Diffuse Cutaneous Mastocytosis: A Current Understanding of a Rare Disease.

Mastocytosis is a heterogeneous disease characterized by the expansion and accumulation of neoplastic mast cells in various tissues. Diffuse cutaneous mastocytosis (DCM) is a rare and most severe form of cutaneous mastocytosis, which typically occurs in childhood. There have been reports of a familial DCM with specific gene mutations, indicating both sporadic and hereditary factors involved in its pathogenesis. DCM is associated with severe MC mediator-related symptoms and an increased risk of anaphylaxis. The diagnosis is based on the appearance of skin lesions, which typically show generalized thickening, erythroderma, blistering dermographism, and a positive Darier's sign. Recognition, particularly in infants, is challenging due to DCMs resemblance to other bullous skin disorders. Therefore, in unclear cases, a skin biopsy is crucial. Treatment focuses on symptom management, mainly including antihistamines and mast cell stabilizers. In extremely severe cases, systemic steroids, tyrosine kinase inhibitors, phototherapy, or omalizumab may be considered. Patients should be equipped with an adrenaline autoinjector. Herein, we conducted a comprehensive review of literature data on DCM since 1962, which could help to better understand both the management and prognosis of DCM, which depends on the severity of skin lesions, intensity of mediator-related symptoms, presence of anaphylaxis, and treatment response.

Treatment of nail psoriasis with pulsed dye laser versus combined pulsed dye and Nd:YAG lasers-An intrapatient left-to-right study.

BACKGROUND AND OBJECTIVES: There are limited treatment options for nail psoriasis. It is important to find new therapies and improve existing ones. The aim of this study was to compare the effectiveness of pulsed dye laser (PDL) versus combined PDL and Nd:YAG lasers in patients with nail psoriasis. METHODS: Fourteen patients (with a total of 126 nails affected by nail psoriasis) were treated with PDL (6 J/cm2, 7 mm, 0.45 milliseconds) on both hands and additionally with Nd:YAG (10 J/cm2 , 6 mm, 15 milliseconds) on the right hand. Three treatment sessions were applied at 4-week intervals and patients were followed up for 6 months after the last session. Disease severity was assessed using the Nail Psoriasis Severity Index, both 8- and 32-point variant. Additionally, Dermatology Life Quality Index was assessed before and after treatment. RESULTS: Overall, there was a statistical difference in 8- and 32-point NAPSI score before and after treatment for both hands. However, there was no statistical difference between the score for the right and left hands based on both scale variants. Some aspects of patients' lives showed improvement due to the treatment. CONCLUSIONS: Both PDL in monotherapy and combined Nd:Yag+PDL lasers are safe and effective in treatment of nail psoriasis, although combined therapy shows no advantage over the use of a single laser.

JAK-STAT Inhibitors in Atopic Dermatitis from Pathogenesis to Clinical Trials Results.

A common disease worldwide is known as atopic dermatitis (AD), named also as atopic eczema, which is a chronic recurrent complex inflammatory skin disorder. It affects 2-10% of the adult population and up to 20% of the pediatric population. The clinical AD picture appears in typically localized eczema and dry skin, and is dominated by a persistent pruritus followed by sleep disturbances. AD strongly impacts on the quality of life of AD patients and their families as well as on social and economic aspects. The pathogenesis of the disease is complex and consists of multiple interactions between immunological disturbances, skin barrier defect, and microbial dysbiosis with environmental influences. The treatment of AD reflects the pathogenetic disorders, starting from basic emollient therapy, and goes to topical anti-inflammatory regimens followed by phototherapy, systemic immunosuppressive drugs, and new biologic immunomodulators. This paper will thus summarize the novel collection of biological treatment JAK-STAT inhibitors dedicated to AD.

Atopic dermatitis. Interdisciplinary diagnostic and therapeutic recommendations of the Polish Dermatological Society, Polish Society of Allergology, Polish Pediatric Society and Polish Society of Family Medicine. Part II. Systemic treatment and new therapeutic methods.

The treatment goal in atopic dermatitis is eliminating clinical symptoms of the disease, preventing exacerbations and complications, as well as improving patients' quality of life. In cases of severe atopic dermatitis and lack of response it is recommended to introduce systemic therapy. Patients ofter require multi-specialist consultations, and occasionally hospitalization. It is not recommended to use acupuncture, acupressure, bioresonance, homeopathy, or Chinese herbs in the treatment of atopic dermatitis.

Atopic dermatitis. Interdisciplinary diagnostic and therapeutic recommendations of the Polish Dermatological Society, Polish Society of Allergology, Polish Pediatric Society and Polish Society of Family Medicine. Part I. Prophylaxis, topical treatment and phototherapy.

Atopic dermatitis is a chronic and recurrent inflammatory dermatosis with concomitant intensive pruritus, and is diagnosed both in children and adults. Atopic dermatitis-patients are predisposed to have bacterial, viral and fungal skin infections; they also suffer from an increased risk of developing food allergies (especially, at an infantile age), allergic rhinitis, or bronchial asthma (a so-called atopic march). Currently, an increasing atopic dermatitis incidence constitutes a serious medical problem that regards not only dermatology and allergology, but also paediatrics, and family medicine. The basis for atopic dermatitis treatment and prophylaxis is restoration of epidermal barrier functions by means of tailored emollients. Atopic dermatitis therapies should effectively eliminate clinical symptoms of the disease, prevent exacerbations as well as complications, and improve patients' quality of life.

Cutaneous mastocytosis treatment: strategies, limitations and perspectives.

Mastocytosis is a rare myeloproliferative disease, characterized by excessive proliferation and accumulation of mast cells in the tissues. In cutaneous mastocytosis (CM), mast cells infiltration is limited to the skin, whereas in systemic mastocytosis (SM) internal organs are involved. The first-line treatment in CM is antimediator therapy (mainly H1 and H2 antihistamines) and short-term topical corticosteroids. Phototherapy is the second-line therapy which may be considered when antihistamines do not produce the expected improvement. New therapeutic options include omalizumab and KIT-targeting agents. Although the disappearance of skin lesions has been reported as a result of cytoreductive therapies in SM, the use of potentially toxic drugs in CM is not recommended. In all adults with mastocytosis and in pediatric patients with severe CM, a persistently elevated serum tryptase level and anaphylaxis in medical history, equipping with epinephrine autoinjector for use in case of anaphylaxis is recommended.