Polymorphous light eruption in Child
Juvenile spring eruption is considered a subtype of PMLE that occurs most frequently in boys and young men. Juvenile spring eruption is characterized by papules and vesicles on the helices of the ears. Cases generally occur in the late winter and early spring. Cases have also been frequently noted to occur in concurrent cold weather with sun exposure.
PMLE is most commonly seen in women with lighter skin phototypes aged 20-30 years, but it can occur in all sexes, ages, and ethnicities. Approximately 20% of cases of PMLE present during childhood. Attacks most commonly occur during the spring and early summer months, especially after the first sun exposure of the season, and disappear during the winter.
The etiology of PMLE is not well understood. One theory is that UVR modifies antigens in the skin of PMLE patients to make them immunogenic. There are also data to suggest that PMLE involves a type IV delayed-type hypersensitivity reaction. The action spectrum for PMLE is UVA in most cases, but UVB and both UVA and UVB may also incite the response. Recent studies have shown that PMLE is associated with mutations in the NOD2 and TLR5 genes.
Although the condition frequently recurs, the tendency toward the development of PMLE and the severity of the eruption diminish with repeated sunlight exposure. This phenomenon is termed "hardening." As the summer proceeds, it has been observed that the incidence of new eruptions decreases. Prophylactic phototherapy supports this concept of hardening or tolerance.
Despite the variability of presentations, individual patients tend to experience the same clinical manifestations with each episode.
L56.4 – Polymorphous light eruption
238525001 – Polymorphous light eruption
Differential Diagnosis & Pitfalls
- – Shorter time to onset of eruption with urticarial plaques lasting 1-2 hours.
- – Check for circulating antinuclear antibodies (ANA) and other associated lupus antibodies; direct immunofluorescence will be positive; skin lesions can be located on sun-exposed and sun-protected areas (in contrast to PMLE, which is primarily in sun-exposed sites).
- – Characteristic target lesions; systemic symptoms are prominent (in contrast to PMLE, where fever, malaise, nausea, and headache are rare).
- – Lesions are very painful, and there is an elevated red blood cell protoporphyrin concentration.
- – Investigate for drug history; not seasonally associated and does not improve over time.
- – Will have abnormal porphyrin profile (elevated urine uroporphyrin and stool isocoproporphyrin).