Idiopathic Atrophoderma of Pasini and Pierini Occurring in Two Brothers

Weiner, Marc Allen

doi:10.1001/archderm.1959.01560200063006

Cited by 12 publications

(4 citation statements)

References 1 publication

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The opinions whether APP is a separate entity are divided. Sev eral authors regard it as entity differing from morphea in clinical and histological features and the course of the dis ease [4][5][6], others reported both to occur in the same patients and believe them to be closely linked within the spectrum of the same disease [2,7,8]. This problem is of practical importance because of a different therapy and quite different prognosis.…”

Section: Introductionmentioning

confidence: 99%

Atrophoderma Pasini-Pierini Is a Primary Atrophic Abortive Morphea

Kencka¹,

Błaszczyk²,

Jabłońska³

1995

Dermatology

View full text Add to dashboard Cite

Background: There are divergent opinions whether atrophoderma Pasini-Pierini (APP) is a nosologic entity or a primary atrophic morphea. Objective: Since usually single cases are reported without a long-term follow-up the present study was performed in order to elucidate the natural history of the disorder. Methods: We followed a large series of 139 patients, 91 adults and 48 children, for 4–30 years (mean over 10 years). Results: APP was found to be 6 times more frequent in females and not uncommon in children (10% of our series of localized scleroderma). At some time during the follow-up period, indurations appeared in the central parts of the lesions in 17% of the patients, and in 22% they coexisted with morphea plaques outside the atrophies. The histological pattern was similar to morphea at the stage of atrophy. No case developed full-blown morphea. Conclusion: APP appears to be an abortive morphea, in which the indurations failed to develop. The differentiation from morphea is of practical importance because of different management and prognosis.

show abstract

Section: Introductionmentioning

confidence: 99%

Atrophoderma Pasini-Pierini Is a Primary Atrophic Abortive Morphea

Kencka¹,

Błaszczyk²,

Jabłońska³

1995

Dermatology

View full text Add to dashboard Cite

show abstract

“…Por otro lado, la teoría genética supone la existencia de elementos relacionados con la genética y que podrían permitir la trasmisión hereditaria de la dermatosis. Weiner y Gant reportan la aparición de AIPP en dos hermanos 10 . Kim Sung Kwon describe el caso de una niña de dos años de edad, cuya dermatosis estaba presente al nacimiento; sin embargo, no se describe un cuadro clínico similar en otro miembro de la familia 11 .…”

Section: Discussionunclassified

Atrofodermia Idiopática de Pasini y Pierini, un abordaje inusual.

Flores Hernández,

Moreno Ruíz,

Guevara Cerritos

et al. 2023

Alerta (San Salvador)

View full text Add to dashboard Cite

La atrofodermia idiopática de Pasini y Pierini es una entidad poco frecuente y de etiología aún no esclarecida, se presenta con una frecuencia hasta seis veces mayor en mujeres que en hombres y una posible asociación con la esclerodermia localizada (morfea). Presentación del caso. Paciente femenina de 30 años, quien consultó por una lesión asintomática de dos años de evolución en el glúteo izquierdo. En el examen físico se evidenció una placa ovalada, deprimida y acrómica en su centro, que mide cinco por diez centímetros. La paciente había sido tratada previamente con múltiples terapias tópicas sin obtener mejoría clínica. Intervención terapéutica. Se realizó la biopsia de piel que demostraba cambios mínimos en epidermis, homogenización y adelgazamiento de colágeno sin afección de anexos. Se hizo correlación con los hallazgos clínicos y se decidió iniciar tratamiento con esteroides intralesionales de alta potencia (acetónido de triamcinolona). Evolución clínica. Posterior a la administración de dos aplicaciones del medicamento, con cuatro semanas de diferencia entre ellas, se evidenció la resolución completa de la dermatosis. Un mes después de la última dosis la paciente no mostró recidivas.

show abstract

“… ABSTRACT Atrophoderma of Pasini and Pierini (APP) is an uncommon form of localized morphoea that occurs as superficial, hyperpigmented plaques distributed mainly on the trunk and proximal part of the limbs. There is little information about the influence of genetic and environmental factors on disease susceptibility and expression for localized scleroderma, although APP familial cases have been reported 1 . We report three siblings without a family history of autoimmune disease presenting cutaneous lesions suggesting morphoea (APP variant). …”

mentioning

confidence: 89%

“…There is little information about the influence of genetic and environmental factors on disease susceptibility and expression for localized scleroderma, although APP familial cases have been reported. 1 We report three siblings without a family history of autoimmune disease presenting cutaneous lesions suggesting morphoea (APP variant).…”

mentioning

confidence: 97%

Morphoea in three siblings

Iranzo¹,

López‐Lerma

Palou

et al. 2001

Acad Dermatol Venereol

View full text Add to dashboard Cite

Atrophoderma of Pasini and Pierini (APP) is an uncommon form of localized morphoea that occurs as superficial, hyperpigmented plaques distributed mainly on the trunk and proximal part of the limbs. There is little information about the influence of genetic and environmental factors on disease susceptibility and expression for localized scleroderma, although APP familial cases have been reported. We report three siblings without a family history of autoimmune disease presenting cutaneous lesions suggesting morphoea (APP variant).

show abstract

Idiopathic Atrophoderma of Pasini and Pierini Occurring in Two Brothers

Cited by 12 publications

References 1 publication

Atrophoderma Pasini-Pierini Is a Primary Atrophic Abortive Morphea

Atrophoderma Pasini-Pierini Is a Primary Atrophic Abortive Morphea

Atrofodermia Idiopática de Pasini y Pierini, un abordaje inusual.

Morphoea in three siblings

Contact Info

Product

Resources

About