Pustular psoriasis as an autoinflammatory keratinization disease (AiKD): Genetic predisposing factors and promising therapeutic targets.

Pustular psoriasis is a chronic inflammatory skin disease characterized by erythematous plaques with sterile pustules. It includes the distinct clinical entities generalized pustular psoriasis (GPP), acrodermatitis continua of Hallopeau (ACH) and palmoplantar pustular psoriasis (PPPP). Recently clarified pathomechanisms of pustular psoriasis indicate that hyperactivation of the skin innate immunity, including of the IL-1/IL-36 axis, plays an important role in the pathogenesis of pustular psoriasis. Autoinflammatory keratinization disease (AiKD) is the umbrella clinical entity for inflammatory keratinization disorders with genetic autoinflammatory pathomechanisms, and pustular psoriasis is a representative AiKD. To date, mutations/variants in five genes-IL36RN, CARD14, AP1S3, MPO and SERPINA3-have been reported to be genetic causative or predisposing factors for pustular psoriasis. The pathogenic mechanisms induced by the mutations/variants in these genes are all closely related to the excessive activation of skin innate immunity and autoinflammation. A number of biologics (e.g., tumor necrosis factor inhibitors, IL-17/IL-17 receptor inhibitors and IL-23 inhibitors) and granulocyte and monocyte adsorption apheresis are used to treat pustular psoriasis. Recently, based on novel information on the pathomechanisms of pustular psoriasis, which are mainly associated with autoinflammation, inhibitors of several pathogenic pathways, including of the IL-1, IL-36, IL-8 and granulocyte colony-stimulating factor signaling pathways, have been studied as emerging treatments.

Effectiveness of saline water and lidocaine injection treatment of intractable plantar keratoma: a randomised feasibility study.

BACKGROUND: An intractable plantar keratoma (IPK) is a conical thickening of the epidermis' stratum corneum and a common cause of foot pain which can have a significant, detrimental impact on the mobility, quality of life and independence of individuals. Conservative treatments are currently offered to patients with IPK, but they are unsatisfactory since they do not offer a sufficient or permanent reduction of symptoms. The purpose of this study was the evaluation of the feasibility, safety and effectiveness of innovative treatments for intractable plantar keratoma (IPK). METHODS: A randomized single blind trial with 40 patients divided with block randomization in four parallel groups was conducted to compare treatment combinations: conservative sharp debridement only or sharp debridement with needle insertion, physiological water injection or lidocaine injection. All patients obtained the same treatment four times at a four-week interval. At each visit, visual analog scale (VAS), Foot Function Index (FFI) and IPK size were evaluated. VAS and FFI were also completed at a six and twelve-month follow-up. RESULTS: Our findings in regards to feasibility demonstrated recruitment challenges because of the anticipated pain that would be provoked by needle insertion may not be worth the potential pain relief compared to debridement alone from the patient's perspective. This was also the principal cause of drop out. Our preliminary results show no main effect of group for any of the clinical outcomes: pain felt on VAS, FFI score, IPK's size (p > 0.05). However, the analysis revealed a statistically significant effect of time on VAS (p < 0.001), FFI score (p < 0.001) and IPK's size (width and depth (p < 0.001); length (p = 0.001)), but no group x time interaction was found (p > 0.05). CONCLUSIONS: This study demonstrates that IPK treatment consisting of sharp debridement with needle insertion, physiological saline water injection or lidocaine injection is feasible and safe. There was a non-statistically significant trend toward diminishing pain intensity compared to scalpel debridement alone. The pain provoked by needle insertion and injection treatments must be addressed with a scientifically proven protocol to make it more comfortable for patients before these treatments could be considered in further studies. TRIAL REGISTRATION: ClinicalTrials.gov, NCT04777227 . 2 March, 2021 - Retrospectively registered (All participants were recruited prior to registration).

Hyperkeratosis lenticularis perstans (Flegel's disease): our experience and review of the literature.

Hyperkeratosis lenticularis perstans (HLP), or Flegel's disease (FD), is a rare disorder of keratinization first described in 1958 by Flegel. HLP is characterized by asymptomatic small papules distributed symmetrically on the dorsal feet and lower part of the legs. It typically presents in the fourth or fifth decade of life. FD has been reported more commonly in women, and little is known about the pathogenesis of this rare dermatosis. Histological examination is mandatory to confirm the diagnosis, and the characteristic features are: focal compact hyperkeratosis, thinned stratum malpighii, epidermal atrophy, and a dense, band-like lymphocytic infiltrate in the papillary dermis. The treatment of FD is challenging, and a fully effective standard therapy does not exist. We report four cases of HLP that have been diagnosed at the Dermatology Unit of IRCCS Ca' Granda Ospedale Maggiore Policlinico in Milan, Italy, and a literature review is enclosed.

Chronic Oral Lesions.

Chronic oral mucosal lesions can be associated with several mucocutaneous diseases. This article reviews the autoimmune and immune-mediated, reactive, genetic, and infectious diseases that may present with chronic oral and/or cutaneous manifestations and provides a rational approach to diagnosis and management.

Milia-like idiopathic calcinosis cutis of the forehead in an adult without Down's syndrome successfully treated with a CO2 laser.

BACKGROUND: Idiopathic calcinosis cutis is an uncommon form of calcinosis cutis. It may present as tumoral calcinosis, subepidermal calcified nodules or scrotal calcinosis. Subepidermal calcified nodules may also present as milia-like lesions commonly seen in children with Down's syndrome in the absence of tissue damage or metabolic disorders, it has been seldom reported in adults. The treatment of choice is surgical excision. However, a surgical approach may not always be beneficial to the patient given the cosmetic outcomes. Here, we describe the successful use of a CO2 laser in the treatment of milia-like calcinosis cutis of the forehead in an adult without Down's syndrome. AIMS: To describe the treatment of Milia-like idiopathic calcinosis cutis of the forehead in an adult without Down's syndrome successfully treated with a CO2 laser. METHODS: We report a case of a 48-year-old man who presented with skin-colored hard asymptomatic papules on the forehead that started about 9 years ago, a biopsy was performed and a diagnosis of milialike calcinosis cutis was made. Surgical excision was discarded regarding the location and the number of lesions, instead, a CO2 laser was used to treat this condition. RESULTS: Successful laser excision of the lesions with appealing cosmetic results. CONCLUSIONS: CO2 laser seems to be a valuable tool to treat milia-like calcinosis cutis lesions.

[Chemical peeling in dermatology]. / Chemisches Peeling in der Dermatologie.

In this article, a comprehensive, yet introductory overview on chemexfoliation is given. The molecular mechanisms for selected indications are exemplified and the prerequisites, necessary precautions as well as potential complications are addressed. Finally, selected available superficial peeling substances are presented and the principal peeling procedure is outlined briefly.

Effectiveness of photopneumatic technology: a descriptive review of the literature.

Usage of photopneumatic technology has recently increased for treatment of different skin conditions such as acne, keratosis pilaris (KP), and rosacea. Photopneumatic devices combine gentle negative pressure with broad band pulsed light simultaneously to attack multiple targets in the skin for better treatment outcomes. In this literature review, we evaluate the efficacy of photopneumatic therapy on treatment of acne, keratosis pilaris (KP), and rosacea.

Hyperkeratotic hand eczema compared to other subgroups of hand eczema - a retrospective study with a follow-up questionnaire.

BACKGROUND: Only a few clinical trials on hand eczema have included further classification into subtypes, and there is a need for studies evaluating the present classifications. OBJECTIVES: To examine differences in demographic factors, lifestyle factors and severity between subgroups of hand eczema patients, with a focus on hyperkeratotic hand eczema. METHODS: This was a retrospective study including hand eczema patients referred to the outpatient dermatological clinic, Bispebjerg Hospital, between January 2013 and July 2014. The study comprises information on subdiagnoses, treatment and foot eczema from patient files, as well as a follow-up questionnaire. RESULTS: A total of 120 patients were included, 10 of whom were diagnosed with hyperkeratotic hand eczema. A significantly higher proportion of the patients with hyperkeratotic hand eczema were male (p = 0.002) and received systemic or ultraviolet (UV) treatment (p = 0.026). The frequency of tobacco smoking was significantly higher in patients with hyperkeratotic hand eczema (p = 0.016), as well as in the other subgroups combined (p = 0.049), than in the background population. CONCLUSIONS: Studies evaluating the subdiagnoses of hand eczema are needed, to further validate the classification system, and to provide more detailed information about demographic factors, severity and possible risk factors for different subgroups of hand eczema.

Successful treatment of facial milia in an infant with orofaciodigital syndrome type 1.

We report the successful treatment of multiple facial milia with manual extraction and tretinoin in a child with orofaciodigital syndrome type 1. Treatment with topical medications may be insufficient in individuals with orofaciodigital syndrome type 1, and pitted scarring is often a sequala. This case demonstrates that manual extraction is well tolerated and effective in the treatment of multiple milia. In addition, clinicians need to be aware of this rare genetic condition, which commonly presents de novo and can lead to significant morbidity if untreated.

[Clinicopathological aspects of terra firma-forme dermatosis]. / Dermatose en terre sèche : aspects anatomo-cliniques.

BACKGROUND: Terra firma-forme dermatosis was first described in 1987. It is characterized by the appearance in children or adolescents of dirt-like patches despite normal cleansing. The lesions are removed when the skin is rubbed with a 70°C alcohol swab. This largely unknown condition is of uncertain origin but could be due to a keratinisation disorder. PATIENTS AND METHODS: Herein we report the case of a 17-year-old male patient who, after seeking the opinions of a number of doctors, consulted in dermatology for multiple pigmented patches on the trunk and limbs that had appeared one year earlier. These lesions had persisted despite appropriate cleansing. He had no history of endocrine disorders and no familial history of pigmentation disorders. A skin biopsy revealed compact orthokeratotic hyperkeratosis resembling a second layer of stratum corneum on top of a normal layer. Very small granules were seen within this layer and PAS staining revealed spores. These skin lesions disappeared when rubbed with an alcohol swab, resulting in a diagnosis of terra firma-forme dermatosis. DISCUSSION: Where a diagnosis of terra firma-forme dermatosis is suspected, confirmation is easy to obtain using an alcohol swab. Nevertheless, the disease is not familiar to dermatologists. The most common differential diagnoses are acanthosis nigricans, reticulate and confluent papillomatosis, and Dowling-Degos disease. Their histopathological appearances are very different and skin biopsy can be useful for diagnosis. These entities belong to a group of disorders involving abnormal keratinisation and pigmentation. Anatomoclinical correlation is essential for optimal management. The appearance of the stratum corneum may play an important role in the diagnosis of terra firma-forme dermatosis.

Incidence of trichostasis spinulosa at a single institution in Yemen.

BACKGROUND: Trichostasis spinulosa (TS) is a common, underdiagnosed cosmetic skin condition. OBJECTIVES: The main objectives of this study were to determine the occurrence of TS relative to age and gender, to analyze its cutaneous distribution, and to investigate any possible familial basis for this condition, its impact on patients, and the types and efficacy of previous treatments. METHODS: All patients presenting to the outpatient dermatology clinic at the study institution and their relatives were examined for the presence of TS and were questioned about family history and previous treatment. Photographs and biopsies of suspected cases of TS were obtained. RESULTS: Of 2400 patients seen between August and December 2013, 286 patients were diagnosed with TS (135 males, 151 females; prevalence: 11.9%). Women presented more frequently than men with complaints of TS (6.3 vs. 4.2%), and more women had received prior treatment for TS (10.5 vs. 2.8%). The most commonly affected sites were the face (100%), interscapular area (10.5%), and arms (3.1%). Lesions involved the nasal alae in 96.2%, the nasal tip in 90.9%, the chin in 55.9%, and the cheeks in 52.4% of patients. Only 15.7% of patients had forehead lesions, and only 4.5% had perioral lesions. Among the 38 previously treated patients, 65.8% reported temporary improvement. CONCLUSIONS: Trichostasis spinulosa is a common condition that predominantly affects the face in patients of all ages. Additional studies employing larger cohorts from multiple centers will be required to determine the prevalence of TS in the general population.

Keratosis lichenoides chronica: Case-based review of treatment options.

Keratosis lichenoides chronica (KLC) is a rare dermatological condition characterized by keratotic papules arranged in a parallel linear or reticular pattern and facial lesions resembling seborrheic dermatitis or rosacea. The clinical, histological and therapeutic information on 71 patients with KLC retrieved through a PubMed search plus one our new case were analyzed. KLC affects patients of all ages, with a modest male predominance. Pediatric cases represent about one quarter of patients. Diagnosis is usually delayed and histologically confirmed. All patients have thick, rough and scaly papules and plaques arranged in a linear or reticular pattern, on limbs (>80%) and trunk (about 60%). Face involvement is described in two-thirds of patients. Lesions are usually asymptomatic or mildly pruritic. Other manifestations, such as palmoplantar keratoderma, mucosal involvement, ocular manifestations, nail dystrophy, are reported in 20-30% of patients. Children present more frequently alopecia. No controlled trials are available. Results from small case series or single case reports show that the best treatment options are phototherapy and systemic retinoids, alone or in combination, with nearly half of patients reaching complete remission. Systemic corticosteroids as well as antibiotics and antimalarials are not effective.

Hiperqueratosis nevoide bilateral de areola y pezón. presentación de un caso / Hyperkeratosis of the nipple and areola

La hiperqueratosis nevoide de areola y pezón es una entidad patológica de la dermatología escasamente presentada en la literatura. Descrita por primera vez en 1938 por Levy – Frankel,2 quien además establece una clasificación etiológica de la misma. En este trabajo se presenta un caso de hiperqueratosis nevoide bilateral de areola y pezón, y su tratamiento con galvanocauterio que da muy buenos resultados no solo estéticos sino primordialmente por la nula recidiva.

Hyperkeratosis of the nipple and the areola is a pathological entity of dermatology sparsely reported, first described in 1938 by Levy - Frankel, who established an etiological classification of that entity. In this paper a case of bilateral hyperkeratosis of the nipple and the areola and its successful treatment with galvanocautery surgery with no long-term recurrence is reported.