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Cureus Aug 2023Pyoderma gangrenosum (PG) is a challenging cutaneous manifestation associated with Dubowitz syndrome, a rare genetic disorder characterized by multiple congenital... (Review)
Review
Pyoderma gangrenosum (PG) is a challenging cutaneous manifestation associated with Dubowitz syndrome, a rare genetic disorder characterized by multiple congenital anomalies, developmental delay, and distinctive facial features. This review article aims to provide a comprehensive overview of the association between Dubowitz syndrome and pyoderma gangrenosum, emphasizing the clinical presentation, challenges in diagnosis and management, and potential underlying mechanisms. A comprehensive literature search was conducted to gather relevant studies, and inclusion and exclusion criteria were applied to select appropriate articles. The association between Dubowitz syndrome and pyoderma gangrenosum has been documented in reported cases and studies. Clinical characteristics of Pyoderma gangrenosum in Dubowitz syndrome include painful necrotic ulcers with undermined borders. Diagnosing pyoderma gangrenosum in the context of Dubowitz syndrome can be challenging due to the overlapping clinical features and complexities associated with the syndrome. Managing pyoderma gangrenosum involves a multidisciplinary approach, with general principles of wound care, systemic therapy, and pain management. Specific considerations for treating pyoderma gangrenosum in Dubowitz syndrome include collaboration among specialists and careful monitoring. Future directions for management include further research to understand the underlying mechanisms and develop targeted therapies. Recognizing and addressing pyoderma gangrenosum in Dubowitz syndrome is crucial for optimal patient care. This review enhances awareness among healthcare professionals and provides insights for improving diagnosis, management, and treatment outcomes for individuals with this challenging combination of conditions.
PubMed: 37706150
DOI: 10.7759/cureus.43408 -
Journal of Medical Genetics Feb 1986
Topics: Eczema; Face; Facial Bones; Female; Fetal Growth Retardation; Follow-Up Studies; Humans; Infant, Newborn; Male; Pregnancy; Syndrome
PubMed: 3950932
DOI: 10.1136/jmg.23.1.11 -
Ryoikibetsu Shokogun Shirizu 2000
Review
Topics: Abnormalities, Multiple; Craniofacial Abnormalities; Diagnosis, Differential; Fetal Growth Retardation; Genes, Dominant; Humans; Immune System Diseases; Infections; Intellectual Disability; Prognosis; Recurrence; Skin Diseases; Syndrome
PubMed: 11212722
DOI: No ID Found -
Ryoikibetsu Shokogun Shirizu 2001
Review
Topics: Craniofacial Abnormalities; Facies; Female; Humans; Infant; Skin Abnormalities; Syndrome
PubMed: 11462575
DOI: No ID Found -
Zeitschrift Fur Kinderheilkunde 1971
Topics: Abnormalities, Multiple; Body Height; Dwarfism; Eczema; Facial Bones; Facial Expression; Female; Humans; Intellectual Disability; Male; Microcephaly; Micrognathism; Pedigree
PubMed: 5088752
DOI: 10.1007/BF00440382 -
American Journal of Medical Genetics 1980An autosomal recessive disorder characterized by intrauterine growth retardation, postnatal retardation, microcephaly, sparse hair, toe syndactyly, and characteristic... (Review)
Review
An autosomal recessive disorder characterized by intrauterine growth retardation, postnatal retardation, microcephaly, sparse hair, toe syndactyly, and characteristic facial appearance is now recognized as the Dubowitz syndrome. Five addition additional cases of the Dubowitz syndrome are reported, including 2 with documented vascular abnormalities.
Topics: Face; Female; Follow-Up Studies; Growth Disorders; Humans; Infant; Infant, Newborn; Male; Microcephaly; Syndactyly; Syndrome; Vascular Diseases
PubMed: 6258433
DOI: 10.1002/ajmg.1320070209 -
European Journal of Anaesthesiology Aug 2019
PubMed: 31274551
DOI: 10.1097/EJA.0000000000000959 -
European Journal of Pediatrics Apr 1986The Dubowitz syndrome is a rare, autosomal, recessively inherited disorder of intrauterine and postnatal growth retardation leading to microcephaly, moderate mental...
The Dubowitz syndrome is a rare, autosomal, recessively inherited disorder of intrauterine and postnatal growth retardation leading to microcephaly, moderate mental retardation and such characteristic facial anomalies as telecanthus, epicanthic folds, blepharophimosis, ptosis, broadening of the bridge and tip of the nose, abnormal ears and retrogenia. Further findings include hyperactivity, eczema, cryptorchidism in the affected males, and brachy-clinodactyly of the fifth fingers. Thirty-three cases with this syndrome have been reported in the literature. Five additional patients are presented. All five are sporadic cases. The diagnostic symptoms and the differential diagnosis are discussed.
Topics: Abnormalities, Multiple; Child, Preschool; Face; Female; Fingers; Heart Defects, Congenital; Humans; Hyperkinesis; Infant; Intellectual Disability; Male; Microcephaly; Pregnancy; Syndrome
PubMed: 3709570
DOI: 10.1007/BF00496039 -
Klinische Padiatrie 1987A boy with the Dubowitz syndrome is presented. This autosomal recessive disorder is characterized by variable degrees of intrauterine and postnatal growth retardation,... (Review)
Review
A boy with the Dubowitz syndrome is presented. This autosomal recessive disorder is characterized by variable degrees of intrauterine and postnatal growth retardation, microcephaly, mild mental retardation, hyperactivity, "eczema", characteristic facial appearance and combination of minor abnormalities. Thirty-eight cases of this syndrome have been reported in the literature. Symptoms and difficulties in differential diagnosis are discussed.
Topics: Abnormalities, Multiple; Blepharoptosis; Dwarfism; Genes, Recessive; Humans; Infant; Intellectual Disability; Male; Syndrome
PubMed: 3316825
DOI: 10.1055/s-2008-1026822 -
Ryoikibetsu Shokogun Shirizu 2000
Review
Topics: Abnormalities, Multiple; Diagnosis, Differential; Fetal Growth Retardation; Growth Disorders; Hematologic Diseases; Humans; Immune System Diseases; Prognosis; Psychomotor Disorders; Syndrome
PubMed: 11057184
DOI: No ID Found