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Pustular psoriasis
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Santanu Banerjee, Neerja Saraswat
Evidence suggests that some cases of GPP may actually represent a new genetic auto-inflammatory disease based on mutations in the IL36RN gene, which encodes the IL-36 receptor antagonist. This disease is known as the deficiency of interleukin-36-receptor antagonist (DITRA).
Inflammatory dermatoses affecting the nail
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
The nail organ is an integral part of the functional and sensory fingertip unit.8,9 It is formed by the nail apparatus itself, all constituents of the distal phalanx, the distal interphalangeal joints with their capsule, tendons, and ligaments. The bone insertions of ligaments and tendons called entheses play an important role for the functional and esthetic integrity of the nail. They have recently been found to be of utmost importance in psoriatic arthritis and nail psoriasis.10,11 The proximal tip of the matrix is just 0.8 to 1 mm from the bone of the terminal phalanx and also very close to the distal interphalangeal joint.12,13 The joint capsule is enforced by the flexor and extensor tendons which form the dorsal and volar aponeuroses. They insert mostly at the base of the distal phalanx, but there are also fibers radiating to the more distal dorsal surface of the bone and into the connective tissue of the proximal nail fold.14 This led some authors to call the nail a musculoskeletal appendage (see Chapter 1).15,16 These authors believe that the nail involvement in psoriasis is rather a Koebner phenomenon and thus mechanically induced rather than an autoimmune disease. The complex blood supply of the distal joint and nail as well as the anatomic vicinity of matrix and joint are supposed to give an explanation why nail involvement is so frequent in psoriatic arthritis patients.15 The finding of a mutation in the gene for the interleukin 36 receptor antagonist (IL6RN) leading to a defect in interleukin 36 antagonist (DITRA) in generalized pustular psoriasis and acrodermatitis continua suppurativa supports the assumption of these conditions belonging to the group of autoinflammatory diseases.17,18
Pediatric psoriasis
Published in M. Alan Menter, Caitriona Ryan, Psoriasis, 2017
Pustular and erythrodermic psoriasis are rare but severe variants of pediatric psoriasis,35 occurring in about 1% of pediatric cases.8 Erythrodermic psoriasis involves greater than 90% of the total body surface area. Pustular psoriasis has a mean onset at 6 years of age.36–39 In children (vs. adults), pustular psoriasis is often the first manifestation of disease. Generalized pustular psoriasis is often annular (60%) and is more common than pustulosis palmaris et plantaris (limited to the palms and soles).36,38,40 Pustules in infancy can begin in intertriginous areas, especially the neck fold, and is misdiagnosed as dermatitis, bacterial, or candida infection.38 Pustules are sterile unless secondarily infected. Biopsy shows parakeratosis, elongated rete ridges, spongiotic pustules and Munro's abscesses, and can confirm the clinical diagnosis. Patients often have fever, malaise, and anorexia, and require hospitalization. Courses may be complicated by cutaneous infection and bacterial septicemia. Relapses are common, and frequent recurrences can be associated with failure to thrive. The onset of psoriasiform pustules during the first year of life raises concern about deficiency of interleukin-1 receptor antagonist (DIRA), which results from inappropriate activation of interleukin-1. Sterile multifocal osteomyelitis or periostitis, joint swelling, stomatitis, and pyoderma gangrenosum are other features.41 Patients respond rapidly to subcutaneous administrations of anakinra 2–4 mg/kg/day. Deficiency of interleukin-36 receptor antagonist (DITRA) is the major known genetic cause of generalized pustular psoriasis in patients of any age without initial plaque psoriasis. DITRA is associated with irritability, tender skin, diarrhea, dysphagia, and failure to thrive. Laboratory evaluation may reveal neutrophilia and thrombocytosis. Skin biopsies in DIRA and DITRA show features similar to those seen in pustular psoriasis.42 A genetic variant of psoriasis seen in patients with CARD14 mutations may also present with pustules overlying erythroderma. Typical presentation for patients with CARD14 mutation is a recalcitrant plaque-type psoriasis or pityriasis rubra pilaris, with onset during infancy or early childhood. Patients may be fairly recalcitrant to systemic medications but respond rapidly to ustekinumab.
Perspectives on the pharmacological management of psoriasis in pediatric and adolescent patients
Published in Expert Review of Clinical Pharmacology, 2021
Emmanuel Mahé, Maud Amy De La Bretêque, Céline Phan
The near future looks promising for the treatment of children and adolescents with psoriasis, particularly if new therapeutics can be evaluated rapidly in young patient populations. In the field of topical therapies, the arrival of combined therapies and new nonsteroidal drugs – such as benvitimob, a first-in-class aryl hydrocarbon receptor (AhR)-modulating agent (TAMA). Among oral treatments, validation of the use of apremilast in young patients is expected in 2021, and new classes of drugs, such as Janus kinase inhibitors, are set to arrive on the market. Among the biologics, the evaluation of anti–interleukin-23 is ongoing. The better knowledge of genetic of psoriasis also leads to new approaches. For instance, generalized pustular psoriasis is a rare, life-threatening type of psoriasis that may develops in childhood. In some of these patient’s loss-of-function mutations have been found in IL36RN, which encodes an interleukin-36–receptor antagonist. Recently a monoclonal antibody against the interleukin-36 receptor (BI 655,130) has been developed with promising results [76].
Rationale for IL-36 receptor antibodies in ulcerative colitis
Published in Expert Opinion on Biological Therapy, 2020
Clemens Neufert, Markus F. Neurath, Raja Atreya
Interleukin 36 (IL-36) is a group of closely related molecules that belong to the IL-1 family of pro-inflammatory cytokines. IL-36α (IL-1F6), IL-36β (IL-1F8), and IL-36γ (IL-1F9) are activators of the IL-36 receptor (IL-36R), whereas IL-36Ra (IL-1F5), and IL-38 (IL-1F10) are two naturally occurring inhibitors blocking IL-36 receptor (IL-36R) signaling. The clinical importance of a tightly regulated balance of IL-36R signaling was initially highlighted by an autosomal recessive disease in individuals carrying loss-of-function mutations in the IL36RN [7]. This genetic alteration is the molecular basis for the deficiency of IL-36 receptor antagonist (DITRA) syndrome, representing a group of patients suffering from generalized pustular psoriasis (GPP). GPP/DITRA syndrome is the prototypical IL36-mediated disease, which is characterized by recurrent flares of pustular, erythematous rashes of the skin, often resulting in life-threatening consequences [7].
A review of antibiotics and psoriasis: induction, exacerbation, and amelioration
Published in Expert Review of Clinical Pharmacology, 2019
Generalized pustular psoriasis (GPP): amoxicillin was listed as an induction or exacerbation factor of psoriasis in literature. Two case reports found induction of GPP after amoxicillin use [7,8]. Sugiura et al. especially suggested against use of amoxicillin in Interleukin 36 receptor antagonist (IL-36RN) mutation carriers [7].Streptococcus pneumoniae exposed to penicillin induced human macrophages to release pro-inflammatory cytokines such as TNF-α and IL-1β [9]. Sugiura et al. suggested that GPP triggered by amoxicillin is through similar mechanisms. IL-36 (also belongs to IL-1 family) production is increased when bacteria are exposed to β-lactam antibiotics [7] (Table 1).Guttate psoriasis: there was a case report of guttate psoriasis following streptococcal vulvovaginitis successfully treated with amoxicillin [10].