Aplasia cutis congenita type VI, also known as Bart syndrome, is a rare genetic mechanobullous disorder characterized by congenital localized absence of skin, mucocutaneous blistering lesions, and nail abnormalities. We present the case of a 4-h-old male newborn who presented with complete absence of skin over the anteromedial aspect of both lower legs associated with nail dystrophy since birth. After a few days, he developed blisters that were consistent with epidermolysis bullosa in histopathological examination. There was no systemic involvement such as pyloric atresia, ureteral stenosis, renal abnormalities, or arthrogryposis. All laboratory work and imaging studies were normal. A diagnosis of Bart syndrome was made based on previous presentation. We managed the patient with conservative methods. Complete epithelialization occurred after several weeks.
Focal dermal hypoplasia (FDH) or Goltz syndrome is a rare X-linked dominant multisystemic disease involving the ectoderm, mesoderm, and endoderm. About 95% of the cases appear de novo, and 90% of them are females. Recently, the studies revealed that FDH is caused by a mutation in the PORCN gene. We report a case of unilateral FDH or Goltz syndrome in a 16-year-old girl presenting with hypopigmented-reticulated atrophic macules and patches in a linear pattern distributed along the lines of Blaschko over the right side of the face and the right arm. Also she is having hypoplasia of the right breast with dental enamel abnormality and partial anodontia in the lower jaw. Sparse hair and partial alopecia on the right side (scalp, eyebrows, and eyelashes) were also observed.
Purpura fulminans is a critical, mostly fatal, thrombotic syndrome that requires urgent intervention. Purpura fulminans is an acute purpuric rash characterized by coagulation of the microvasculature, which leads to purpuric lesions and skin necrosis. In this report, we present a rare case of an adult patient who was admitted with rapidly forming purpura fulminans in an uncommon location.
Darier disease is a rare type of autosomal dominant genodermatosis, and it is caused by a mutation in the gene coding for the endoplasmic reticulum membrane calcium pump Ca2+-ATPase type 2, leading to compromised intercellular adhesion. Moreover, this condition is characterized by multiple keratotic greasy papules with a seborrheic distribution and is worsened by heat and sun exposure, sweating, and friction. Occasionally, it may be associated with nail abnormalities and may involve the mucosa. Unilateral segmental Darier disease is a rare variant characterized by unilateral eruption of erythematic keratotic papules not associated with other conditions. Herein, we report a case of type 1 segmental Darier disease. Furthermore, the main characteristics and treatment options are discussed.
A cutaneous horn is a rare clinical condition characterised by a conical projection of hyperkeratotic epidermis. Cutaneous horns most commonly arise from sun-exposed skin in elderly men, but may arise from any part of the body at any age in men and women. When a cutaneous horn forms, it is important to determine the underlying cause. Various skin diseases may present with cutaneous horns including viral warts, actinic keratosis, keratoacanthoma, seborrhoeic keratosis, pyogenic granuloma, discoid lupus erythematosus, verruca vulgaris, Bowen's disease, basal cell carcinoma and squamous cell carcinoma. The underlying pathology is benign in 61.1% of cases, premalignant in 23.2% of cases and malignant in 15.7% of cases. We report a patient with a cutaneous horn arising from an area of discoid lupus erythematosus on the scalp.
Background
Knowing normal values of scalp hair thickness and density is beneficial for diagnosing hair disorders, monitoring responses to therapy, performing hair transplants, and conducting relevant research. Such normal values have not yet been studied for Arabs.
Objective
To assess the hair density and diameter of the Arab population at different scalp sites using phototrichogram.
Methods
A cross-sectional descriptive study was performed at King Saud University Medical City from December 2021 to August 2022. The study was conducted on Arab adults who were not suffering from any hair disorder. The study was conducted using a questionnaire and clinical examination by phototrichogram.
Results
A total of 120 participants were enrolled in this study. The mean hair density was 147.1± 7.8 hairs/cm2, and the mean hair diameter was 87±4.9 μm. There were significant differences between hair density (P=0.0001) and diameter (P=0.0001) in different regions of the scalp. Females had higher hair density than males at occiput (P=0.038).
Conclusion
Arab hair has a higher thickness and lower density than hair of other ethnicities. This needs to be considered when diagnosing and/or treating hair problems in Arabs.
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