What is Naegeli syndrome?
Naegeli-Franceschetti-Jadassohn (NFJ) syndrome is a rare autosomal dominant form of ectodermal dysplasia that affects the skin, sweat glands, nails, and teeth. The incidence is estimated to be 1 case in 2-4 million population.
What causes Naegeli syndrome?
Naegeli syndrome is caused by mutations in the KRT14 gene and inherited in an autosomal dominant manner. While there is no cure for Naegeli syndrome, treatment is based on each individual’s symptoms.
What is Dermatopathia pigmentosa reticularis?
Dermatopathia pigmentosa reticularis (DPR) is a rare ectodermal disorder with the diagnostic triad of generalized reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy.
What is reticulate hyperpigmentation?
Reticulate hyperpigmentation is a feature of a number of conditions, which differ in age of onset and distribution of the lesions. Associated clinical findings (e.g. hair, nail, teeth, systemic involvement), are used to differentiate between the conditions. Histopathological examination is useful in some disorders.
What is ashy dermatosis?
Ashy dermatosis is a chronic condition characterized by asymptomatic, slate-gray or violaceous hyperpigmented macules distributed most commonly over the trunk and proximal extremities, and less frequently over the face and neck.
What is acral Melanosis?
The term “acral” means the distal portions of the limbs (hand, foot) and the head (ears, nose). The term acral melanosis is loosely applied to an increase in melanin pigmentation, in a diffuse, reticulate, or focal pattern over the distal parts of the arms and legs.
Is lichen planus curable?
Lichen planus has no cure, but different treatments can help relieve your symptoms and speed healing. Possible treatments include: Antihistamine medicine to relieve itching.
Can lichen planus Pigmentosus cured?
Brief Summary: There are currently no effective treatments for lichen planus pigmentosus (LPP) and erythema dyschromicum perstans (EDP). Tranexamic acid, which may downregulate pigmentation through a reduction in plasmin, has been shown to decrease pigmentation in patients with melasma, another pigmentary disorder.
What does acral lentiginous melanoma ALM look like?
The main sign of acral lentiginous melanoma is a black or brown discoloration that appears on the sole of the foot or palm of the hand. It may resemble a bruise or stain, but over time it grows in size. Acral lentiginous melanoma occurs equally among people of all races and backgrounds.
Why is there skin hyperpigmentation with Addison’s disease?
Presentation of chronic Addison disease It is caused by the stimulant effect of excess adrenocorticotrophic hormone (ACTH) on the melanocytes to produce melanin. The hyperpigmentation is caused by high levels of circulating ACTH that bind to the melanocortin 1 receptor on the surface of dermal melanocytes.
Can I get a tattoo if I have lichen planus?
Tattooing can lead to koebnerisation in patients with active psoriasis,, lichen planus and vitiligo. Tattooing can lead to reactivation of infections such as herpes simplex and herpes zoster, subacute cutaneous and discoid lupus erythematosus, and pyoderma gangrenosum.
What kind of dysplasia is Naegeli-Franceschetti-Jadassohn syndrome?
Naegeli–Franceschetti–Jadassohn syndrome ( NFJS ), also known as chromatophore nevus of Naegeli and Naegeli syndrome, is a rare autosomal dominant form of ectodermal dysplasia, characterized by reticular skin pigmentation, diminished function of the sweat glands, the absence of teeth and hyperkeratosis of the palms and soles.
How is the diagnosis of Naegeli syndrome made?
In most cases of Naegeli syndrome, a diagnosis is made based on the typical clinical features of this condition. The diagnosis may be confirmed by genetic testing of the KRT14 gene. Treatment for Naegeli syndrome is based on an individual’s symptoms. Dry skin can be moisturized with creams.
What are the names of the reticulate pigmentary disorders?
NFJ syndrome is a reticulate pigmentary disorder. Other reticulate pigmentary diseases include X-linked reticulate pigmentary disorder, dermatopathia pigmentosa reticularis, Dowling-Degos disease, dyschromatosis, confluent and reticulated papillomatosis of Gougerot and Carteaud, and reticulated acropigmentation of Kitamura.
Can a dyskeratosis congenita patient have reticulate hyperpigmentation?
Dyskeratosis congenita patients can have dental findings, reticulate hyperpigmentation, adermatoglyphia, palmoplantar hyperkeratosis, and nail anomalies similar to NFJ patients.