lichenification or eczematization may also occur. [visualdx.com]
Patients present with photodistributed, erythematous excoriated papules, cheilitis, and conjunctivitis. There is strong association with human leukocyte antigen DR4, especially the DRB1*0407 subtype. [semanticscholar.org]
Persistent pruritic papules or nodules may develop in covered and exposed areas, usually in the first decade. Photosensitivity may be severe. The condition may be perennial. [gpnotebook.co.uk]
[…] summer, but not always clearly related to sun exposure and often fail to clear completely in the winter Clinical findings Distribution Sun-exposed sites predominate although non-exposed sites can be affected Morphology Erythematous and often excoriated papules [pcds.org.uk]
- Excoriation
Patients present with photodistributed, erythematous excoriated papules, cheilitis, and conjunctivitis. There is strong association with human leukocyte antigen DR4, especially the DRB1*0407 subtype. [semanticscholar.org]
AT-A-GLANCE A rare chronic pruritic and excoriated papular or nodular eruption of sun-exposed and, to a lesser extent, nonexposed skin. Beginning in childhood, it may remit at puberty, exacerbate most often in summer, and fade in winter. [accessmedicine.mhmedical.com]
It is triggered by exposure to ultraviolet (UV) radiation and consists of intensely pruritic papules, plaques, or nodules that are typically excoriated and may scar. Secondary lichenification or eczematization may also occur. [visualdx.com]
Homepage Rare diseases Search Search for a rare disease Actinic prurigo Disease definition A rare, chronic, photodermatosis disease characterized by intensely pruritic, polymorphic, erythematous, excoriated and/or lichenified papules, macules, plaques [orpha.net]
The eruption of AP is characterized by pruritic papules and nodules, which are frequently excoriated. [ijdvl.com]
- Eczema
Other historical terms for the disease include solar eczema, prurigo solar, hereditary polymorphic light eruption, and hydroa aestivale. 1 AP occurs throughout much of the world. [accessmedicine.mhmedical.com]
Photoaggravated atopic dermatitis – assess for personal and family history of atopy, predominance in flexures; should respond to usual eczema treatment. Lymphomatoid papulosis – skin biopsies. [visualdx.com]
Actinic prurigo may look very like atopic dermatitis (eczema) but is more severe on sun-exposed sites. Areas affected include: Sun-exposed areas of the face such as the cheeks, nose, forehead, chin and earlobes. [dermnetnz.org]
- Ulcer
Ulceration was also present. [wjgnet.com]
Histology of APC shows hyperkeratosis, scale crusts covering ulcerated areas, mild acanthosis, spongiosis, and mild vacuolar alteration of the basal layer. [obgynkey.com]
Lower lip involvement includes swelling, scaling, fissures, hyperpigmentation, and ulcerations of the vermilion border. and in some cases could represent the only manifestation of the disease. [insights.ovid.com]
Chronic scratching, ulcers, weeping, crusting and scaling are found in 60–70% of patients. Actinic prurigo may look very like atopic dermatitis (eczema) but is more severe on sun-exposed sites. [dermnetnz.org]
- Pruritus
Symptoms: Intense pruritus Pruritus, tingling, or pain of the vermilion border (83%) Ocular symptoms – photophobia, pruritus, and lacrimation Signs: Excoriated papules are apparent, mostly on uncovered skin of the face and distal extremities, but AP can [visualdx.com]
PUBLISHED Jan 2017 Update in progress ClosePUBLISHED Jan 2017 Actinic prurigo The term 'actinic prurigo’ is the term used for a rare (less than 1:1,000) type of skin sensitivity induced by sunlight (photosensitivity); 'actinic' is Greek for 'sunlight', ‘pruritus [bad.org.uk]
Pruritus is universally present and is typically severe. 5, 10 Pain or tingling sensations also may be reported, especially ... [accessmedicine.mhmedical.com]
Cheilitis and pruritus are prominent features. The face and other sun-exposed areas are usually involved. Actinic prurigo has a high prevalence in women of childbearing age. Its treatment has been, among others, with thalidomide. [cab.unime.it]
Objective: To assess the clinical efficacy of pentoxifylline (PTX) in the treatment of AP patients by measuring its effects on lesions and pruritus. [karger.com]
Psychiatrical
- Suggestibility
Multiple studies note the presence of eosinophils and mast cells in the dermal layers of involved tissue which suggests a type-IVb (TH2)-driven response specifically. [ncbi.nlm.nih.gov]
Neurologic
- Headache
Adverse effects include drowsiness, headache and weight gain. Nerve conduction studies should be performed regularly to assess for peripheral neuropathy, another side-effect of thalidomine treatment. [clinuvel.com]
He responded well, but azathioprine was eventually stopped due to gastrointestinal side-effects and headache. Patient 6 was treated with azathioprine for a few months, but this was stopped due to side-effects. [ijdvl.com]
Workup
Chronic actinic dermatitis: clinical cases, diagnostic workup, and therapeutic management. J Cutan Med Surg 2009 ; 13(3): 121 – 128. Google Scholar | SAGE Journals | ISI 3. DeKoven, JG, Warshaw, EM, Belsito, DV. [journals.sagepub.com]
Treatment
Treatment Options Treatment options are summarized in Table I. Table I. [dermatologyadvisor.com]
Treatment with thalidomide has a major limiting side effect profile, which includes peripheral neuropathy and teratogenicity. Treatment with thalidomide is contraindicated during pregnancy or in individuals trying to become pregnant. [ncbi.nlm.nih.gov]
You can help by adding to it. ( May 2018 ) Treatment [ edit ] Currently there is no cure for actinic prurigo, and treatment focuses on relieving the dermatologic symptoms, by way of topical steroid creams or systemic immunosuppressants. [en.wikipedia.org]
Prognosis
@article{Lane1992ActinicPC, title={Actinic prurigo: clinical features and prognosis.}, author={Peter C. R. Lane and Daniel J Hogan and M. [semanticscholar.org]
The prognosis for AP is poor as it tends to run a chronic course with suboptimal response to treatment. [ FULL TEXT ] [ PDF ]* [ijdvl.com]
Immunocompromised patients have much poorer prognosis. The rate of developing congenital varicella syndrome is low. [clinuvel.com]
Characteristics and prognosis of idiopathic solar urticaria: a cohort of 87 cases. Arch Dermatol. 2003;139(9):1149–54. https://doi.org/10.1001/archderm.139.9.1149. Article Google Scholar 46. [link.springer.com]
Etiology
Only 19 variables were found to be statistically significant ( P P Conclusions Multiple epidemiologic, geographic, clinical, and immunologic factors are involved in the etiology of AP. [onlinelibrary.wiley.com]
Clinical Features: Familial photodermatosis of unknown etiology, most often affecting Native Americans Most commonly presents on the face with coarsening of facial features There is often an associated cheilitis, called follicular cheilitis or actinic [dermpedia.org]
Cheilitis (28%) Conjunctivitis (associated conjunctivitis is uncommon in whites) The precise etiology is not known, but HLA-DR4 is found in 93% of patients with AP (60-70% have the subtype DRB1*0407), suggesting that AP may be an HLA-restricted subset [visualdx.com]
Without treatment, this disease course remains chronic and can persist into adulthood. [1] [2] [3] Etiology The etiology remains unclear, but there is a strong genetic component suggesting an autoimmune basis for actinic prurigo. [ncbi.nlm.nih.gov]
Epidemiology
Only 19 variables were found to be statistically significant ( P P Conclusions Multiple epidemiologic, geographic, clinical, and immunologic factors are involved in the etiology of AP. [onlinelibrary.wiley.com]
However, in Latin America, AP is considered a separate entity, with characteristic epidemiologic, clinical, and histopathologic features. Immunohistochemical studies of Mexican patients showed that keratinocytes contain abundant TNF alfa. [dermatologyadvisor.com]
Epidemiology Begins in childhood between 6 and 8 years of age and is more common in women. Commonly seen in dark-skinned people, Fitzpatrick’s phototypes IV and V. Patients usually live in high-altitude, dry, sunny geographical areas. [obgynkey.com]
The human leukocyte antigen DR4 allele variant is present in 90% of cases, especially the DR4 subtype DRB1*0407 which is seen in 60% of cases. [4] Epidemiology Actinic prurigo is a rare photodermatosis in the United States. [ncbi.nlm.nih.gov]
Pathophysiology
The presence of increased levels of IgE, eosinophils, and mast cells suggests a hypersensitivity reaction as one of the mechanisms in the pathophysiology of AP [ 25 ]. [obgynkey.com]
Some studies suggest an increased prevalence of actinic prurigo in certain geographic areas with dry, warm climates at an altitude of at least 1000 meters above sea level.[6][7][8] Pathophysiology The pathophysiology of actinic prurigo remains largely [ncbi.nlm.nih.gov]
Prevention
A similar vaccine is also available to prevent herpes zoster. Treatment is required for all patients with herpes zoster. [clinuvel.com]
Association with HLA-DR4, specifically the HLA- DRB1*0407 and DRB1*0401 variants Prevention through avoidance of sunlight is the first-line therapy; thalidomide or other immunosuppressive agents may be required. [accessmedicine.mhmedical.com]
Photoprotection Taking measures to avoid sunlight exposure is important to prevent its occurrence and may require major adjustments to a person’s lifestyle. [skinsupport.org.uk]
The main goal is prevention by avoiding sun exposure. Patients must realise their condition will worsen during the sunniest months of the year and they must adhere to sun protection strategies to avoid or reduce outbreaks. [dermnetnz.org]