Symptoma

Actinic Prurigo

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]

[…] is often present all year round, but it is commonly worse in summer. [accessmedicine.mhmedical.com]

Patients will present with intensely itchy, erythematous papulonodular lesions on predominantly sun-exposed areas of the skin. [ncbi.nlm.nih.gov]

  • 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]

  • Burning Pain

    The lack of vesicles, burning pain, crusting, scarring, or an elevated red blood cell protoporphyrin test ruled out other differential diagnoses, including hydroa vacciniforme and erythropoietic protoporphyria. Dr. [mdedge.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]

  • Photosensitivity

    Actinic prurigo (AP) is a chronic photosensitivity disease, which is mainly described in Amerindians and rarely in Caucasians(1-6). [discovery.dundee.ac.uk]

    Photosensitivity Disorders. In Medicine International. 102. [gpnotebook.co.uk]

    Definition: Actinic prurigo is a chronic, intensely itchy skin disease due to an abnormal reaction to sunlight (photosensitivity) and predominantly affecting women. [ec.europa.eu]

    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]

    Association of Dermatologists Autoimmune photodermatoses – European Dermatology Forum Photosensitivity Guidelines Books about skin diseases See the DermNet NZ bookstore. [dermnetnz.org]

  • Hyperkeratosis

    Histologic Features: Acute lesions (indistinguishable from polymorphous light eruption): Spongiosis Superficial dermal perivascular lymphocytic infiltrate Dermal edema Chronic lesions: Hyperkeratosis Acanthosis Signs of photoadaptation External Links: [dermpedia.org]

    Histology of APC shows hyperkeratosis, scale crusts covering ulcerated areas, mild acanthosis, spongiosis, and mild vacuolar alteration of the basal layer. [obgynkey.com]

    Skin biopsies will show hyperkeratosis, spongiosis, and acanthosis in the epidermis with lymphocytic perivascular infiltration in the dermis. [ncbi.nlm.nih.gov]

  • 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]

    The symptoms of actinic prurigo include: Extremely itchy (pruritic) skin rash; Red and inflamed bumps (papules); Thickened patches (plaques); and/or Lumps (nodules) The symptoms of AP are often described as similar to those seen in atopic dermatitis (eczema [clinuvel.com]

    Initial studies with dupilumab showed that 85% of patients receiving dupilumab had a 50% reduction in the Eczema Area and Severity Index score compared to 35% in the placebo group. 8 While further studies are needed to determine the direct efficacy of [journals.sagepub.com]

  • Skin Thickening

    Since the lesions are intensely pruritic, excoriations, skin thickening, scarring, and hyper- or hypopigmentation may occur. Actinic cheilitis has been described as a hallmark finding in patients with actinic prurigo. [ncbi.nlm.nih.gov]

  • Hypopigmented Skin

    Congenital varicella syndrome is characterized by microcephaly, limb hypoplasia, cutaneous defects, hypopigmented skin, and autonomic neuropathy. Herpes zoster: The skin lesions that occur in herpes zoster are similar to those in varicella. [clinuvel.com]

  • Peripheral Neuropathy

    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]

  • Confusion

    AP cheilitis should not be confused with actinic cheilitis because they are separate entities. [ijpmonline.org]

    These reactions are usually caused by a confused immune system which reacts to compounds in sun-exposed skin. This is the commonest cause of sunlight reactions. actinic prurigo vs pmle [ekometal.com.tr]

  • 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]

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]

  • Lymphocytic Infiltrate

    Histologic Features: Acute lesions (indistinguishable from polymorphous light eruption): Spongiosis Superficial dermal perivascular lymphocytic infiltrate Dermal edema Chronic lesions: Hyperkeratosis Acanthosis Signs of photoadaptation External Links: [dermpedia.org]

    At the superficial portion of the submucosa, eosinophils and melanophages can be found, and deeper in the lamina propria, there are dilated capillary vessels, interstitial edema, and a band-like or nodular lymphocytic infiltrate. [obgynkey.com]

    The basal cells are vacuolised with lymphocytic infiltration in the sub-mucosal follicles. Eosinophils are conjunctival pigmentation are common findings. [clinuvel.com]

    Common histopathological features of AP are hyperkeratosis, parakeratosis, acanthosis, thickening of the basement membrane, perivascular inflammatory, lymphoid follicles, or nodular lymphocytic infiltrate, as well as eosinophils, mast cells, and angiogenesis [ijpmonline.org]

    It is necessary to differentiate AP from polymorphic light eruption, which is clinically similar, but microscopically does not show lymphocytic infiltrate with lymphoid follicles[ 12 ]. [wjgnet.com]

  • HLA-DR4

    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]

    Other studies have shown a strong association with HLA-DR4. [emedicine.medscape.com]

    Pivotal in the diagnosis of the boy was a positive association with an HLA DR4 test subtype: DRB1 Similarly, Mexican mestizos with actinic prurigo are likely to test positive to HLA A29-B39, and Canadian Cree First Nations people tend to test positive [mdedge.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]

    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]

If this is not possible, it is imperative that pregnancy be excluded before this treatment is instituted. Effective contraception must be used during the 4 weeks preceding and following treatment, as well as during the treatment period. [apps.who.int]

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]

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]

By means of the children's dermatology life quality index (CDLQI), children with actinic prurigo show high scores and closer to those obtained in children with xeroderma pigmentosum. [28] Prognosis The prognosis is poor, with frequent relapses, especially [emedicine.medscape.com]

Prognosis Actinic prurigo is a chronic disease process and can reoccur with repeated sun exposure. Treatment with immunosuppressive agents like thalidomide and cyclosporine A have been shown to be effective at long-term suppression of symptoms. [ncbi.nlm.nih.gov]

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]

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]

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. [5] [6] [7] Pathophysiology The pathophysiology of actinic prurigo remains largely [ncbi.nlm.nih.gov]

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]

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]

In case 1, the patient responded well to treatment with a topical corticosteroid and prevention measures; she had no lesions up to the last follow-up (14 mo after diagnosis). [wjgnet.com]

Although thalidomide is the best treatment known for this disease, there are various adverse effects that the patient should know and prevent. [dermatologyadvisor.com]