 |
|
|
|
|
|
|
|
|
|
 |
 |
 |
 |
 |
 |
 |
 |
|
Background
This rash is classified as a photodermatosis, a subset of skin rashes which are exacerbated or initiated by exposure to ultraviolet radiation. These lesions are small, pruritic papules, papulovesicles, or urticarial plaques occurring in areas of sun-exposure. Characteristically, there is involvement of the dorsum of the hands and forearms, upper part of the arms, neck and face. In patients with the papulovesicle variant, the face is usually spared. Most patients are in the third decade and usually female.
The lesions occur several hours to days following sun exposure and subside within a 7-10 days if there is no further exposure. Populations living in temperate climates appear to be at greater risk affecting 10-20% of patients. There is also a genetic predisposition with autosomal dominant inheritance documented in cases in Finland.
Under the microscope, there are variable changes with a superficial and deep dermal infiltrate of lymphocytes and occasionally eosinophils and neutrophils. Early cases may only show changes in the papillary dermis. Edema may be prominent in the upper dermis and may form subepidermal bullae. The epidermis may show varying degrees of spongiosis with parakeratosis and acanthosis.
A variant of the disease has been termed juvenile spring eruption of the ears and is found in young males.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS INCIDENCE/PREVALENCE AGE SEX GEOGRAPHY EPIDEMIOLOGIC ASSOCIATIONS AFRICAN AMERICANS
Polymorphous light eruption in African Americans: pinpoint papular variant.
Kontos AP, Cusack CA, Chaffins M, Lim HW.
Department of Dermatology, Henry Ford Health System, Detroit, MI 48202, USA.
Photodermatol Photoimmunol Photomed. 2002 Dec;18(6):303-6. Abstract quote
BACKGROUND: Polymorphous light eruption (PMLE) is the most common chronic idiopathic photodermatosis usually manifesting as a papular eruption (3-6 mm), with several other morphological variants described.
METHODS: Between June 1998 and August 2001, nine patients presented with complaints of a pruritic pinpoint papular eruption associated with sun exposure. A detailed history and complete skin examination were performed along with a skin biopsy if active lesions were present. Phototesting to ultraviolet-A (UV-A), ultraviolet-B (UV-B) and visible light was performed in four patients. Antinuclear antibody (ANA) testing was performed in three patients. The diagnosis of PMLE was made based on the history, morphology of the lesions, results of phototesting and skin biopsy if available.
RESULTS: In all patients, pinpoint papules (1-2 mm) were observed on sun-exposed areas, sparring the face and flexural surfaces. All patients were African American women with skin type IV-VI and a mean age of 39.3 years (range 21-52 years). Phototest results were normal in three patients; one patient, who was on glyburide, had a decreased minimal erythema dose to UV-A. ANA testing was negative. Two histopathologic patterns were observed: (i) focal lichenoid and perivascular lymphohistiocytic infiltrate with red blood cell extravasation in four specimens and (ii) superficial and deep interstitial lymphocytic infiltrate with papillary dermal edema in the remaining three specimens. All patients responded to topical corticosteroids, broad-spectrum sunscreens and antihistamines.
CONCLUSION: Recognition of this pinpoint papular variant of PMLE in dark-skinned individuals is important in the evaluation and management of these patients.
DISEASE ASSOCIATIONS CHARACTERIZATION
PATHOGENESIS CHARACTERIZATION CYTOKINES
Differential Expression of Cytokines in UV-B-Exposed Skin of Patients With Polymorphous Light Eruption: Correlation With Langerhans Cell Migration and Immunosuppression.
Kolgen W, Van Meurs M, Jongsma M, Van Weelden H, Bruijnzeel-Koomen CA, Knol EF, Van Vloten WA, Laman J, De Gruijl FR.
Department of Dermatology, University Medical Center Utrecht, Utrecht.
Arch Dermatol. 2004 Mar;140(3):295-302. Abstract quote
BACKGROUND: Disturbances in UV-induced Langerhans cell migration and T helper (T(H)) 2 cell responses could be early steps in the pathogenesis of PLE.
OBJECTIVE: To establish whether UV-B exposure induces aberrant cytokine expression in the uninvolved skin of patients with polymorphous light eruption (PLE).
DESIGN: Immunohistochemical staining and comparison of microscopic sections of skin irradiated with 6 times the minimal dose of UV-B causing erythema and the unirradiated skin of patients with PLE and of healthy individuals.
SETTING: University Medical Center (Dutch National Center for Photodermatoses).Patients Patients with PLE (n = 6) with clinically proven pathological responses to UV-B exposure and normal erythemal sensitivity. Healthy volunteers (n = 5) were recruited among students and hospital staff.
MAIN OUTCOME MEASURES: Expression of cytokines related to Langerhans cell migration (interleukin [IL] 1, IL-18,and tumor necrosis factor [TNF] alpha); T(H)2 responses (IL-4 and IL-10); and T(H)1 responses (IL-6, IL-12, and interferon gamma). Double staining was performed for elastase (neutrophils), tryptase (mast cells), and CD36 (macrophages). RESULTS: The number of cells expressing IL-1beta and TNF-alpha was reduced in the UV-B-exposed skin of patients with PLE compared with the skin of healthy individuals (P<.05 for TNF-alpha). No differences were observed in the expression of T(H)1-related cytokines but fewer cells expressing IL-4 infiltrated the epidermis of patients with PLE 24 hours after irradiation (P =.03). After UV exposure TNF-alpha, IL-4, and, to a lesser extent, IL-10 were predominantly expressed by neutrophils.
CONCLUSIONS: The reduced expression of TNF-alpha, IL-4, and IL-10 in the UV-B-irradiated skin of patients with PLE appears largely attributable to a lack of neutrophils, and is indicative of reduced Langerhans cell migration and reduced T(H)2 skewing. An impairment of these mechanisms underlying UV-B-induced immunosuppression may be important in the pathogenesis of PLE.EXPERIMENTAL
An optimal method for experimental provocation of polymorphic light eruption.
Van De Pas CB, Hawk JL, Young AR, Walker SL.
St John's Institute of Dermatology, Guy's, King's, and St Thomas' School of Medicine, King's College London, England.
Arch Dermatol. 2004 Mar;140(3):286-92. Abstract quote
BACKGROUND: There is controversy about the best method to induce polymorphic light eruption (PLE) experimentally.Objectives To review articles on PLE induction and design a UV radiation protocol that improves success rates with clinically relevant doses of environmentally relevant solar-simulated radiation (SSR).
DESIGN AND SETTING: All articles on the experimental provocation of PLE published since 1980 were reviewed. Photoprovocation of lesions was studied in 25 PLE patients. The 24-hour minimal erythemal dose (MED) of SSR was determined. Thereafter, six 4 x 4-cm adjacent sites on previously affected and previously unaffected skin were exposed to 0.25, 0.5, 0.75, 1.0, 1.25, 1.5 MED of SSR for 3 to 4 consecutive days. The study periodwas autumn to spring in London, England (51 degrees north latitude).
MAIN OUTCOME MEASURES: Relationship between PLE induction and biological and physical exposure parameters.
CONCLUSIONS: The review shows that fractionated erythemally effective UV-A exposures were more successful than single-sunburning UV-B doses. Photoprovocation of PLE was successful in 68% of patients after 2 to 3 SSR exposures that were not necessarily erythemal. There was no difference in success rate between previously affected and previously unaffected skin. Our data indicate that PLE is more likely to be induced when the natural causes of the disease are simulated.
LABORATORY/RADIOLOGIC/
OTHER TESTSCHARACTERIZATION RADIOLOGIC LABORATORY MARKERS
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL VARIANTS ACTINIC PRURIGO
- Actinic prurigo.
Scheen SR 3rd, Connolly SM, Dicken CH.
J Am Acad Dermatol. 1981 Aug;5(2):183-90. Abstract quote
Actinic prurigo is a chronic photodermatitis found predominantly in North American Indians. Other terms have been used to describe similar cases in Central and South America and in Europe. Relatively little has been written about this condition in the English literature, and confusion exists over whether this is a form of polymorphic light eruption.
Actinic prurigo can be considered a unique variant of polymorphic light eruption; however, we believe that certain differences help to distinguish actinic prurigo as a separate disease entity.
Herein we report three cases of this disease and review the related literature. Characteristic clinical features include prurigolike papules and cheilitis. Pruritus is the predominant symptom, and a familial tendency and an early age at onset are usually noted. Results of karyotyping and analysis of sister chromatid exchanges were normal in two of our patients so tested. Skin testing for photosensitivity has yielded inconsistent results, and use of light testing for diagnosing actinic prurigo does not seem to be a predictable procedure.
Actinic prurigo is a chronic disease that often is refractory to therapy.JUVENILE SPRING ERUPTION
Juvenile spring eruption: clinicopathologic features and phototesting results in 4 cases.
Stratigos AJ, Antoniou C, Papadakis P, Papapostolou A, Sabatziotis D, Tranaka K, Tsara K, Katsambas AD.
Department of Dermatology, Photobiology Unit, University of Athens, Andreas Sygros Hospital, 5 Dragoumi Street, Athens 161 21, Greece.
J Am Acad Dermatol. 2004 Feb;50(2 Suppl):S57-60. Abstract quote
Juvenile spring eruption is a distinct photodermatosis characterized by the development of papules and vesicles on light-exposed areas of the ears usually in the early springtime. It primarily affects boys and young men, and has a tendency to occur in the form of small epidemics.
We report a similar outbreak in separate groups of soldiers who were performing military exercises during cold and sunny weather of a midwinter season. The clinicopathologic features and phototesting results are described in 4 of these cases. All patients showed normal erythemal responses to monochromator phototesting with UV and visible wave bands. Photoprovative testing with repeated daily exposures of the ears to a broadband UVA source provoked diffuse erythema and itching in 1 case, whereas similar photoprovocation of a nonaffected area, ie, the flexor surface of the forearm, in 2 patients did not yield a skin reaction.
Although the cause of juvenile spring eruption is not known, our observations further strengthen the hypothesis that the disorder is a localized variant of polymorphous light eruption.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL
SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHERCHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE
Polymorphic light eruption: an immunopathological study of evolving lesions.
Norris PG, Morris J, McGibbon DM, Chu AC, Hawk JL.
Institute of Dermatology, St. Thomas' Hospital, London, U.K.
Br J Dermatol. 1989 Feb;120(2):173-83. Abstract quote
Polymorphic light eruption (PLE) papules were successfully induced on previously affected sites in 11 out of 14 patients with PLE 4-20 h after single exposures to suberythemogenic doses of solar simulated radiation.
Histological examination of biopsies performed 1 h, 5 h, 24 h, 72 h and 144 h post-irradiation revealed onset within 5 h of perivascular cellular infiltration. The infiltrate was dominated by lymphocytes in both early and established lesions, without evident epidermal pathology. Immunohistochemistry demonstrated a predominance of CD4+ cells in lesions up to 72 h post-induction, but later biopsies were dominated by a CD8+ infiltrate. Significantly increased numbers of dermal macrophages and CD1b+ cells were detected 1 h and 5 h post-irradiation, respectively.
These findings are consistent with a delayed type hypersensitivity response underlying the pathogenesis of polymorphic light eruption.ELECTRON MICROSCOPY
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
PROGNOSIS CHARACTERIZATION
TREATMENT CHARACTERIZATION GENERAL
Management of polymorphous light eruption : clinical course, pathogenesis, diagnosis and intervention.
Fesq H, Ring J, Abeck D.
Department of Dermatology and Allergy, Technical University Munich, Munich, Germany.
Am J Clin Dermatol. 2003;4(6):399-406. Abstract quote
Optimal management of patients with polymorphous light eruption (PLE), the most frequent photodermatosis, requires knowledge of the individual clinical course of the disease and pathogenic factors. As PLE often causes problems during leisure-time activities and holidays, resulting in a substantial loss of quality of life, prophylaxis is the most important therapeutic approach.
Management of PLE must, therefore, focus on basic preventative measures and additional therapeutic approaches, depending on the clinical condition. PLE can be classified into four severity groups (mild, moderate-to-severe, severe and therapy-resistant), which are useful for determining appropriate prophylactic measurements. No specific laboratory tests are available for the diagnosis of PLE, therefore, a clinician must rely on the clinical appearance of the disorder (e.g. clinical symptoms, the location of the lesions, the relationship of the occurrence of the lesions with sun exposure and the time course of the lesions) as well as a patient's medical history in order to make a diagnosis.Basic preventative management of PLE consists of adequate sun protection comprising avoidance of sun exposure, the use of textile sun protection and the application of broadband sunscreens with high UVA protection potential. Other supportive measurements have to be managed individually and are dependent on the patient's medical history and the severity of the disease.
Topical antioxidants, systemic immunomodulation, photo(chemo)therapy and systemic immunosuppression may be required in some cases of PLE. Topical antioxidants represent a new treatment approach for moderate-to-severe PLE and are an effective and well tolerated option for this patient population. Severe PLE also requires photo(chemo)therapy. Phototherapy can be in the form of 311 nm UVB or UVA1 irradiation. In cases where 311 nm UVB or UVA1 are ineffective, psoralen plus UVA (PUVA) bath therapy may be used. However, PUVA bath therapy must be used with caution because it is associated with acute and long-term adverse effects. In rare exceptions we would consider using oral PUVA therapy. However, in our outpatient department, quality of life of most patients is improved with the treatment regimens that are recommended for patients with moderate-to-severe PLE, without the need for photo(chemo)therapy.Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
Basic Principles of Disease
Learn the basic disease classifications of cancers, infections, and inflammation
Commonly Used Terms
This is a glossary of terms often found in a pathology report.Diagnostic Process
Learn how a pathologist makes a diagnosis using a microscopeSurgical Pathology Report
Examine an actual biopsy report to understand what each section meansSpecial Stains
Understand the tools the pathologist utilizes to aid in the diagnosisHow Accurate is My Report?
Pathologists actively oversee every area of the laboratory to ensure your report is accurateGot Path?
Recent teaching cases and lectures presented in conferences
Pathologists Who Make A Difference
Search for a Physician Specialist
Clinical Photo
Last Updated October 17, 2005
Send
mail to The Doctor's Doctor with
questions or comments about this web site.
Read the Medical Disclaimer.
Copyright © The Doctor's Doctor