ABSTRACT
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.
Subject(s)
Humans , Female , Middle Aged , Osteoma/pathology , Bone Neoplasms/pathology , Ossification, Heterotopic/pathology , Osteoma/diagnostic imaging , Physical Examination , Biopsy , Clinical Laboratory TechniquesABSTRACT
Cutaneous bone formation may be primary or secondary. If it is primary, there are no preceding cutaneous lesions. If it is secondary, bone forms through metaplasia within a preexisting lesion caused by inflammation, traumatic injury, and a neoplastic tumor. Paronychia is inflammation of the proximal nail fold and presents as painful periungual erythema, sometimes with associated purulence. Chronic paronychia is most commonly related to mechanical or chemical factors. Here, we report a case of chronic paronychia accompanied by cutaneous ossification in a 33-year-old woman who presented with a 2-year history of recurrent paronychia on the left first finger.
Subject(s)
Adult , Female , Humans , Erythema , Fingers , Inflammation , Metaplasia , Osteogenesis , ParonychiaABSTRACT
A 52-year-old man presented with a brown to black nodule on his left cheek. Histological examination revealed nests of nevus cells throughout the upper and mid-dermis. An eosinophilic oval-shaped bony structure was found within the nevus. We report here on a case of nevus of Nanta combined with metastatic renal cell carcinoma.
Subject(s)
Humans , Middle Aged , Carcinoma, Renal Cell , Cheek , Eosinophils , NevusABSTRACT
A 36 year-old man and his 6 month-old daughter presented with multiple, slightly depressed, skin-colored, hard nodules and plaques on the abdomen, back, and both extremities. They also showed abnormal physical appearance, including short stature and neck, round face, and short digits, which suggested Albright's hereditary osteodystrophy (AHO). Histopathologically, various sized bony spicules were present in the dermis. The laboratory results showed normal serum calcium, phosphorus and parathyroid hormone levels. On the basis of clinical, laboratory and histologic findings, we diagnosed these cases as Albright's hereditary osteodystrophy with cutaneous ossification occurring in pseudopseudohypoparathyroidism.
Subject(s)
Abdomen , Calcium , Dermis , Extremities , Fibrous Dysplasia, Polyostotic , Neck , Nuclear Family , Parathyroid Hormone , Phosphorus , PseudopseudohypoparathyroidismABSTRACT
A 36 year-old man and his 6 month-old daughter presented with multiple, slightly depressed, skin-colored, hard nodules and plaques on the abdomen, back, and both extremities. They also showed abnormal physical appearance, including short stature and neck, round face, and short digits, which suggested Albright's hereditary osteodystrophy (AHO). Histopathologically, various sized bony spicules were present in the dermis. The laboratory results showed normal serum calcium, phosphorus and parathyroid hormone levels. On the basis of clinical, laboratory and histologic findings, we diagnosed these cases as Albright's hereditary osteodystrophy with cutaneous ossification occurring in pseudopseudohypoparathyroidism.
Subject(s)
Abdomen , Calcium , Dermis , Extremities , Fibrous Dysplasia, Polyostotic , Neck , Nuclear Family , Parathyroid Hormone , Phosphorus , PseudopseudohypoparathyroidismABSTRACT
Nevus cell nevus in bone has been called osteo-nevus of Nanta since Nanta reported it in detail in 1911. We report a 46-year-old female patient with osteo-nevus of Nanta on her forehead. Histologic examination revealed nests of nevus cells, with numerous osteocytes and osteoblasts in the bony tissue. Inflammatory cell infiltrate and foreign body granuloma were not found.
Subject(s)
Female , Humans , Middle Aged , Forehead , Granuloma, Foreign-Body , Nevus , Osteoblasts , OsteocytesABSTRACT
In Albright's hereditary osteodystrophy (AHO) including the syndromes of pseudohypoparathyroidism (PHP) and pseudopseudohypoparathyroidism (PPHP), multiple areas of intracutaneous ossification are often encountered. The characteristic features are short stature, round face, short neck, obesity, cutaneous ossifications, and various skeletal anomalies including short metacarpal and metatarsal bones, curve of radius, and brachydactyly. The patient was a 10-month-old male infant. He presented slightly depressed erythematous hard plaques on the left upper chest and left thigh. We had taken biopsies from both skin lesions, confirming cutaneous ossification or bone formation. He also had the characteristic features of AHO. He had a history of admission due to patent ductus arteriosus and atrial septal defect. The laboratory results showed slightly decreased calcium, increased phosphorus and PTH levels. The patient received no specific corrective measures because his calcium and phosphorus levels were not far from normal values until newly developed similar skin lesions appeared.
Subject(s)
Humans , Infant , Male , Biopsy , Brachydactyly , Calcium , Ductus Arteriosus, Patent , Heart Septal Defects, Atrial , Metatarsal Bones , Neck , Obesity , Osteogenesis , Phosphorus , Pseudohypoparathyroidism , Pseudopseudohypoparathyroidism , Radius , Reference Values , Skin , Thigh , ThoraxABSTRACT
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.