Atrofodermia lineal de Moulin / Linear atrophoderma of Moulin

Resumen Es una enfermedad poco frecuente, descripta en 1992 por Moulin, que se presenta entre los 6 y los 20 años de edad, caracterizada por bandas hiperpigmentadas atróficas de distribución blaschkoide, localizadas principalmente en tronco, unilaterales, que no son precedidas por inflamación o cambios esclerodérmicos, induración ni adherencias a planos profundos. En general es una afección autolimitada, cuyos tratamientos resultan ineficaces. Presentamos el caso de un niño de 12 años, con lesiones en tronco compatibles con el diagnóstico de Atrofodermia lineal de Moulin (ALM).

Abstract It is a disease not very frequent, described in 1992 by Moulin, which occurs between 6 and 20 years of age, characterized by hyperpigmented atrophic bands distribution blaschokoide, mainly located in trunk, unilateral, that are not preceded by inflammation or changes sclerodermal, induration, or adhesions to deep. It is a self-limited condition, whose treatments are ineffective. We present the case of a boy, 12 years old, with atrophic, hyperpigmented and asymptomatic plates, distributed from the left mammary region to the homolateral back, following the lines of Blaschko. Refers that is started as a hyperpigmented macula and in recent years it has been atrophying, it is not accompanied by any symptomatology, and was not preceded by inflammatory. We performed laboratory test with complete blood count, renal function, liver function and antibody titers, which were normal; and incisional biopsy by punch, that reported, for a sample stained with hematoxylin-eosin, epidermis of variable thickness, canned, no cellular atypia or disorders madurativos. Dermis impresses discreetly thickened, with homogenization of collagen. Slight perivascular inflammatory infiltrate. With clinical and anatomopathology we arrive at the diagnosis of Linear atrophoderma of Moulin.

Psoriasis linear y liquen estriado: una asociación rara entre dos dermatosis lineares / Linear psoriasis and lichen striatus: a rare association between two linear dermatoses

Resumen: La psoriasis linear y el liquen estriado son dos dermatosis de distribución linear por su localización sobre las líneas de Blaschko. Aunque estas dos enfermedades comparten algunas características, su asociación no es común y su diagnóstico en algunas ocasiones puede ser un reto. Presentamos el caso de una paciente pediátrica que desarrolló las dos patologías, con adecuada respuesta al tratamiento con corticoides tópicos.

Abstract: Linear Psoriasis and Lichen Striatus are known as linear dermatoses for their distribution along the Blaschko lines. Although they share some characteristics, their association is not common and accurate diagnosis can sometimes be a challenge. We present the case of a girl who develops two linear dermatoses, with adequate response to topical corticosteroids.

Patrón cutáneo pigmentario de disposición lineal en un niño, vitíligo segmentario vs. hipomelanosis de Ito / Linear disposition pigmentary skin pattern in a child, segmental vitiligo vs. Hypomelanosis of Ito

Introducción: algunas enfermedades dermatológicas siguen disposición con patrones lineales. Con hipopigmentación en la infancia se encuentran el vitíligo segmentario, que sigue los dermatomas, aunque puede seguir las líneas de Blaschko y la hipomelanosis de Ito, que a su vez sigue las líneas de Blaschko. Estas dermatosis son infrecuentes en la práctica dermatólogica. Objetivo: profundizar en los elementos diagnósticos que permiten diferenciar dos dermatosis clínicamente caracterizadas por hipopigmentación segmentaria lineal de tipo blaschkoide y el tratamiento. Presentación del caso: a la consulta de Genodermatosis en Las Tunas acude un niño con máculas acrómicas en hemicuerpo izquierdo, sin otras alteraciones. Después de ser evaluado por varias especialidades (Dermatología, Genética, Pediatría, Oftalmología y Neurología), se determina que solo presentaba afectación cutánea, se le realizó biopsia de piel, que corroboró el diagnóstico de vitíligo segmentario. Conclusiones: se presenta el caso porque el vitíligo segmentario es infrecuente, sigue un patrón lineal que puede ser diferenciado de otra dermatosis infrecuente, como la hipomelanosis de Ito, y en el tratamiento es importante brindar apoyo psicológico al paciente para favorecer la obtención de mejores resultados con la Melagenina Plus(AU)

Introduction: some dermatological diseases are still available with linear patterns. In childhood with hypopigmentation can be found segmental vitiligo (which follows the dermatomes although it can follow the lines of Blaschko), and Hypomelanosis of Ito (which in turn follows the lines of Blaschko). These dermatoses are infrequent in dermatological practice. Objective: to deepen into the diagnostic elements that allows the differentiation of two dermatoses clinically characterized by linear segmental hypopigmentation of blaschkoid type and treatment. Case presentation: a child attends to the consultation of Genodermatoses in Las Tunas presenting acromic macules in left half of the body, without other alterations. After being evaluated by several specialties (Dermatology, Genetics, Pediatrics, Ophthalmology and Neurology), it was determined that only skin affectation was present. A skin biopsy was performed, which corroborated the diagnosis of segmental vitiligo. Conclusions: The case is presented because segmental vitiligo is infrequent, it follows a linear pattern that can be differentiated from another uncommon dermatosis, such as Hypomelanosis of Ito, and in the treatment it is important to provide psychological support to the patient to favor obtaining better results with Melagenina Plus(AU)

Extragenital Lichen Sclerosus Et Atrophicus along the Blaschko's Lines of the Forehead / 대한피부과학회지

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis presenting as white papules and atrophic patches in the anogenital region. Extragenital LSA is rare and commonly affects the neck, thighs, and the trunk. A 68-year-old woman presented with a well-demarcated, whitish linear plaque on the forehead that was observed several months prior to presentation. A biopsy specimen showed hyperkeratosis and thinning of the epidermis and homogenization of collagen in the papillary dermis with a subepidermal cleft. Mild periappendageal lymphocytic infiltration and eccrine gland atrophy secondary to thickening of collagen bundles were observed in the deep dermis. Based on the clinical and histopathological findings, this patient was diagnosed with extragenital LSA and concomitant morphea along the Blaschko's lines.

Two Cases of Unilateral Linear Syringoma on Trunk Following Blaschko Lines / 대한피부과학회지

No abstract available.

Liquen plano blaschkoide facial como expresión de mosaicismo cutáneo, a propósito de un caso / Lichen planus blaschkoide facial as expression of cutaneous mosaicism, on a case by case

El liquen plano es un trastorno inflamatorio adquirido de etiología desconocida que, excepcionalmente, puede presentarse de forma lineal, debido a la predisposición genética de un clon que se produce durante el desarrollo embrionario. El liquen plano lineal o Blaschkoide de localización facial, es aún más infrecuente, y traduce una mutación genética postcigótica, que así como en otras patologías inflamatorias dermatológicas, aumenta la susceptibilidad de los individuos a desarrollarla.

Lichen planus is an acquired inflammatory disorder of unknown etiology that in exceptional cases can occur linearly. This is due to the genetic predisposition of a clone that occurs during embryonic development. Facial localization of the lichen planus is even more infrequent, and translates to a postcigotic genetic mutation. This mutation increases individual susceptibility, just as in other dermatological inflammatory pathologies.

Hipomelanosis de ito en paciente de 15 meses / Hypomelanosis of Ito in a patient 15 months

La Hipomelanosis de Ito (HI) es un trastorno neurocutáneo poco prevalente en Chile y el mundo, caracterizado por lesiones hipopigmentadas que siguen las líneas de Blaschko y que se asocian principalmente a alteraciones del sistema nervioso central y/o musculoesqueléticas. Se origina como expresión de un mosaicismo inespecífico de las células pigmentarias, durante la embriogénesis. Se presenta el caso de un paciente masculino de 15 meses con lesiones hipopigmentadas características, retraso del desarrollo psicomotor, crisis convulsivas tónico ­ clónicas, microcefalia, hipotonía central severa e hipoacusia bilateral, retraso en el desarrollo dental y dismorfias faciales. Se realizó estudio, resultando sin alteraciones metabólicas, excepto por aumento progresivo de TSH (11,3 mUI/L), por lo cual se inicia tratamiento con levotiroxina. Con los hallazgos clínicos y resultados de laboratorio descritos se planteó diagnóstico de mosaicismo pigmentario, continuando estudio de forma ambulatoria. En cuanto al diagnóstico, se recomienda la utilización de los criterios de Ruiz-Maldonado que consideran la presencia de lesiones cutáneas asociado a un criterio mayor o dos menores para determinar el diagnóstico definitivo (Ver Tabla 1). La patología más importante a descartar, es la Incontinencia Pigmentaria, que se caracteriza por estar ligado exclusivamente al cromosoma X y evolución por etapas de las lesiones cutáneas en las líneas de Blaschko. En la actualidad la HI solo tiene tratamiento sintomático por cual es importante hacer un diagnóstico precoz para sobrellevar la patología adecuadamente.

Hypomelanosis of Ito (HI) is a rarely prevalent neurocutaneous disorder in Chile and the world, that is characterized by hypopigmented lesions following Blasko lines that are primarily asociated with Central Nervous Sistem and or musculoskeletal disorders. It origins as an expression of an inespecific mosaicisism of the pigmented cells during embriogénesis. We present a case of a 15 months pediatric male patient with characteristic hypopigmented lesions, delayed psychomotor development, tonic-clonic seizures, microcephaly, central hypotonia and bilateral hypoacusia, delayed dental development and facial dysmorphia. He was hospitalized for further studies resulting without metabolic disorders except for progressive enhancement of TSH (11,3 mUI/L), and thyroxine supplement was initiated. With the described clinical and laboratory findings we proposed the diagnosis of Pigmentary Mosaicism and continued ambulatory treatment. Regarding the diagnosis, given the low prevalence of this disease we recommend the use of Ruiz ­ Maldonado criteria wich considers the precense of cutaneous lesions associated with one mayor or two minor criteria for the definitive diagnosis previously discarding the more frecuent diseases. Speaking about the differential diagnosis the most important disease is Pigmentary Incontinence, characterized by its exclusive presentation in female patients and the phasic evolution of the cutaneous lesions in Blasko lines. Nowadays the IH has only sintomatic treatment wich is why its important to make an early diagnosis in order to endure adequately the disease.

A Case of Lichen Planus Occurred on Lower Leg and Foot Following Blaschko's Lines / 대한피부과학회지

No abstract available.

A Case of Lupus Erythematosus Panniculitis of the Scalp Presenting as Doughnut-shaped Alopecia along Blaschko's lines / 대한피부과학회지

A 9-year-old girl presented with a 2-year history of doughnut-shaped hair loss on the frontal scalp, resembling the symmetric distribution of Blaschko's lines. Physical examination showed an alopecic patch with mild scalp induration. Histopathology revealed lymphoplasmacytic infiltration of the perifollicular dermis and subcutaneous lobules with abundant mucin deposition, consistent with a diagnosis of lupus erythematosus panniculitis. Three cases of linear lupus erythematosus panniculitis of the scalp presenting as alopecia along Blaschko's lines have been documented in Korean dermatologic literature. However, there have been no reports of doughnut-shaped alopecia until now. Herein, we present an interesting case of a pediatric patient with a unique feature of doughnut-shaped alopecia along Blaschko's lines.

Atrofodermia lineal de Moulin / Linear atrophoderma of Moulin

La atrofodermia lineal de Moulin es una patología poco frecuente que se presenta en niños sanos y adultos jóvenes. Se manifiesta con lesiones que siguen las líneas de Blaschko. Ocurre sin estar precedida de inflamación, induración, esclerodermia o atrofia epidérmica.Debemos hacer diagnóstico diferencial principalmente con atrofodermia idiopática de Pasini y Pierini y morfea. Tiene pronóstico favorable y no se la ha asociado a otras anomalías. Los tratamientos descritos han sido poco favorables. Presentamos el caso de unamujer de 15 años, con lesiones lineales en dorso y brazo, compatibles con atrofodermia lineal de Moulin.

The linear atrophoderma of Moulin is a rare disease that occurs in healthy children andyoung adults. It manifests with lesions along the lines of Blaschko. It occurs without precedinginflammation, induration, scleroderma or epidermal atrophy. Primarily, we shouldmake the differential diagnosis with idiopathic atrophoderma of Pasini and Pierini andmorphea. It has a favorable prognosis and it has not been associated with other anomalies.The treatments described have been unsuccessfull. We report a 15-year-old woman,with linear lesions on the back and arm compatible with linear atrophoderma of Moulin.

Lichen Planus Pigmentosus along the Blaschko's Lines / 대한피부과학회지

Lichen Planus Pigmentosus (LPP), an uncommon variant of lichen planus, is clinically characterized by insidious onset of diffuse or reticulated hyperpigmented patches on the sun-exposed areas and flexural folds. Histopathologic findings of LPP show atrophic epidermis with vacuolar degeneration of the basal cell layer and sparse lichenoid lymphohistiocytic infiltration with melanophages in the dermis. Although there have been few reports of LPP, clinical and histological pathogenesis and prognosis are not well known. Herein, we describe a case of linear LPP along the Blaschko's lines in 34-year old Korean man who presented with linear hyperpigmented, dark brown patches on his left thigh, repeatedly developing new lesions of erythematous reticular patches since puberty. This report shows the early events of LPP and the protracted course.

Angioma Serpiginoso / Angioma Serpiginosum

El angioma serpiginoso es una rara dermatosis adquirida de origen desconocido. El 90% de los casos ocurre en mujeres y se manifiesta en la infancia o en la adolescencia. Las lesiones consisten en máculas puntiformes, eritematosas o violáceas, con una disposición serpiginosa característica, que le da su nombre. Con mayor frecuencia compromete las extremidades, principalmente los miembros inferiores. Se presenta un paciente adulto con angioma serpiginoso en miembro superior, que se inició en la adolescencia con lesiones típicas, de distribución lineal, siguiendo las líneas de Blaschko.

Angioma serpiginosum is an uncommon acquired dermatosis of unknown origin. 90% of the cases occur in women and usually begin to manifest in childhood or adolescence. Lesions consist of punctuate erythematous or purple maculae, with a serpiginous disposition, feature that gives it its name. More often it compromises the extremities, mainly the lower ones. We report a case of angioma serpiginosum in an adult man with typical lesions, of adolescence-onset, on the upper limb, following Blaschko's lines.

A Case of Linear Lichen Planus Pigmentosus

Lichen planus pigmentosus (LPP) is chronic pigmentary disorder that shows diffuse or reticulated hyperpigmented, dark brown macules on the sun-exposed areas such as the face, neck and other flexural folds. Clinically, it is different from classical lichen planus because LPP has a longer clinical course and it manifests with dark brown macules. In case of LPP, involvement of the scalp, nail or mucosal area is rare. The histopathological findings of the lesions show an atrophic epidermis, the presence of melanophages and a vacuolar alteration of the basal cell layer with a sparse lymphohistiocytic lichenoid infiltration. Although there have been a few reports of LPP, there have only 3 cases of linear LPP along the lines of Blaschko in the Korean dermatologic literature. Our patient had lesions on the neck and chin with a linear pattern. In this report, we describe a very rare case of LPP with a linear distribution related to Blaschko's lines on the neck and chin areas.

A Case of Lichen Planus Pigmentosus Along the Lines of Blaschko / 대한피부과학회지

Lichen planus pigmentosus (LPP) is an uncommon variant of lichen planus that presents clinically as hyperpigmented, dark brown macules or patches in sun-exposed areas and flexural folds. LPP has been described previously as a pigmented form of actinic lichen planus or as erythema dyschromicum perstans. Recently, however, these diseases have been recognized as different clinical entities. Histopathologically, in atrophic epidermis, vacuolar alteration of the basal cell layer and scarce lymphohistiocytic lichenoid infiltration and pigment incontinence are seen. Herein, we report a case of LPP that developed in a unilateral linear pattern, probably in relation to Blaschko's lines.

Nevo epidérmico inflamatorio lineal (NEVIL): Presentación en la edad adulta / Inflammatory linear verrucous epidermal nevus (ILVEN): Late onset disease

El NEVIL es la variedad inflamatoria del nevo epidérmico; es un tumor benigno hamartomatoso, que se distribuye siguiendo las líneas de Blaschko. Estas líneas representarían mosaicismos cutáneos del desarrollo embriológico. El NEVIL suele aparecer en la infancia. Son generalmente unilaterales y en ocasiones muy pruriginosos. El diagnóstico diferencial es frecuentemente dificultoso. Presentamos una paciente en la cual la manifestación de esta patología se produce en forma tardía, siendo esto poco frecuente. El tratamiento es un desafío, se han probado múltiples modalidades terapéuticas, con resultados en ocasiones desalentadores. La cirugía, puede utilizarse en lesiones de pequeño tamaño. Los tratamientos con luz láser podrían ser de utilidad.

The ILVEN is the inflammatory variety of epidermal nevi, benign hamartomatous tumor that distributed following Blaschko lines. These lines represent cutaneous mosaicism in embryologic development. The ILVEN usually appear in the infancy. Generally unilateral and occasionally is very pruriginous. The differential diagnosis is often difficult. We present a patient in which the manifestation of her disease is produced in late onset, being this infrequent. Management is a challenge, multiple therapeutic modalities have been tested, with discouraging results. Surgery can be utilized in small size lesions. Laser therapy could be of utility.

A Case of Congenital Melanocytic Nevi Following Blaschko's Lines / 대한피부과학회지

Nevus spilus, also known as speckled lentiginous nevus, is a relatively common cutaneous lesion that is characterized by multiple pigmented macules within a pigmented patch. It may be congenital or acquired, and it can show the histological features of congenital melanocytic nevus (CMN). The possible relationship between nevus spilus and CMN has been contentious. We report a case of a congenital nevus that followed the line of Blaschko and clinically manifested as nevus spilus, but showed the histological features of CMN.

Congenital Café-au-Lait Spot and Sequentially Occurred Nevus Spilus

No abstract available.

A Case of Atypical Progressive Cribriform and Zosteriform Hyperpigmentation / 대한피부과학회지

We report a case of atypical progressive cribriform and zosteriform hyperpigmentation (PCZH) in a 4-year-old boy. Progressive cribriform and zosteriform hyperpigmentation is a distinctive pigmentary disorder first described by Rower et al. in 1978. It is characterized clinically by uniformly tan cribriform macular pigmentation in a zosteriform distribution and histopathologically by increased melanin in the basal cell layer and absence of nevus cells. This case has two distinctive findings; the onset age was below 10 years and the lesions distributed on the whole body along Blaschko's lines. Histopathologic examination showed pigmentary incontinence.