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A 28-year-old female patient suffering from LEOPARD syndrome presented, asking for the removal of lentigines (covering her face and most of her body) for aesthetic reasons. Intense pulsed light technology has been already used successfully for the removal of various benign pigmented lesions and it proved effective in this very rare case as well.
and the introduction of pulsed lasers (both in the early 80's) has lead to more effective and safer treatments of various benign skin disorders (e.g. port wine stains), in comparison to continuous wave lasers that were being used earlier. In the early 90's an intense pulsed light (IPL) source was introduced that emitted pulsed broadband wavelength light and not single wavelength light as lasers do. Both laser and IPL devices have been extensively used for the treatment of pigmented and vascular skin lesions.
We present a case of LEOPARD syndrome where the multiple lentigines covering the face and body of the patient have been treated with the use of an IPL source.
1. Case report
A 28-year-old woman with LEOPARD syndrome presented, asking for aesthetic treatment of multiple lentigines covering her face, upper arms, neck, chest, abdomen and back. The lentigines were congenital and associated with the syndrome. Her skin phototype was III according to Fitzpatrick's scale, making it difficult to treat and more prone to complications and side effects from the treatments.
Photoderm VL/PL (ESC Scharplan, Yoknaem, Israel) was used for the treatments. It is a flashlamp IPL source, emitting noncoherent light (from 400 to 1200 nm). Several cut-off filters are available (at 515, 550, 570, 590, 615, 645, 695, 755 nm) to block from the emitted pulse, light of shorter wavelength. Each pulse can be fired as a single shot or it can be divided in two or three sub-pulses. Energy flux (in J/cm2), duration of each sub-pulse (in ms) and delay between sub-pulses (in ms) can be adjusted.
Several test spots (with the 8 mm×15 mm waveguide) were performed whenever a new area was treated, in order to determine the most appropriate treatment parameters for the specific area.
When treating the entire area however, the 8 mm×35 mm waveguide was used. A thin (2–3 mm) layer of chilled clear water-based gel was applied to the skin in the area to be treated, right before each pulse was fired and the spot of the treatment head was kept in contact with the surface of the gel. The patient experienced only a mild stinging–burning sensation after each pulse and local anaesthesia was not necessary.
Facial skin was treated more aggressively than any other area. When an area was to be retreated, depending upon patient's feedback on post-treatment erythema and side effects and on the result of previous treatment a 10% increase in energy fluence was applied, if necessary. Treatment parameters are shown in Table 1.
During a time period of 19 months, the patient visited us 14 times altogether. She had five treatments on the face, two on the neck, three on the abdomen, three on the chest, one on the back, three on the shoulders and two on the upper arms. More than one area was sometimes treated during a single session. A minimum 4-week interval was kept between treatments of the same area.
Immediately after the treatment, erythema developed over the treated areas, lasting for an average of 24 h. Instructions for the application of hydrating and sun-blocking creams during the next couple of weeks following each treatment were given. Only once, 3 days after treating the entire back, crusting developed over many ‘footprints’ of the treatment waveguide. As the crusting subsided, hyperpigmentation developed under some of those ‘footprints’, but resolved completely in a time period of 18 months.
All of the treated lentigines responded to therapy. Some were completely removed and the rest have all faded from their original dark-brown colour to a varying extent. The patient feels very satisfied with the result so far (Fig. 1, Fig. 2, Fig. 3, Fig. 4) . None of the treated lentigines have relapsed and returned to their original colour so far (2 years after the first treatment).
Fig. 4Photo of patient's back 12 months after the 1st (and only) treatment. Notice significant improvement but hyperpigmented footprints are still vaguely present. They completely resolved 6 months later.
Gorlin et al. first described LEOPARD syndrome in 1969. It is a complex of congenital abnormalities (L—lentigines, E—electrocardiographic conduction defects, O—ocular hypertelorism, P—pulmonary stenosis, A—abnormalities of genitalia, R—retardation of growth, D—deafness sensironeural). It is a familial syndrome following a dominant autosomal transmission with varying degrees of penetration.
The presence of lentigines is an aesthetic problem since malignant transformation of the lentigines has not been reported. Biopsy of lentigines in our patient was not considered necessary for the diagnosis of the syndrome. We were able to find only two reports for the treatment of lentigines in LEOPARD syndrome: in a young woman repetitive self-induced factitial dermatitis lead to a sequence of erosive nonscarring lesions that eradicated the lentigines within a time period of approximately 6 years. Residual lentigines were left only in her back because it was inaccessible to her to scratch.
The second case is one where facial dermabrasion and light electrodessication of the lentigines on exposed surfaces has lead to considerable cosmetic improvement.
Many different modalities other than laser or light sources can be used to treat benign pigmented skin lesions: dermabrasion, electrodessication (as already mentioned), cryotherapy, surgical excision, chemical peels. However, the high rates of effectiveness and low rates of complications and side effects of laser treatments have made it the treatment of choice.
To our knowledge, no other report exists on the use of laser or other light source for the improvement of lentigines in LEOPARD syndrome. However, laser and light sources have been used extensively for the treatment of various, benign pigmented (natural or artificial) lesions: tattoos (of art, cosmetic, medical or traumatic), nevi of Ota, labial melanocytic macules, solar lentigos, poikiloderma of Civatte, melasma, melanocytic nevi, café-au-lait macules, Becker nevus, Peutz-Jegher's spot, post-inflammatory hyperpigmentation, etc.
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