Hailey-Hailey Disease: An Updated Review With a Focus on Therapeutic Mechanisms. [PDF]
ABSTRACT Background Hailey‐Hailey disease (HHD), or familial benign chronic pemphigus, is a rare autosomal dominant genodermatosis characterized by chronic, painful, erythematous, erosive plaques and fissures at sites of friction such as the neck, axilla, groin, and perineum.
Mathur M +4 more
europepmc +2 more sources
Evaluating quality of life tools in North American patients with erythropoietic protoporphyria and X-linked protoporphyria. [PDF]
BackgroundErythropoietic protoporphyria (EPP) and X-linked Protoporphyria (XLP) are rare photodermatoses presenting with severe phototoxicity. Although anecdotally, providers who treat EPP patients acknowledge their life-altering effects, tools that ...
Anderson, Karl E +10 more
core +1 more source
Dipropylamine for 9-Fluorenylmethyloxycarbonyl (Fmoc) Deprotection with Reduced Aspartimide Formation in Solid-Phase Peptide Synthesis. [PDF]
Herein, we report dipropylamine (DPA) as a fluorenylmethyloxycarbonyl (Fmoc) deprotection reagent to strongly reduce aspartimide formation compared to piperidine (PPR) in high-temperature (60 °C) solid-phase peptide synthesis (SPPS).
Javor, Sacha +3 more
core +1 more source
Acute Hepatic Porphyrias: Review and Recent Progress. [PDF]
The acute hepatic porphyrias (AHPs) are a group of four inherited diseases of heme biosynthesis that present with episodic, acute neurovisceral symptoms.
Andant +40 more
core +1 more source
Translational advances of melanocortin drugs: Integrating biology, chemistry and genetics. [PDF]
Melanocortin receptors have emerged as important targets with a very unusual versatility, as their widespread distribution on multiple tissues (e.g. skin, adrenal glands, brain, immune cells, exocrine glands) together with the variety of physiological ...
Boesen, T +2 more
core +1 more source
Medication related to pigmentation of oral mucosa [PDF]
The diagnosis of oral melanotic lesions is, more often than not, challenging in the clinical practice due to the fact that there are several reasons which may cause an increase in pigmentation on localized or generalized areas.
Castro-Janeiro, María +4 more
core +1 more source
Paternal Split-Liver Transplantation Followed by Haploidentical Hematopoietic Cell Transplantation in an Adult Patient With Protoporphyria-Induced Liver Failure. [PDF]
ABSTRACT Introduction Erythropoietic Protoporphyria (EPP) caused skin light sensitivity and liver cirrhosis in a 35‐year‐old patient who subsequently developed liver‐failure. Methods In absence of a human leukocyte antigens (HLA)‐matched‐unrelated donor, the father consented in donating for split liver transplantation (SLT) and allogeneic hematopoietic
Stölzel U +12 more
europepmc +2 more sources
Emerging drugs for the treatment of vitiligo [PDF]
Introduction: Vitiligo is a relatively common autoimmune depigmenting disorder of the skin. There has been a great advance in understanding the pathological basis, which has led to the development and utilization of various new molecules in treating ...
Goldust, M. +9 more
core +1 more source
Erythropoietin signaling regulates heme biosynthesis [PDF]
Heme is required for survival of all cells, and in most eukaryotes, is produced through a series of eight enzymatic reactions. Although heme production is critical for many cellular processes, how it is coupled to cellular differentiation is unknown ...
Acin-Perez +96 more
core +2 more sources
Indications and Limitations of Afamelanotide for Treating Vitiligo—Reply [PDF]
Vitiligo is characterized by depigmented patches of skin due to loss of cutaneous melanocytes. Many recent studies have demonstrated defects in the melanocortin system in patients with vitiligo, including decreased circulating and lesional skin levels of α-melanocyte-stimulating hormone (α-MSH).
Henry W, Lim +2 more
openaire +4 more sources

