New skin diseases discoveredA red meat allergy related to tick vaccination – just one example of previously unknown skin conditions. New diseases in the field of dermatology were presented at a training week.
By Sebastian Lux Published: 30.08.2016, 05:03
New skin disease: Eating red meat causes contact allergy in some people.
Thousands of skin diseases have already been described, but new conditions are still emerging in dermatology. In the 25. Advanced training week for practical dermatology. Venereology in Munich (FOBI 2016), some new additions have now been presented. Training week for practical dermatology. Venereology in Munich (FOBI 2016), some new additions have now been presented.
The latest dermatological diseases have Professor Thomas A. Luger from Munster thereby divided into two groups: To the first group belong thereby new Dermatosen with to a large extent unknown Pathomechanismus. For these, mostly only descriptive case descriptions are available. The second group includes dermatoses whose pathomechanism has been elucidated and which are defined on the basis of this mechanism.
Infantile transient contraction of skin smooth muscle belongs to the first group (J Am Acad Dermatol 2013; 69: 498). These protrusions occur in affected newborns within the first days or weeks of life, usually on both lower extremities. Trigger factors are cold or also mechanical stimuli. Histologically shows normal skin. By the age of 18 to 24 months, the phenomenon has completely normalized. Possible causes are immaturity of the autonomic nervous system. A transient hypersensitivity of the menthol receptor TRPM8 discussed. The receptor is responsible for the cold sensation. "If one should see something like this, one need not be alarmed", said Luger. Because the findings regress on their own.
Aplasia cutis congenita (ACC) was first described in 1767, but a variant with extensive intracranial arteriovenous malformations is new (Pediatr Dermatol 2015; 32: e163). In the case described, an infant had several oval sunken areas without hair growth on the scalp, but there was no evidence of other abnormalities.
These did not manifest until the age of seven, including recurrent hemiplegic headaches, orbital edema and motor disturbances. Magnetic resonance imaging finally revealed the extensive intracranial arteriovenous malformations.
Luger recommended long-term follow-up of group I ACC patients (scalp, with no or only isolated abnormalities).
The second group includes the inherited periodic fever syndromes, which are distinguished from the classic autoimmune diseases after the discovery of the corresponding genes. In recent years, a number of new diseases have emerged, including the new variant known as cryopyrin-associated periodic fever syndrome (CAPS).
These include familial cold urticaria (also incorrectly called urticarial vasculitis), muckle-wells syndrome and neonatal inflammatory multiorgan syndrome (NIM).
Syndrome with conjunctivitis and hearing loss
Clinically, CAPS are very heterogeneous, so diagnosis is often delayed. In familial cold urticaria, the trigger is cold, but in muckle-wells syndrome, the symptoms occur independently of cold. The initial manifestation is highly variable; conjunctivitis and progressive neurosensory hearing loss make the disease a multisystem disorder.
Even more extreme is NIM, with massive CNS involvement the most severe course of CAPS. CAPS can be treated with the drugs anakinra, rilonacept and canakinumab with response rates between 75 and 80 percent. Treatment with anakinra. Canakinumab is already possible from the 8. Possible by the age of one month. month of life.
Alpha-gal syndrome: meat allergy and tick bite
Probably the best known new skin disease is alpha-gal syndrome, a novel IgE-mediated immediate-type allergy directed against the disaccharide galactose-α-1,3-galactose. The syndrome presents as a food allergy after consumption of mammalian meat and offal and as a drug allergy (to cetuximab, among others) in suspected sensitization by tick bites (Allergo J Int 2016; 25: 55).
Luger recommended increased attention to delayed reactions after consumption of red meat. In addition, persistent local reactions after a tick bite are a possible indication of alpha-gal syndrome, he said.