CLAPO syndrome is a rare vascular disorder described by López-Gutiérrez and Lapunzina in 2008, characterized by lower lip capillary malformation, lymphatic malformation, asymmetry of the face and limbs and partial/generalized overgrowth.1 Preliminary studies have demonstrated mosaic activating mutations in PIK3CA which may contribute to its variable clinical expressions.2 However, despite its heterogeneous phenotypic spectrum, capillary malformation of the lower lip seems to be a constant feature1,3 and is almost pathognomonic for this syndrome. This is usually a midline, symmetric, erythematous patch, present at birth and that may progressively expand beyond the vermilion (Fig. 1). In addition, capillary malformations might also affect other locations of the face including the forehead, lower cheek, mandible and chin.

The authors report a series of seven cases, wishing to discuss the efficacy and safety of 595-nm PDL for the treatment of CLAPO's capillary malformation.

Figure 1.

Capillary malformation of the lip. a. Patient 2. b. Note the irregular “sawtooth” border on dermoscopy.

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Seven patients with a clinical diagnosis of CLAPO syndrome were enrolled in this study at the Dermatology Department of the Hospital Universitario Ramon y Cajal, Madrid, Spain. The group of patients was composed of 6 women and 1 man, ranging in age from 18 to 58 years old. All had a symmetric, sharply defined erythematous-violaceous patch of the lower lip, consistent with a capillary malformation. Additional clinical findings are summarized in Table 1. Patients were given detailed information prior to the first treatment, including the risks, benefits, potential complications and alternative treatments. Written informed consent was obtained to participate.

Patients were treated with 595-nm PDL irradiation (Cynergy, Cynosure, Massachusetts, USA) using a spot size of 7–10mm, a pulse duration from 0.5 to 1.5ms and fluence from 6 to 8J/cm2. Continuous airflow cooling was set at level 3 (Cryo5, Zimmer Medizinsysteme GmbH, Neu-Ulm, Germany) and the lesions were covered by a thin layer of ultrasound gel for epidermal protection. Intense purpura was intentionally produced in all cases; this occasionally required double passing, but pulse stacking was avoided. Treatments were repeated approximately every 2 months, up to four sessions.

Clinical improvement was assessed by comparing digital photographs of each patient taken at baseline and 3 weeks after the end of therapy.

Results are shown in Table 1. All lesions responded to treatment, and a clearance of erythema superior to 75% was obtained in four patients. Treatment was well tolerated with no need for anesthesia or analgesia. Side effects included transient purpura for about 2 weeks, which was present in all patients and post-inflammatory hypopigmentation in one case (Fig. 2).

Figure 2.

Images before treatment (left side) and 3 weeks after the last treatment (right side). a, b. Refers to patient 4. c, d. Refers to patient 6.

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Capillary malformations are the most common type of vascular malformations. However, capillary malformation of the lower lip is an infrequent finding and is almost invariably related to the spectrum of CLAPO syndrome. This sign is always present at birth and may appear years to decades before the remaining features. Therefore, physicians must be aware of the possibility of this syndrome and maintain follow-up during infancy and adolescence.4 In comparison with the anterior midline lesions of the upper lip, that are typical of macrocephaly capillary malformation syndrome,5,6 capillary malformations of the lower lip do not tend to regress and may slowly expand beyond the vermilion. Change in color can also be expected, observing a transformation from red to more violaceus tones.

Pulsed dye laser has been widely used in vascular lesions, such as haemangiomas,7 port wine stains,8 telangiectasias9 and other conditions that present with facial erythema. To the best of our knowledge, there is only one case reported of CLAPO syndrome treated with PDL. However, parameters were not defined, and no substantial improvement was observed.4

From our experience, an intense purpura is necessary to obtain a good clinical response. This can be achieved either by using high fluences (6–8J/cm2) or by several passes separated by a minute. Nevertheless, a minimum of 2 sessions is required for a significant reduction (>50%) of the erythematous component.

Severe inflammatory reaction, swelling, crusting and rarely ulceration can be seen after treatment with PDL, especially when it involves the mucosa. However, the administration of oral or intramuscular glucocorticoids is not usually needed.

This report suggests that PDL is an effective option for the treatment of CLAPO's capillary malformations, since a marked reduction in erythema was achieved in all patients. Likewise, a good tolerance to treatment and a low incidence of serious side effects make it a very safe therapy and a first-line option in controlling the disease.

This work has not received any funding.

The authors declare that they have no conflict of interest.