Clinical analysis of 11 children with primary osteoma cutis / 中华皮肤科杂志

Objective To investigate clinical manifestations,pathological features and outcomes of primary osteoma cutis in children.Methods Eleven children with confirmed primary osteoma cutis diagnosed in Department of Dermatology,Beijing Children's Hospital between 2011 and 2018 were included into this study.The clinical manifestations,histopathological features,and outcomes were analyzed retrospectively.Results Among the 11 patients,7 were males and 4 were females.Primary osteoma cutis occurred within 22 months after birth in all the children,the median age of onset was 1 month,and the disease occurred during the first 6 months of life in 10 children.The skin lesions were characterized by skincolored or reddish indurated papules,plaques or nodules of varying size with slight epidermal atrophy.Three patients had local skin lesions,and 8 had multiple skin lesions.Serum calcium and parathyroid hormone levels were within normal limits in all the children,and no developmental deformity was observed at birth in any of the children.Histopathological examination revealed the formation of mature lamellar bone in the dermis in all the cases,which involved the subcutaneous adipose tissue in 5 cases.The skin lesions became stable 8-18 months after the occurrence in 10 patients,which was consistent with primary plaquelike osteoma cutis.Only 1 patient underwent a slowly progressive course,and the skin lesions involved subcutaneous deep tissues,leading to dyskinesia,which was consistent with progressive osseous heteroplasia.Conclusions Primary osteoma cutis in children mostly occurs in infancy,whose clinical manifestations are atrophic,indurated plaques or nodules,and its main pathological feature is the formation of mature lamellar bone.Long-term follow-up is needed,and attention should be paid to the occurrence of progressive osseous heteroplasia.

Osteoma cutis, a propósito de un caso / Osteoma cutis, about a case

El osteoma cutis (OC) es una lesión compuesta de tejido óseo en dermis y/o hipodermis. Se clasifican en primarios, cuando se desarrollan en piel sana, y secundarios, cuando aparecen sobre lesiones preexistentes. Mujer de 61 años con antecedente de linfoma de Hodgkin en remisión, presentó una lesión de larga data, desarrollada en piel sana, en primer dedo de la mano izquierda, que cursaba con brotes recurrentes y autolimitados de dolor y aumento de temperatura local. La exploración física mostró un nódulo pétreo, con úlcera de fondo calcáreo. El estudio histopatológico de la biopsia escisional reveló tejido óseo, sin otras lesiones cutáneas asociadas. El estudio complementario, incluyendo hemograma, función renal y hepática, calcio y fósforo séricos, PTH, TSH, vitamina D y calciuria fue normal. Descartándose alteraciones en el metabolismo calcio-fosforo y otras lesiones cutáneas preexistentes, se realizó el diagnostico de OC primario en forma de placa. El OC es una lesión benigna, pudiendo ser primario o secundario. Los secundarios representan cerca del 85% y se originan por la osificación de lesiones preexistentes tumorales o inflamatorias. Los OC primarios se presentan aislados o formando parte de síndromes como la fibrodisplasia osificante progresiva, la osteodistrofia hereditaria de Albright y la heteroplasia osificante progresiva. A pesar de la asociación a mutaciones en GNAS de OC y estos síndromes, el diagnóstico de ambos es fundamentalmente clínico. Los OC primarios sin síndrome asociado, se dividen en osteomas miliares de la cara y osteomas en forma de placa (plate-like OC), como este caso. Ante un OC primario, debemos descartar síndromes asociados a osificaciones heterotópicas.

Osteoma cutis (OC) is a lesion composed of bone tissue located in the dermis and/or hypodermis. It is classified as primary when it develops over healthy skin; and secondary when it develops over preexisting lesions. A 61-year-old woman with history of Hodgkin lymphoma in remission, presents a longstanding lesion located on the first finger of her left hand which developed over healthy skin, associated with recurrent and self-limited episodes of pain and increased local temperature. Physical examination reveals a stone-hard nodule, with a calcareous-bottomed ulcer. The histopathological evaluation of the excisional biopsy revealed bone tissue. Complementary studies included: complete blood count, renal and hepatic function, plasma calcium and phosphorus, PTH, TSH, vitamin D, and urinary calcium, all of them in normal ranges. Once systemic disease was ruled out, platelike OC was diagnosed. OC is a benign lesion which can be primary or secondary. Secondary OC represent nearly 85% of cases, and it corresponds to an ossification of preexisting tumoral or inflammatory lesions. Primary OC is less frequent, it appears isolated or as part of syndromes like fibrodysplasia ossificans progressiva, Albright hereditary osteodystrophy, and progressive osseous heteroplasia. GNAS mutations have been described in primary OC as in these syndromes, but the final diagnosis relies on the clinical picture. Isolated primary OC is classified in milliary osteomas of the face and plate-like OC, like our case. When facing primary OC, syndromes that present with heterotopic ossification must be ruled out.

A Case of Multiple Osteoma Cutis in Infant / 대한피부과학회지

No abstract available.

Miliary Osteoma Cutis of the Face in Middle-aged Women / 대한피부과학회지

Miliary osteoma cutis of the face is a variant of osteoma cutis, and this malady occurs in middle-aged to older women with or without a previous history of acne vulgaris. Case 1 is a 54-year-old woman who presented with asymptomatic, multiple, 2~3 mm-diameter, skin-colored, firm papules on both cheeks and she'd had these lesions for 10 years. Case 2 is a 61-year-old woman who showed the same type of skin lesions on both cheeks and temporal areas for 10 years. Histopathological examinations for the two cases revealed compact bone formation in the dermis. We report here on 2 rare cases of miliary osteoma cutis of the face.

Osteoma Cutis as the Presenting Feature of Albright Hereditary Osteodystrophy Associated with Pseudopseudohypoparathyroidism

Primary osteoma cutis is characterized by the formation of normal bone tissue in the dermis or subcutis without any underlying tissue abnormality or pre-existing calcification. This illness is associated with Albright hereditary osteodystrophy (AHO), which is characterized by such physical features as a short stature, round face, obesity, brachydactyly and osteoma cutis. Pseudohypoparathyroidism (PHP) is an inherited metabolic disorder that's characterized by resistance to parathyroid hormone, and PHP is present in most AHO patients. An AHO phenotype without hormonal resistance is called pseudopseudohypoparathyroidism (PPHP). Osteoma cutis is less common in patients with PPHP than in patients with PHP. We present here a case of osteoma cutis as the cardinal manifestation of AHO associated with PPHP. Osteoma cutis is an important sign of AHO and its significance should not be overlooked, even if the patient has normal values on the serum biochemical tests.

Osteoma Cutis as the Presenting Feature of Albright Hereditary Osteodystrophy Associated with Pseudopseudohypoparathyroidism

Primary osteoma cutis is characterized by the formation of normal bone tissue in the dermis or subcutis without any underlying tissue abnormality or pre-existing calcification. This illness is associated with Albright hereditary osteodystrophy (AHO), which is characterized by such physical features as a short stature, round face, obesity, brachydactyly and osteoma cutis. Pseudohypoparathyroidism (PHP) is an inherited metabolic disorder that's characterized by resistance to parathyroid hormone, and PHP is present in most AHO patients. An AHO phenotype without hormonal resistance is called pseudopseudohypoparathyroidism (PPHP). Osteoma cutis is less common in patients with PPHP than in patients with PHP. We present here a case of osteoma cutis as the cardinal manifestation of AHO associated with PPHP. Osteoma cutis is an important sign of AHO and its significance should not be overlooked, even if the patient has normal values on the serum biochemical tests.

A Case of Osteoma Cutis, a Diagnostic Clue for Albright's Hereditary Osteodystrophy / 대한피부과학회지

Albright's hereditary osteodystrophy (AHO) has a broad spectrum of physical findings, including short stature, flattened nasal bridge, round facies, obesity, skeletal anomalies of the hands with brachydactyly, osteoma cutis, mental retardation, dental defects, cataracts, and calcification in the basal ganglia. It also includes endocrinologic abnormalities such as pseudohypoparathyroidism and pseudopseudohypoparathyroidism. Primary osteoma cutis, which can precede other physical findings of AHO, might be a diagnostic clue for AHO. Therefore, close follow-up and regular laboratory tests should be done to detect the early development of AHO in young children with primary osteoma cutis. Early diagnosis and treatment of AHO can prevent irreversible mental retardation and developmental delay.

Osteoma cutis: informe de un caso / Osteoma cutis: a case report

El propósito de esta comunicación es dar a conocer la experiencia con un caso de osteoma cutis en una paciente de 46 años de edad, quien solicitó tratamiento odontológico en las clínicas de la Escuela de Odontología de la Universidad del Valle. El examen radiografico periapical mostró peque±as zonas radiopacas de aproximadamente 2 mm de dißmetro a nivel de molares superiores derechos e izquierdos. El caso se estudió y se diagnosticó en la Sección de Radiología de la Escuela. Como era un hallazgo radiografico poco frecuente, se motivó el interés por una revisión detallada del tema y con otras ayudas diagnósticas fue posible establecer el diagnóstico definitivo.

The purpose of this comunication is to share the experience with a case of osteoma cutis in a 46 years old woman who requested odontological treatment at the dental clinic of the Universidad del Valle. The periapical radiographic image of the superior molar zone showed multiple radiopaque bilateral rounded bodies of approximately 2 mm in diameter. The case was further studied and analyzed at the radiological section of the school. It was characterized as a non-frequent radiological finding. This unusual finding motivated a detailed review and the use of other diagnostic tools, such as clinical and histopathological tests that established the final diagnosis.

A Case of Perforating Osteoma Cutis / 대한피부과학회지

Among skin disorders associated with changes to the bony tissue or abnormalities of calcification, osteoma cutis is a rare, benign tumor of the skin composed of compact bone. Osteoma cutis is primary ossification without preceding skin disease, trauma or evidence of Albright's hereditary osteodystrophy. A 59-year-old man presented with a firm erythematous nodule containing centrally punctuated ulcer on the right mandibular angle. A biopsy specimen showed calcification and ossification with many osteocytes, which had extruded through the perforating epidermal channel. We herein report a rare case of perforating osteoma cutis.

A Case of Miliary Osteoma Cutis on the Jaw / 대한피부과학회지

Miliary osteoma cutis of the face is a form of primary osteoma cutis. We report a case of miliary osteoma cutis on the jaw of a 55-year-old female. She presented with several 2~3 mm sized, skin-colored firm papules over both lower jaws for 5 years. Skin biopsy was performed and the specimen showed numerous osteocytes and osteoblasts in the bony tissue which was located in the deep dermis.

A Case of Miliary Osteoma Cutis on the Face / 대한피부과학회지

Osteoma cutis is a rare disorder characterized by compact bone formation in the dermis or subcutaneous tissue. According to the absence or presence of previous cutaneous lesions, it is classified as primary and secondary forms. Miliary osteoma of the face is a form of primary osteoma cutis that generally occurs in middle-aged to older adult women. A 50-year-old woman presented with asymptomatic, multiple, 2~3 mm-diameter, skin-colored, firm papules on both cheeks for 5 years. The histopathologic specimen of the papule showed compact bone formation in the dermis. We herein report a rare case of military osteoma cutis on the face.

Osteoma cutis in Albright's Hereditary Osteodystrophy / 대한피부과학회지

Albright's hereditary osteodystrophy is an inherited syndrome that encompasses endocrinologic anomaly of pseudohypoparathyroidim or less commonly, pseudo-pseudohypoparathyroidism and various physical stigmata such as mental retardation, short stature, skeletal anomaly of the hands, abnormal dentition, round facies, and osteoma cutis. Primary osteoma cutis in this syndrome presents at birth or in early infancy, preceding most of the other manifestations. This case is a typical presentation with osteoma cutis as the sole initial manifestation. Rather unfamiliar to dermatologists, Albright's hereditary osteodystrophy still deserves to be included in the differentials when an isolated case of osteoma cutis presents in a young child.

A Case of Osteoma Cutis / 대한피부과학회지

Osteoma cutis is an infrequent primary ossification arising in the dermis or subcutaneous fat tissue without preceding trauma, skin disease or evidence of Albright's hereditary osteodystrophy. We report a case of osteoma cutis in a 12-year-old female, who had had a 1x1cm sized, asymptomatic, round, skin-coloured, hard nodule on her left forearm for 7 years. The history and physical examinations failed to reveal any sign of secondary cutaneous ossification, due to inflammation, scars and dysembryoplastia. Histologically, the lesion showed bony structures with osteocytes and osteoblasts in the subcutaneous fat tissue.

Case of Perforating Osteoma Cutis

A 24-year-old man presented with a 1.5 × 0.5 cm-sized erythematous nodule with central crust on the forehead since 5 years ago. There was no history of trauma or previous skin disorders. Histopathologic examination showed a typical picture of osteoma cutis. In addition, transepidermal elimination of bony material was observed: red linear plate-like calcified lamella structures had extruded to the skin surface through the perforated epidermis. The perforating type of osteoma cutis was discussed.

A Case of Multiple Miliary Osteomas of the Face / 대한피부과학회지

Osteoma cutis is a rare disorder characterized by compact bone formation in the dermis and subcutaneous tissue. It is classified in primary and secondary forms according to the presence or absence of previous cutaneous lesions. Multiple miliary osteomas of the face is a form of primary miliary osteoma cutis that generally occurs in middle-aged and older women. We report a typical case of multiple miliary osteomas of the face in a 63-year-old woman.

A Case of Multiple Osteoma Cutis in Infant / 대한피부과학회지

Osteoma cutis was first described by Wilkins in 1858 and it is a primary cutaneous ossification, which has no preceding trauma or skin disease and no evidence of Albright's hereditary osteodysttophy in the patient or his family. The lesions appear as hard, round to irregular, sharply defined tumors of varying size within the dermis or subcutis, and their color range from red to purple or brown. We report a rare case of multiple osteoma cutis in an infant. A 10-month-old female infant presented with multiple hard purplish nodules on the abdomen for several months and did not show any evidence of Albright's hereditary osteodystrophy and signs of secondary cutaneous ossification such as trauma, injection, and previous skin lesions. The histopathologic finding shows intradermal bony spicules with numerous osteocytes, cement lines, osteoblasts, and osteoclasts.

A Case of Multiple Osteoma Cutis of the Face / 대한피부과학회지

We report a case of multiple osteoma cutis of the face in a 51-year-old female. She presented with 1-2mm sized skin colored multiple firm papules involving both cheeks for 2 years. She did not have any family history. Topical isotretinoin was prescribed for 6 months without any effects. Skin biopsy was done. Biopsy specimen of a skin colored papule on the right cheek showed numerous osteocytes and osteoblasts in the bony tissue. Other papules are treated by punch biopsy.

A Case of Congenital platelike Osteoma Cutis / 대한피부과학회지

Osteoma cutis is a rare, benign, frequently congenital tumor of the skin composed of bone tissue, showing neither invasive nor metastasizing tendency. The term osteoma cutis is applied to cases of primary cutaneous ossification in which there is no evidence of Albrights hereditary osteodystrophy. Congenital platelike osteoma cutis is a clinically different variant of primary cutaneous osteoma. We report the case of a 10-month-old male infant who presented with two walnut-sized firm, poorly demarcated plaques containing pinhead-sized erythematous papules on the abdomen and the right leg and multiple variably sized erythematous subcutaneous nodules on the left leg sinee birth. The initial lesions did not enlarge nor did new lesions develop. A biopsy specimen of the abdominal lesion showed bony plate and cartilaginous structures in the subcutaneous fat tissue. There was no history of any skin disease prior to the development of osteoma.

A case of osteoma cutis / 대한피부과학회지

Osteoma cutis is a primary cutaneous ossification, which has no preceding trauma or skin disease and no evidence of Albrights hereditary osteodystrophy n the patient or his family. The lesion appears as hard, round to irregular, sharply defined tumor of varying size within the skin or subcutis, and color ranges from flesh-colored to purple or brown. We report herein a case of osteoma cutis in a 32-year-old female, who had a 1 x 1cm sized, asymptomatic, round, flesh colored, hard nodule on the right side of her forehead for 5 years. Histopathologic examination showed mature bone with many ostocytes, osteoblasts, cement lines and Haversian canals in the dermis.

A Case of Multiple Osteoma Cutis in Infant / 대한피부과학회지

The term "osteoma cutis" is limited only to primary cutaneous ossification in which there is no evidence of Albrights hereditary osteodystrophy in either the patient or his farnily. We herein present a case of osteorna cutis in a 2-year-old male. He had had multiple hard plaques on the extremities since birth and had not had the evidences of Albrights hereditary osteodystrophy and signs of secondary cutaneous ossification such as trauma, injection, previous skin lesions and abnormal laboratory findings. The histopathologic findings revealed bony spicules with numerous osteocytes, cement lines, Haversian canals, osteoblasts and osteoclasts.