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Ossificação Cutânea Heterotopica em Criança
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Authors
Advisor(s)
Abstract(s)
Reporta-se o caso de um doente do sexo masculino observado aos 12 meses de idade por lesões eritemato azuladas deprimidas com 2 a 4 mm de diâmetro no tronco e membros com início aos 3 meses e carácter aditivo. Tinha
também, na face posterior da perna direita, placa eritematosa de contornos irregulares, na qual se verificou
posteriormente uma textura granulosa.
Tratava-se de criança prematura, com história de múltiplas complicações neonatais. A biópsia cutânea revelou
ossificação da derme reticular e a Tomografia Computorizada da perna direita imagens lineares de densidade elevada
compatíveis com ossificação cutânea.
A Ossificação Cutânea é um fenómeno raro sendo na maioria dos casos secundário. A Ossificação Primária é ainda
mais rara e as entidades principais em que ela se observa são a Heteroplasia Óssea Progressiva (HOP), a Osteodistrofia
Hereditária de Albrigth (OHA) e o Osteoma Cutis em placa (OCP). A HOP caracteriza-se por ossificação com início na
derme e em idades precoces e com extensão progressiva para a profundidade ocasionando morbilidade significativa. A
OHA associa ossificação da derme e hipoderme não progressiva a alterações fenotípicas (baixa estatura, obesidade,
braquidactilia) e hormonais (pseudohipoparatiroidismo). O OCP caracteriza-se pela presença de uma ou mais placas
de ossificação da derme e hipoderme não progressivas e sem morbilidade significativa.
O diagnóstico definitivo deste caso torna-se difícil, neste momento, uma vez que a HOP pode ter evolução lenta ao
longo de anos mimetizando em fases iniciais um OCP; apenas o follow-up as poderá diferenciar em definitivo conforme
se verifique progressão ou não da ossificação. A hipótese de HOP parece, contudo, mais provável e a confirmar-se,
tanto quanto é do conhecimento dos autores, tratar-se-á do primeiro caso reportado em Portugal.
The authors report a case of a 12 month-old male infant that presented with numerous bluish/erythematous depressed lesions, measuring between 2 and 4 mm, scattered on the trunk and limbs that were first noted when he was 3- month-old and kept appearing over time. He also had a larger erythematous plaque on the posterior aspect of the right leg that later acquired a gritty texture. This was a premature child with a history of numerous neonatal complications. Skin biopsy showed ossification of the reticular dermis and right leg CT scan showed high density linear images compatible with cutaneous ossification. Cutaneous Ossification is most often a secondary phenomenon. Primary ossification of the skin is exceedingly rare and the main clinical entities where it occurs include Progressive Osseous Heteroplasia (POH), Albrigth Hereditary Osteodistrophy (AHO) and Plaquelike Osteoma Cutis (POC). POH usually manifests itself in the first months of life and presents with ossification starting in the dermis and extending progressively to involve deeper structures with significant morbidity. AHO presents with non-progressive cutaneous ossification, phenotypic features (short stature, obesity, brachydactily) and pseudohypoparathyroidism. In POC there are usually one or more plaques of cutaneous ossification with no significant morbidity. At this moment, definitive diagnosis in this case is difficult as POH can have a slow progression mimicking POC initially; only follow-up will allow the differential diagnosis depending on whether there is progression of the cutaneous Fig. 1 - Lesões ovalares, deprimidas e atróficas da face anterior do tronco. Dr.ª Bárbara Pereira Hospital dos Marmeleiros Serviço de Dermatologia Estrada dos Marmeleiros 9054-535 Funchal Tel.: 291705730 Email: barbararosa@hotmail.com Correspondência: ossification or not. POH seems, however, more likely and being confirmed, as far as the authors are aware, this will be the first reported case in Portugal.
The authors report a case of a 12 month-old male infant that presented with numerous bluish/erythematous depressed lesions, measuring between 2 and 4 mm, scattered on the trunk and limbs that were first noted when he was 3- month-old and kept appearing over time. He also had a larger erythematous plaque on the posterior aspect of the right leg that later acquired a gritty texture. This was a premature child with a history of numerous neonatal complications. Skin biopsy showed ossification of the reticular dermis and right leg CT scan showed high density linear images compatible with cutaneous ossification. Cutaneous Ossification is most often a secondary phenomenon. Primary ossification of the skin is exceedingly rare and the main clinical entities where it occurs include Progressive Osseous Heteroplasia (POH), Albrigth Hereditary Osteodistrophy (AHO) and Plaquelike Osteoma Cutis (POC). POH usually manifests itself in the first months of life and presents with ossification starting in the dermis and extending progressively to involve deeper structures with significant morbidity. AHO presents with non-progressive cutaneous ossification, phenotypic features (short stature, obesity, brachydactily) and pseudohypoparathyroidism. In POC there are usually one or more plaques of cutaneous ossification with no significant morbidity. At this moment, definitive diagnosis in this case is difficult as POH can have a slow progression mimicking POC initially; only follow-up will allow the differential diagnosis depending on whether there is progression of the cutaneous Fig. 1 - Lesões ovalares, deprimidas e atróficas da face anterior do tronco. Dr.ª Bárbara Pereira Hospital dos Marmeleiros Serviço de Dermatologia Estrada dos Marmeleiros 9054-535 Funchal Tel.: 291705730 Email: barbararosa@hotmail.com Correspondência: ossification or not. POH seems, however, more likely and being confirmed, as far as the authors are aware, this will be the first reported case in Portugal.
Description
Keywords
HCC DER HCC ANPAT Ossificação Osteoma
Citation
Trab Soc Port Dermatol Venereol 2006;64(4):517-525
Publisher
Sociedade Portuguesa de Dermatologia e Venereologia