Journal of Cutaneous Laser Therapy 2000; 2: 151-156
O r i g in a l R e s e a r c h 151 - 156; Received 22 August 2000 Accepted 8 November 2000
Keywords: erythema - keratosis pilaris atrophicans - pulsed dye laser - roughness
Authors: SM Clark MD, MbChB, MRCP (UK) Department of Dermatology, University Hospital of Wales, Cardiff, Wales, UK CM Mills MD, MbChB, MRCP (UK) Department of Dermatology, The Royal Gwent Hospital, Newport, Wales, UK SW Lanigan MD, MbChB, MRCP (UK) Department of Dermatology, The Princess of Wales Hospital, Bridgend, Wales, UK
Correspondence: Dr SM Clark, Department of Dermatology, Leeds General Infirmary, Great George Street, Leeds, LS1 3EX, UK. Tel: (+44) 0113 3925724; Fax: (+44) 0113 3923565
BACKGROUND: Few therapies are currently available to treat keratosis pilaris atrophicans (KPA), a spectrum of disorders which includes ulerythema ophryogenes and atrophoderma vermiculata.
OBJECTIVE: To evaluate the response of KPA to treatment with the pulsed dye laser (PDL) with regard to improvements in erythema and skin roughness, treatment tolerability, and side effects.
METHODS: Treatment of all facial areas involved with KPA with the PDL at 585 nm was evaluated in 12 patients. Prior to and after each treatment skin erythema was estimated using an erythema meter, and skin roughness was analysed using micrometer evaluation of a skin surface biopsy taken from the same mapped area and anaysed by computer to calculate the roughness profile.
RESULTS: Patients received 2 to 8 treatments with the PDL with energies ranging from 6.0 to 7.5 J/cm2. Clinical improvement was noted in all patients with significant reduction in erythema scores. Pre-treatment scores ranged from 4 to 13 (mean 8.3) and post- treatment 0 to 8 (mean 3.1) (P< 0.05). Improvements in skin roughness were clinically apparent in all but two patients, but these were not significant on evaluation of skin surface biopsies. Treatment was generally well tolerated, and side effects other than local pain during treatment were very few.
CONCLUSION: PDL treatment appears to be a safe and effective treatment for the erythema
associated with KPA but does not give significant improvement in associated skin roughness. J Cutan Laser Ther 2000; 2: 151-156
Keratosis pilaris atrophicans (KPA) is one of the terms used to describe a group of conditions characterized by keratosis pilaris (pinhead follicular plugs with erythema due to vasodilation) succeeded by atrophy as the plugs are
shed.1 The degree of erythema may be marked, persistent, and psychologically quite upsetting for the patient involved.
The condition most commonly occurs on the cheeks, pre-auricular area, eyebrows and occasionally forehead, chin and scalp. Involvement of the eyebrows is termed ulerythema ophryogenes (UO) and is often seen from birth or infancy. Involvement of the cheek usually begins later between the ages of 5 and 12 years. Atrophoderma vermiculata (AV) is the term used when principally the cheeks are extensively involved. Involvement of the scalp does not tend to occur until the teens.
Many cases of the disorder occur sporadically and are presumed to be the results of an inborn defect, however autosomal dominant, and X-linked recessive inheritance has been reported. Though not usually associated with
physical or mental abnormality, KPA has been reported in association with wooly hair and Noonan's syndromes,2-4 monosomy
18p,5 and with other congenital abnormalities including developmental delay, central nervous and dental abnormalities, and undescended
testes.6
Treatments used for KPA have included topical keratolytics, vitamin D3 analogues,7 and topical and oral retinoids.1,8 None has been particularly effective.1,6,7 The pulsed tunable dye laser (PDL) has been used successfully to treat the erythematous element of a number of skin disorders, particularly port wine stains (PWS),9-11 facial telangiectasias12,13 (including rosacea 14), and adenoma sebaceum.15 It has also been used experimentally in the treatment of more inflammatory disorders including psoriasis,16,17 and cutaneous lupus erythematosus.18 Treatment is usually well tolerated with a low risk of side effects.11 The PDL appears to have the greatest margin of safety of currently available laser treatments in the treatment of cutaneous vascular lesions, especially for children.10 To our knowledge, the PDL has not been used to treat KPA, possibly because of the unknown effect on the inflammatory component of the disorder.
The aim of this study was to prospectively evaluate the use of the PDL in this condition, not only examining the reduction in erythema, but also to assess whether there was any reduction in skin roughness produced by the follicular keratoses.
After obtaining informed consent 12 patients were enrolled in the study. The age and sex of the patients was documented, and in addition the site and duration of KPA.
In 9 of the 12 patients a 2 X 2 cm2 area of involved skin (usually the cheek) was assessed prior to treatment, and at each subsequent follow up for the degree of erythema and skin roughness. A template was made to ensure that readings were taken from the same site on each occasion.
Skin erythema was estimated using an erythema meter.19 Three readings were taken from the specified area, and a mean value calculated. To take into account fluctuation in vasodilation with environmental changes, a mean control reading taken from an uninvolved area of the temple was subtracted from this value to give an overall score, i.e. Erythema score = mean erythema reading of the involved area - mean erythema reading of the control area.
Skin roughness was analysed using micrometer evaluation of a skin surface biopsy taken from the same mapped area (Figure 1).20 A smooth paste of adhesive was applied to the mapped area. A microscope slide was placed over the paste and left to set over about 30 seconds. The slide and adherent rubberized adhesive were then removed, resulting in a skin surface imprint. These were analysed by computer micrometer to calculate the roughness profile which estimated the average skin roughness (Ra), and the average roughness depth (Rz).
Ra was estimated by calculation of the arithmetic mean of all deviations of the roughness profile R from the main line within the total measuring length. Rz was estimated by the calculation of the mean of the individual roughness depths of five successive individual sampling lengths in the roughness profile.
Overall clinical improvement and all side effects of treatment were documented in all patients.
Involved areas of facial skin were treated with the pulsed dye laser (SPTL-1b; Candela Corp, Wayland, MA, USA) at 585 nm with a 450-ms pulse duration using 5 mm or 7 mm spot diameters, using contiguous non- or minimally overlapping spots. A local anaesthetic cream (EMLA®) was applied to the treated area 1-2 hours prior to laser therapy. A 2 X 2 cm test area was initially treated with the 5 mm spot-size at energy fluences of 6.0 J/cm2, 6.25 J/cm2 and 6.5 J/cm2 to assess the most appropriate starting dose for future treatments, and to judge treatment tolerability. Post-treatment ice packs were applied to the treated area.
Review and re-treatment of the involved areas was carried out at 2 monthly intervals. When transferring from a 5 to a 7 mm spot diameter, the energy fluence was lowered by 0.5 to 1.0 J/cm2.
The results were analysed statistically using the Wilcoxon Signed Rank test.
Figure 1: A fivefold magnified view of a skin surface biopsy.
Table 1: Age at onset of keratosis pilaris atrophicans in the study group.
Over the last 3 years, 12 Caucasian patients have been treated: 6 males and 6 females, age range 5 to 23 (mean 11) years. All patients had involvement of the cheeks, 7 (58.3%) the eyebrows, 5 (41.7%) the forehead, and 2 (16.7%) the chin. All patients had typical keratosis pilaris of the limbs.
Onset of KPA skin changes occurred from birth to 16 (mean 5.3) years (Table
1), and the duration of skin changes ranged from 2 to 23 (mean 10) years. A quarter of those treated had a clear family history of facial KPA, and a third a family history of keratosis pilaris of the limbs.
Patients received 2 to 8 (mean 4) treatments with the PDL. Treatment energies ranged from 6.0 to 7.5 J/cm2.
Pre-treatment erythema scores showed no significant correlation with either the age of the patient or the duration of KPA. After treatment with the PDL clinical improvement was noted in all patients, with a significant reduction in erythema scores (Figures 2A,B and 3A,B). Erythema scores pre-treatment ranged from 4 to 13 (mean 8.3) and post-treatment 0 to 8 (mean 3.1) (P < 0.05) (Figure 4). In some cases reduction in erythema only occurred with energies of 7.0 J/cm2 or above.
All but two of the patients reported that they felt that there had been a clinical reduction in roughness in the treated area. However, analysis of the skin surface biopsies did not show significant change. Mean Ra pre-treatment ranged from 5.93 to 19.53 (mean 10.49), and post-treatment from 7.53 to 10.77 (mean 9.38) (P > 0.05). This may be due to the fact that initially samples were taken following the removal of local anaesthetic cream prior to treatment.
Figure 2: Clinical improvements in erythema seen (A) pre and (B) post PDL treatment.
(A)
(B)
Figure 3: Clinical improvements in erythema seen (A) pre and (B) post PDL treatment.
(A) 
(B)
Figure 4: Changes in erythema scores pre and post PDL therapy
Five of the patients reported moderate or severe pain during and immediately following laser treatment. This was usually associated with an inflammatory erythematous flare around the treated area that lasted for up to several hours. One patient, the youngest at 5-years-old, was withdrawn from further treatment after two visits due to pain
intolerance during the treatment period. One other patient, a 23-year-old woman with extensive involvement, treated with the 7mm spot size at an energy fluence of 7.5J/cm2, developed such severe swelling and crusting in the 24-hour period following laser therapy that a 5-day course of oral flucloxacillin and topical fucidic acid was administered as a precaution against secondary infection. A dose of oral prednisolone 30 mg was administered immediately before treatment and 24 hours after therapy for the remaining two treatment sessions. This resulted in a considerable reduction in the degree of swelling, crusting and local discomfort, but still produced a good clinical response.
The treatment was well tolerated by the remaining 7 patients, who have reported no significant local pain after the immediate treatment period. One patient was lost to follow up after three treatments.
No other adverse reactions, in particular any type of scarring or pigmentary disturbance, have occurred in any of the patients.
Our results suggest that treatment with the PDL should be considered as a treatment option in patients with KPA. The erythematous element improves significantly after treatment. Though there may appear to be a clinical reduction in skin roughness, this has not been shown to be significant. Energy fluences of 7.0 J/cm2 or higher may be required before clinical improvement is seen.
As with PDL treatment of other vascular disorders the risk of side effects is low. However, almost half of our patients experienced moderate to marked local pain during treatment, despite prior use of local anaesthetic cream. PDL treatment may not be tolerated in patients under the age of 7 years without a general anaesthetic. We have found that in one patient with a severe inflammatory response following treatment, the use of one or two doses of oral prednisolone helped to reduce the degree of swelling, crusting and local discomfort following therapy. This has not as far as we are aware been reported before. The addition of oral steroids did not appear to reduce the effectiveness of the PDL treatment.
If the reaction to PDL therapy is severe, the risk of secondary infection is likely to increase. It has previously been suggested that secondary colonization or infection of sites treated with the PDL may occur in up to one quarter of
patients.21 Previous guidelines on post-treatment management of patients treated with the carbon dioxide laser advise anti-herpes simplex medication and a week of oral
antibiotics.22 We used prophylactic topical and oral antibiotic therapy in the patient who developed severe inflammation and crusting following PDL treatment, and would suggest that if the reaction with the PDL is severe, in addition to considering lower energy level for future treatments, it may be sensible to adopt the same guidelines in the treatment of more inflammatory conditions to prevent secondary complications.
Studies by Katugampola et al16 and also by Ros et al,17 evaluating the effectiveness of PDL in patients with stable plaque psoriasis, demonstrated that following treatment there was not only a reduction in the cutaneous vasculature but also epidermal thinning without any signs of psoriasis, suggesting that the microvasculature may play a role in the pathophysiology of the inflammatory condition. Early treatment in KPA may therefore not only have the effect of reducing local symptoms, but the reduced vascular supply may result in a reduction in local skin inflammation. This in turn may reduce the future risk of atrophy and scarring.
Though KPA is a relatively uncommon condition, for the child involved the prominent facial erythema, and if the eyebrows or scalp areas are involved the risk of permanent hair loss, can have a detrimental psychological effect. Previous studies have demonstrated a significant improvement in psychological well-being as a result of the improvements seen with PDL
therapy.23 Improvement in the cosmetic appearance of KPA affecting the face is likely to reduce the child’s embarrassment and reduce potential problems with respect to interaction with peers at a vulnerable stage in development. Long-term follow up of these patients is required to assess whether an early reduction in erythema in KPA will prevent or reduce the longer-term risk of atrophy and hair loss, and whether recurrence occurs with time.
We are grateful to nursing staff of the Laser Unit, The Princess of Wales Hospital, Bridgend, for their assistance in the laser treatment of the patients throughout this study.
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