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7.
Actas dermo-sifiliogr. (Ed. impr.) ; 113(5): 505-509, Mayo 2022. ilus, tab
Article in Spanish | IBECS | ID: ibc-206497

ABSTRACT

El síndrome Capillary vascular malformation of the lower lip, Lymphatic malformations of the head and neck, Asymmetry and Partial/generalized Overgrowth (CLAPO) es una entidad recientemente descrita, con muy pocos casos publicados en la literatura, y sin tratamiento estandarizado. El objetivo de nuestro estudio fue evaluar la eficacia y seguridad del láser de colorante pulsado (PDL) en los pacientes con este síndrome. Tratamos a siete pacientes con dos a cuatro sesiones de PDL a longitud de onda de 595 nm. La terapia láser fue realizada utilizando un tamaño de spot de 7 a 10 mm, una duración del pulso de 0,5 a 1,5 ms, y una fluencia de 6 a 8 J/cm2. Se realizaron fotografías clínicas antes del tratamiento y tres semanas tras el procedimiento. La supresión del eritema fue > 75% en cuatro pacientes. La púrpura transitoria estuvo presente en todos los pacientes durante alrededor de dos semanas, presentando un paciente con hipopigmentación postinflamatoria. A modo de conclusión consideramos que el PDL parece ser un tratamiento seguro y efectivo para las malformaciones capilares propias del síndrome CLAPO. Se logró una notable reducción del eritema en todos los pacientes, con baja incidencia de efectos secundarios (AU)


CLAPO syndrome (Capillary vascular malformation of the lower lip, Lymphatic malformations of the head and neck, Asymmetry and Partial/generalized Overgrowth) is a recently described entity, with very few published cases in the literature, and no standardized treatment. The objective of our study was to assess the efficacy and safety of PDL in patients with this syndrome. Seven patients were treated with two to four sessions of PDL at 595-nm wavelength. Laser therapy was performed using a spot size of 7 to 10 mm, a pulse duration of 0,5 to 1,5 ms and a fluence from 6 to 8 J/cm2. Clinical photographs were taken before treatment and 3 weeks after procedure. Clearance of the erythema was > 75% in 4 patients. Transient purpura was present in all patients for about 2 weeks and 1 patient presented post inflammatory hypopigmentation. In conclusion we consider that PDL seems to be a safe and effective treatment for capillary malformations of the CLAPO syndrome. A marked reduction in erythema was achieved in all patients with a low incidence of side effects (AU)


Subject(s)
Humans , Male , Female , Adolescent , Young Adult , Adult , Middle Aged , Lasers, Dye/therapeutic use , Hair Diseases/therapy , Vascular Malformations/therapy , Treatment Outcome , Syndrome
8.
Actas dermo-sifiliogr. (Ed. impr.) ; 113(5): t505-t509, Mayo 2022. ilus, tab
Article in English | IBECS | ID: ibc-206498

ABSTRACT

CLAPO syndrome (Capillary vascular malformation of the lower lip, Lymphatic malformations of the head and neck, Asymmetry and Partial/generalized Overgrowth) is a recently described entity, with very few published cases in the literature, and no standardized treatment. The objective of our study was to assess the efficacy and safety of PDL in patients with this syndrome. Seven patients were treated with two to four sessions of PDL at 595-nm wavelength. Laser therapy was performed using a spot size of 7 to 10 mm, a pulse duration of 0,5 to 1,5 ms and a fluence from 6 to 8 J/cm2. Clinical photographs were taken before treatment and 3 weeks after procedure. Clearance of the erythema was > 75% in 4 patients. Transient purpura was present in all patients for about 2 weeks and 1 patient presented post inflammatory hypopigmentation. In conclusion we consider that PDL seems to be a safe and effective treatment for capillary malformations of the CLAPO syndrome. A marked reduction in erythema was achieved in all patients with a low incidence of side effects (AU)


El síndrome Capillary vascular malformation of the lower lip, Lymphatic malformations of the head and neck, Asymmetry and Partial/generalized Overgrowth (CLAPO) es una entidad recientemente descrita, con muy pocos casos publicados en la literatura, y sin tratamiento estandarizado. El objetivo de nuestro estudio fue evaluar la eficacia y seguridad del láser de colorante pulsado (PDL) en los pacientes con este síndrome. Tratamos a siete pacientes con dos a cuatro sesiones de PDL a longitud de onda de 595 nm. La terapia láser fue realizada utilizando un tamaño de spot de 7 a 10 mm, una duración del pulso de 0,5 a 1,5 ms, y una fluencia de 6 a 8 J/cm2. Se realizaron fotografías clínicas antes del tratamiento y tres semanas tras el procedimiento. La supresión del eritema fue > 75% en cuatro pacientes. La púrpura transitoria estuvo presente en todos los pacientes durante alrededor de dos semanas, presentando un paciente con hipopigmentación postinflamatoria. A modo de conclusión consideramos que el PDL parece ser un tratamiento seguro y efectivo para las malformaciones capilares propias del síndrome CLAPO. Se logró una notable reducción del eritema en todos los pacientes, con baja incidencia de efectos secundarios (AU)


Subject(s)
Humans , Male , Female , Adolescent , Young Adult , Adult , Middle Aged , Lasers, Dye/therapeutic use , Hair Diseases/therapy , Vascular Malformations/therapy , Treatment Outcome , Syndrome
9.
Actas Dermosifiliogr ; 113(5): 505-509, 2022 May.
Article in English, Spanish | MEDLINE | ID: mdl-35292145

ABSTRACT

CLAPO syndrome (Capillary vascular malformation of the lower lip, Lymphatic malformations of the head and neck, Asymmetry and Partial/generalized Overgrowth) is a recently described entity, with very few published cases in the literature, and no standardized treatment. The objective of our study was to assess the efficacy and safety of PDL in patients with this syndrome. Seven patients were treated with two to four sessions of PDL at 595-nm wavelength. Laser therapy was performed using a spot size of 7 to 10 mm, a pulse duration of 0,5 to 1,5 ms and a fluence from 6 to 8 J/cm2. Clinical photographs were taken before treatment and 3 weeks after procedure. Clearance of the erythema was > 75% in 4 patients. Transient purpura was present in all patients for about 2 weeks and 1 patient presented post inflammatory hypopigmentation. In conclusion we consider that PDL seems to be a safe and effective treatment for capillary malformations of the CLAPO syndrome. A marked reduction in erythema was achieved in all patients with a low incidence of side effects.


Subject(s)
Lasers, Dye , Vascular Malformations , Arteriovenous Malformations , Capillaries/abnormalities , Erythema/etiology , Humans , Lasers, Dye/therapeutic use , Lymphatic Diseases , Treatment Outcome , Vascular Malformations/complications , Vascular Malformations/radiotherapy , Vascular Malformations/surgery
10.
J Eur Acad Dermatol Venereol ; 35(10): 2079-2084, 2021 Oct.
Article in English | MEDLINE | ID: mdl-33988879

ABSTRACT

BACKGROUND: Maculopapular cutaneous mastocytosis (MPCM) in children is classified in two variants: (i) monomorphic variant, presenting with the small macules or papules typically seen in adult patients; and (ii) polymorphic variant with larger lesions of variable size and shape, typically seen in children. The definition of polymorphic and monomorphic variants is mostly intuitive, and a validation of this classification has not been done. OBJECTIVE: To study interobserver variability in the classification of MPCM in two groups of observers: mastocytosis experts and general dermatologists. MATERIALS AND METHODS: Nineteen cases of childhood MPCM were shown blindly, for classification as monomorphic or polymorphic type, to 10 independent observers (eight dermatologists, one allergist and one haematologist) from Europe and North America with a vast experience in the management of paediatric mastocytosis. Also, the same cases were shown on a screen to 129 general dermatologists attending a meeting; their votes were registered by remote controls. The interobserver variability kappa coefficient (with 95% confidence interval) was calculated to measure the reliability of the correlation. RESULTS: The value of kappa interobserver variability coefficient for the group of 10 experts (95% confidence interval) was 0.39 (0.18-0.63), which is considered as 'fair'. The value of kappa interobserver variability coefficient for the group of 129 general dermatologists (95% confidence interval) was 0.17 (0.06-0.39), which is considered as 'slight'. A complete agreement of all 10 experts was achieved in only four of 19 cases (21.1%) The most voted choice was concordant between the two groups in only 11 of the 19 cases. CONCLUSIONS: We failed to validate the classification system of childhood MPCM in monomorphic and polymorphic types. While the rate of agreement was low for mastocytosis experts, it was nearly the agreement expected by chance in general dermatologists.


Subject(s)
Urticaria Pigmentosa , Adult , Child , Europe , Humans , Observer Variation , Reproducibility of Results
11.
J Ultrasound ; 24(4): 573-576, 2021 Dec.
Article in English | MEDLINE | ID: mdl-33247813

ABSTRACT

PURPOSE: Local therapy is the preferred option of treatment for most cutaneous leishmaniasis (CL); however, local therapy could be challenging, depth and size of the skin lesions are not always clinically evident and treatment response evaluation could occasionally be misleading. High frequency ultrasound is a non-invasive imaging tool which allows initial depth assessment ultrasound-guided infiltrations and ultrasound monitoring until resolution. METHODS: We present two cases of CL treated with ultrasound-guided infiltrations and ultrasound monitoring until resolution. RESULTS: Ultrasound imaging allowed a more accurate diagnosis of CL, defining more precisely the depth and size of the skin lesions. During follow-up, progressive decrease in dermal involvement, marked attenuation of the echogenicity of subcutaneous cellular tissue and a decrease in vascularization in the color Doppler mode was observed, which aided in evaluation of treatment response. Hypodermal inflammation observed through sonography was addressed with image-guided infiltration. CONCLUSION: We would like to highlight the usefulness of skin ultrasound (both B-mode and color Doppler mode) in the diagnosis, depth assessment, imaging guided treatment, and follow-up in CL.


Subject(s)
Leishmaniasis, Cutaneous , Follow-Up Studies , Humans , Inflammation , Leishmaniasis, Cutaneous/diagnostic imaging , Leishmaniasis, Cutaneous/drug therapy , Skin/diagnostic imaging , Ultrasonography
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