Polymorphous light eruption
| Polymorphous light eruption | |
|---|---|
| Classification and external resources | |
| Specialty | dermatology |
| ICD-10 | L56.4 |
| ICD-9-CM | 692.72 |
| DiseasesDB | 10327 |
| MedlinePlus | 001477 |
| eMedicine | derm/342 |
Polymorphous light eruption (PLE), or polymorphic light eruption (PMLE), is a skin condition triggered by sunlight.
Classification
Two subtypes have been described:[1]
- Juvenile spring eruption is a cutaneous condition that affected the helices of the ears, particularly in boys because their ears are relatively more exposed to sunlight.[1]
- Benign summer light eruption is a cutaneous condition, and a name used in continental Europe, and particularly France, to describe a clinically short-lived, itchy, papular eruption particularly affecting young women after several hours of sunbathing at the beginning of summer or on sunny vacations.[1] After a person experiences this condition once, it will likely recur annually. Onset is generally in the teen years or 20s; the condition can then last the remainder of a person's life, with annual flare-ups after the first exposure to the sun each year.
Photosensitivity is also found in some of the Porphyrias. Nearly all cases of Porphyria cutanea tarda[2] exhibit blister formation on the skin within 2–4 days of light exposure. Variegate porphyria[3] and Hereditary coproporphyria[4] can also exhibit symptoms of light induced blisters.
Signs and symptoms
Symptoms include skin irritations, which may be itchy or painful, and are sometimes confused with hives. These irritations appear upon or shortly after exposure to sunlight,[5] and may last from 1 to 7 days. Lesions have been photographed showing fluid-filled blisters.
Secondary symptoms include flu-like symptoms; body aches, skin sensitivity, muscle aches, fever, dizziness, exhaustion, vision impairment, and disorientation.
Causes
The cause of PLE is not yet understood. It is thought to be due to a type IV delayed-type hypersensitivity to an allergen produced in the body following sunlight exposure.[6] It can be provoked by UVA or UVB rays. Some progression to autoimmune disease has been observed.[7]
In cases of cutaneous Porphyria alcohol consumption has been found to be a strong trigger.[8] Consumption of alcohol inhibits certain portions of Porphyrin synthesis which creates the excess enzymatic intermediaries. These compounds, upon circulation in the blood stream and oxidized by light are responsible for the itchy blisters and plaques.[9]
Prognosis
Generally, PLE resolves without treatment; also, PLE irritations generally leave no scar. However, in severe cases the use of steroids is necessary to help reduce inflammation and increase quality of life of the patient. There are also other therapies for patients who are severely impacted, such as light therapy to harden the skin's surface.
Epidemiology
The cases of this condition are most common between the spring and autumn months in the northern hemisphere.
Typically, 5-20% of fair skinned populations are affected, but it can occur in any skin type.[6] It is more common in females than in males. The condition can affect all ethnic groups and research suggests that 20% of patients have a family history of the complaint. Those suffering from PLE usually do so by age 30.
See also
References
- 1 2 3 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
- ↑ http://www.porphyriafoundation.com/about-porphyria/types-of-porphyria/PCT
- ↑ http://www.porphyriafoundation.com/about-porphyria/types-of-porphyria/VP
- ↑ http://www.porphyriafoundation.com/about-porphyria/types-of-porphyria/HCP
- ↑ Schornagel IJ, Sigurdsson V, Nijhuis EH, Bruijnzeel-Koomen CA, Knol EF (July 2004). "Decreased neutrophil skin infiltration after UVB exposure in patients with polymorphous light eruption". J. Invest. Dermatol. 123 (1): 202–6. PMID 15191561. doi:10.1111/j.0022-202X.2004.22734.x.
- 1 2 http://www.clinuvel.com/skin-conditions/common-skin-conditions/polymorphous-light-eruption-ple
- ↑ Hasan T, Ranki A, Jansen CT, Karvonen J (September 1998). "Disease associations in polymorphous light eruption. A long-term follow-up study of 94 patients". Arch Dermatol. 134 (9): 1081–5. PMID 9762018. doi:10.1001/archderm.134.9.1081.
- ↑ http://alcalc.oxfordjournals.org/content/35/2/109
- ↑ Lim, H. W.; Poh-Fitzpatrick, M. B.; Gigli, I (1984). "Activation of the complement system in patients with porphyrias after irradiation in vivo". Journal of Clinical Investigation. 74 (6): 1961–1965. PMC 425382
. doi:10.1172/JCI111616.