ABSTRACT
The typical rash of Still disease is an asymptomatic, salmon-coloured, macular, or maculopapular eruption that appears along with the fever spikes and fades when the body temperature drops. Although not included in the diagnostic criteria, there are other frequent, persistent, pruritic and polymorphic skin manifestations of Still disease that have distinctive clinical features and specific histological findings. Among these atypical persistent pruritic eruptions (PPEs), periorbital erythema and linear flagellate papules and plaques can resemble the cutaneous manifestations of dermatomyositis (DM). The presence of these lesions in adult-onset Still disease has been associated with worse prognosis and higher mortality rates, perhaps due to the development of a secondary macrophage activation syndrome (MAS). We report two cases of Still disease with persistent atypical DM-like eruption, one in a paediatric patient (a very underreported finding) and the other in an adult. Both cases developed a secondary MAS.
Subject(s)
Arthritis, Juvenile , Dermatomyositis , Exanthema , Macrophage Activation Syndrome , Still's Disease, Adult-Onset , Humans , Adult , Child , Macrophage Activation Syndrome/etiology , Macrophage Activation Syndrome/complications , Dermatomyositis/complications , Dermatomyositis/diagnosis , Dermatomyositis/pathology , Exanthema/pathology , Still's Disease, Adult-Onset/complications , Still's Disease, Adult-Onset/diagnosis , Still's Disease, Adult-Onset/pathology , Arthritis, Juvenile/complications , Pruritus/pathologyABSTRACT
No disponible
Subject(s)
Humans , Female , Infant, Newborn , Ichthyosis, Lamellar/pathology , Abnormalities, Multiple/pathology , Skin/pathology , Infant, Newborn, Diseases , Skin/ultrastructure , Skin Abnormalities/pathologyABSTRACT
Lipedematous scalp is an uncommon entity of unknown etiology, rarely described in the pediatric age. It is characterized by boggy thickening of the scalp predominantly located at the vertex and occiput, which acquires a cotton-like consistency. This condition is palpable rather than visible. It is a casual finding because it is usually asymptomatic, although it may involve alopecia, pruritus, or dysesthesia. We report a 10-year-old girl with lipedematous scalp without alopecia. Sonographic and MRI findings confirmed the diagnosis of lipidematous scalp. El lipedema de cuero cabelludo o cuero cabelludo lipedematoso es una entidad infrecuente y de etiología desconocida, rara vez descrita en la edad pediátrica. Se caracteriza por un engrosamiento difuso y de tacto esponjoso del tejido celular subcutáneo localizado principalmente en vértex y occipucio. Suele ser un hallazgo casual dado que habitualmente cursa de forma asintomática, aunque puede asociar alopecia, prurito o disestesias. Presentamos el caso de una niña de 10 años de edad con lipedema de cuero cabelludo sin alopecia asociada. Los hallazgos ecográficos y de resonancia magnética confirmaron el diagnóstico de lipedema de cuero cabelludo.
Subject(s)
Lipedema/pathology , Scalp Dermatoses/pathology , Child , Female , Humans , Lipedema/diagnostic imaging , Magnetic Resonance Imaging , Scalp/pathology , Scalp Dermatoses/diagnostic imaging , UltrasonographySubject(s)
Mucinoses , Pediatrics , Administration, Cutaneous , Child , Humans , Infant , Mucinoses/diagnosis , SkinSubject(s)
Abnormalities, Multiple/genetics , Darier Disease/genetics , Eyebrows/abnormalities , LEOPARD Syndrome/genetics , Mutation , Child , Genotype , Humans , MaleABSTRACT
La morfea profunda solitaria es una forma infrecuente de morfea localizada en la infancia. Se caracteriza clínicamente por la presencia de un área indurada, mal delimitada y asintomática, de localización paraespinal, que no tiende a la progresión ni se asocia con manifestaciones sistémicas. Histológicamente, muestra un engrasamiento e hialinización de las fibras de colágeno dérmico, junto con infiltrados de predominio linfohistiocitario en la dermis reticular y la hipodermis. Se presenta a una paciente de 7 años con una lesión solitaria en la región dorsal media izquierda, cuyos hallazgos clínicos, ecográficos e histológicos fueron compatibles con el diagnóstico de una morfea profunda solitaria.
Solitary morphea profunda is an unusual form of localized scleroderma in childhood. It is characterized by a single, poorly defined and indurate plaque often located on the upper trunk near the spine. Solitary morphea profunda is frequently asymptomatic and shows no associated systemic involvement. Histological examination reveals dense sclerosis of collagen and a marked lymphocytic infiltrate in the reticular dermis and subcutis. We report a 7-year-old girl with a solitary asymptomatic and sclerotic plaque on the back. Clinical, ultrasonographic and histological features were consistent with solitary morphea profunda.
Subject(s)
Humans , Female , Child , Pediatrics , Scleroderma, Localized , Ultrasonography , CollagenABSTRACT
Solitary morphea profunda is an unusual form of localized scleroderma in childhood. It is characterized by a single, poorly defined and indurate plaque often located on the upper trunk near the spine. Solitary morphea profunda is frequently asymptomatic and shows no associated systemic involvement. Histological examination reveals dense sclerosis of collagen and a marked lymphocytic infiltrate in the reticular dermis and subcutis. We report a 7-year-old girl with a solitary asymptomatic and sclerotic plaque on the back. Clinical, ultrasonographic and histological features were consistent with solitary morphea profunda.
La morfea profunda solitaria es una forma infrecuente de morfea localizada en la infancia. Se caracteriza clínicamente por la presencia de un área indurada, mal delimitada y asintomática, de localización paraespinal, que no tiende a la progresión ni se asocia con manifestaciones sistémicas. Histológicamente, muestra un engrasamiento e hialinización de las fibras de colágeno dérmico, junto con infiltrados de predominio linfohistiocitario en la dermis reticular y la hipodermis. Se presenta a una paciente de 7 años con una lesión solitaria en la región dorsal media izquierda, cuyos hallazgos clínicos, ecográficos e histológicos fueron compatibles con el diagnóstico de una morfea profunda solitaria.
Subject(s)
Scleroderma, Localized/diagnosis , Ultrasonography , Child , Female , Humans , Scleroderma, Localized/pathologySubject(s)
Lymphangioma/pathology , Sarcoma, Kaposi/pathology , Skin Neoplasms/pathology , Aged , Diagnosis, Differential , Herpesvirus 8, Human/immunology , Humans , Male , Sarcoma, Kaposi/blood supply , Sarcoma, Kaposi/diagnostic imaging , Skin Neoplasms/blood supply , Skin Neoplasms/diagnostic imaging , Ultrasonography, Doppler, Color/methodsABSTRACT
Subcutaneous granuloma annulare is a benign inflammatory disorder that primarily affects healthy children and manifests as a rapidly growing, painless, nonmobile mass, most commonly located in the leg. The current case highlights the sonographic appearance of subcutaneous granuloma annulare. Ultrasound color Doppler is a helpful noninvasive imaging test that may allow physicians to confirm the diagnosis of subcutaneous granuloma annulare and avoid unnecessary medical procedures.
Subject(s)
Granuloma Annulare/diagnostic imaging , Subcutaneous Tissue/pathology , Ultrasonography, Doppler, Color/methods , Child, Preschool , Diagnosis, Differential , Female , HumansABSTRACT
La aplasia cutis congénita es una rara alteración caracterizada por la ausencia de áreas localizadas de piel en el momento del nacimiento. Suele manifestarse como una lesión solitaria localizada principalmente en el cuero cabelludo, que puede estar asociada con otras malformaciones congénitas. Las complicaciones pueden ser fatales, por lo que es necesario un tratamiento individualizado que vendrá determinado por el tamaño, localización y grado de afectación de estructuras subyacentes. Presentamos un caso de aplasia cutis congénita del cuero cabelludo con múltiples lesiones y defecto óseo subyacente de 3 × 1.5 cm de tamaño, pero sin otras anomalías asociadas. El manejo conservador permitió una adecuada y completa epitelización cutánea con cierre del defecto óseo subyacente sin necesidad de procedimientos invasivos.
Subject(s)
Bone Diseases, Developmental/diagnosis , Bone Diseases, Developmental/therapy , Ectodermal Dysplasia/diagnosis , Ectodermal Dysplasia/therapy , Conservative Treatment , Female , Humans , Infant, Newborn , Musculoskeletal Abnormalities/diagnosis , Musculoskeletal Abnormalities/therapy , Skull/abnormalitiesSubject(s)
Scurvy/diet therapy , Scurvy/diagnosis , Ascorbic Acid Deficiency/complications , Ascorbic Acid Deficiency/diet therapy , Female , Fruit , Humans , Male , Middle Aged , VegetablesABSTRACT
No disponible
Subject(s)
Humans , Female , Child , Chickenpox/diagnosis , Photosensitivity Disorders/diagnosis , Diagnosis, Differential , Herpesvirus 3, Human/pathogenicityABSTRACT
Pilomatricoma is a benign cutaneous tumor originatingfrom hair matrix cells. Anetodermic changes inthe skin overlying pilomatricomas are sometimesreported, although their precise mechanisms remainunknown. We present an unusual case of anetodermicpilomatricoma on the upper extremity of a 17-yearoldboy and report its clinical, histopathologic, andsonographic findings.
Subject(s)
Anetoderma/diagnosis , Calcinosis/diagnosis , Hair Diseases/diagnosis , Pilomatrixoma/diagnosis , Skin Neoplasms/diagnosis , Adolescent , Anetoderma/diagnostic imaging , Anetoderma/pathology , Anetoderma/surgery , Arm , Calcinosis/diagnostic imaging , Calcinosis/pathology , Calcinosis/surgery , Hair Diseases/diagnostic imaging , Hair Diseases/pathology , Hair Diseases/surgery , Humans , Male , Pilomatrixoma/diagnostic imaging , Pilomatrixoma/pathology , Pilomatrixoma/surgery , Skin Neoplasms/diagnostic imaging , Skin Neoplasms/pathology , Skin Neoplasms/surgery , UltrasonographyABSTRACT
No disponible
