Liquen plano linealblashkoide, reporte de 2 casos / Linear lichen planus along the lines of Blaschko, report of 2 cases

RESUMEN El liquen plano es una dermatosis inflamatoria autolimitada, de etiología desconocida, relativamente común. Existen múltiples variedades clínicas,que se basan en la disposición, morfología y localización de las lesiones, siendo el liquen plano lineal a lo largo de las líneas de Blaschko una presentación de distribución rara. Se presentan dos casos en pacientes de sexo masculino de 22 y 21 años de edad, con confirmación histológica y buena respuesta clínica al tratamiento tópico.

SUMMARY Lichen planus is a self-limited inflammatory dermatosis, relatively common, with a unknown cause. There are multiple clinical varieties, based on the disposition, morphology and location of the lesions, being the linear lichen planar along the lines of Blaschko a rare distribution presentation. We report two cases in male patients of 22 and 21 years of age, with histological confirmation and a good clinical response to topical treatment.

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)

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

No abstract available.

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.

Progressive Cribriform and Zosteriform Hyperpigmentation: A Case Series and Review of the Literature / 대한피부과학회지

Progressive cribriform and zosteriform hyperpigmentation (PCZH) is a distinctive pigmentary disorder observed along the lines of Blaschko. Clinically, the lesions appear as uniformly tan, cribriform macular hyperpigmentation with a zosteriform distribution, without a history of rash, injury, inflammation, or other associated cutaneous or internal abnormalities. Histopathological specimens show increased melanin pigmentation in the basal cell layer with a complete absence of nevus cells. We report 8 cases of PCZH and review the literature on this peculiar disorder.

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.

A Case of Linear Grover Disease Distributed along the Blaschko Line / 대한피부과학회지

Grover disease (also known as transient or persistent acantholytic dermatosis) is a pruritic polymorphic papulovesicular eruption that is histologically characterized by the presence of epidermal acantholysis. It primarily occurs in middle-aged individuals and manifests as scattered erythematous or brown papules as well as papulovesicles on the sun-exposed skin of the trunk. A 52-year-old man had erythematous papules and patches linearly arranged on the left thigh and leg with mild pruritus. The skin lesions were successfully treated with a topical corticosteroid. However, 2 months later, the lesions recurred. The histological examination of a punch biopsy revealed focal acantholytic clefts with dyskeratotic cells, hyperkeratosis, and the infiltration of perivascular lymphocytes and eosinophils. Taken together with the late onset and lack of family history, we diagnosed this condition as Grover disease distributed along the Blaschko line, a condition presented here for the first time.

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

No abstract available.

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 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.

Linear Lichen Nitidus Following the Blaschko's Line / 대한피부과학회지

No abstract available.

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.

Enfermedades raras: hipomelanosis de Ito / Rare diseases: hypomelanosis Ito

La hipomelanosis de Ito es un síndrome neurocutáneo que se manifiesta por la despigmentación de grado variabley distribución característica siguiendo las líneas de Blaschko, asociada a alteraciones neuroesqueléticas, oculares,cardíacas, renales y genitourinarias. Las máculas hipocrómicas están presentes desde el nacimiento o pueden aparecer en los primeros meses de vida...

Ito hypomelanosis is a neurocutaneous syndrome that includes skin depigmentation in variable degree, andcharacteristic distribution along Blaschko lines, in association with neurological, skeletal, ocular, cardiac, renal and genitourinary disorders. The hypochromic macules may be present from birth or appear in early months of life...

Two Cases of Unilateral Lichen Planus Following the Lines of Blaschko

A 50-year-old man and 71-year-old woman presented to our clinic with unilateral, linear, erythematous, pruritic lesions along the lines of Blaschko. On the basis of clinical and histopathological findings, the lesions were diagnosed as lichen planus with a Blaschkoian distribution, which is a rare form of lichen planus. The patients were treated with topical corticosteroids and antihistamines.

Linear Macular Amyloidosis Along the Lines of Blaschko / 대한피부과학회지

The primary localized cutaneous amyloidosis (PLCA) is classified into three types: macular amyloidosis, lichen amyloidosis, and nodular amyloidosis. Macular amyloidosis is characterized by pruritic, hyperpigmented macules and is most commonly located on the interscapular area. Skin lesion usually shows pigmentation with a reticulated or rippled pattern. We report an unusual case of linear macular amyloidosis along the lines of Blaschko. A 74-year-old male is presented with asymptomatic unilateral linear hyperpigmented macules on his right leg for 20 years. Skin biopsy has revealed eosinophilic cytokeratin-positive globular deposits occupying the dermal papillae.

Lupus Profundus with Segmental Distribution on Leg, Following the Lines of Blaschko in a Child / 대한피부과학회지

Lupus profundus is a rare subtype of chronic cutaneous lupus erythematosus, which shows a tender subcutaneous nodule or plaque. The face, arm, buttock, trunk and thighs are frequently involved. Lupus profundus can be associated with or without systemic lupus erythematosus. But due to its rarity, it is difficult to diagnosis and there are not many reports regarding its characteristics, including its distribution. In this case, a 13-year-old boy has visited with several violaceous non-tender nodules on Lt. upper thigh with segmental distribution, which follows the Blaschko's line. Through skin biopsy and its immunoflourescent study, we diagnosed lupus profundus. We here report a case of lupus profundus with segmental distribution following the lines of Blaschko's in a child.

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.

Systematized Inflammatory Linear Verrucous Epidermal Nevus / 대한피부과학회지

Inflammatory linear verrucous epidermal nevi (ILVEN) were first described in the literature, in 1971, by Altman and Mehregan. Most cases were reported as solitary lesions. In contrast, systematized ILVEN, involving wide areas of the integument, has only rarely been reported. A variety of treatment modalities has been reported, ranging from topical medications, such as potent corticosteroids or tretinoin 0.1% to variable procedures, including CO2 and pulsed dye laser, cryotherapy, and surgical excision. However, multifocal skin lesion is more difficult to treat. An 8-year old boy was presented with an extensive thick scaly plaques and patches, affecting the trunk and the four extremities sparing face. It developed when he was 3 years old, and he complained pruritus. On physical examination, linear, or whirl-like scaly plaques were seen, along with Blaschko lines. Pathologic finding was in accordance to ILVEN. We treated him with topical calcipotriol, tacrolimus and systemic acitretin. After 8 months, the lesion and symptoms improved.