Unmet Medical Needs in Chronic, Non-communicable Inflammatory Skin Diseases

Hideyuki Ujiie; David Rosmarin; Michael P Schön; Sonja Ständer; Katharina Boch; Martin Metz; Marcus Maurer; Diamant Thaci; Enno Schmidt; Connor Cole; Kyle T Amber; Dario Didona; Michael Hertl; Andreas Recke; Hanna Graßhoff; Alexander Hackel; Anja Schumann; Gabriela Riemekasten; Katja Bieber; Gant Sprow; Joshua Dan; Detlef Zillikens; Tanya Sezin; Angela M Christiano; Kerstin Wolk; Robert Sabat; Khalaf Kridin; Victoria P Werth; Ralf J Ludwig

doi:10.3389/fmed.2022.875492

. 2022 Jun 9;9:875492. doi: 10.3389/fmed.2022.875492

Unmet Medical Needs in Chronic, Non-communicable Inflammatory Skin Diseases

Hideyuki Ujiie ¹, David Rosmarin ², Michael P Schön ^3,⁴, Sonja Ständer ⁵, Katharina Boch ⁶, Martin Metz ^7,⁸, Marcus Maurer ^7,⁸, Diamant Thaci ⁹, Enno Schmidt ^6,¹⁰, Connor Cole ^11,¹², Kyle T Amber ^11,¹², Dario Didona ¹³, Michael Hertl ¹³, Andreas Recke ⁶, Hanna Graßhoff ¹⁴, Alexander Hackel ¹⁴, Anja Schumann ¹⁴, Gabriela Riemekasten ¹⁴, Katja Bieber ¹⁰, Gant Sprow ^15,¹⁶, Joshua Dan ^15,¹⁶, Detlef Zillikens ⁶, Tanya Sezin ¹⁷, Angela M Christiano ¹⁷, Kerstin Wolk ^18,¹⁹, Robert Sabat ^18,¹⁹, Khalaf Kridin ^10,²⁰, Victoria P Werth ^15,¹⁶, Ralf J Ludwig ^6,^10,^*

¹Department of Dermatology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan

²Department of Dermatology, Tufts Medical Center, Boston, MA, United States

³Department of Dermatology, Venereology and Allergology, University Medical Center Göttingen, Göttingen, Germany

⁴Lower Saxony Institute of Occupational Dermatology, University Medical Center Göttingen, Göttingen, Germany

⁵Center for Chronic Pruritus, Department of Dermatology, University Hospital Muenster, Muenster, Germany

⁶Department of Dermatology, University of Lübeck, Lübeck, Germany

⁷Institute for Allergology, Charité—Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany

⁸Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany

⁹Institute and Comprehensive Center for Inflammation Medicine, University of Lübeck, Lübeck, Germany

¹⁰Lübeck Institute of Experimental Dermatology and Center for Research on Inflammation of the Skin, University of Lübeck, Lübeck, Germany

¹¹Division of Dermatology, Rush University Medical Center, Chicago, IL, United States

¹²Department of Internal Medicine, Rush University Medical Center, Chicago, IL, United States

¹³Department of Dermatology and Allergology, Philipps-Universität, Marburg, Germany

¹⁴Department of Rheumatology and Clinical Immunology, University of Lübeck, Lübeck, Germany

¹⁵Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States

¹⁶Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA, United States

¹⁷Department of Dermatology, Columbia University Medical Center, New York, NY, United States

¹⁸Psoriasis Research and Treatment Centre, Charité—Universitätsmedizin Berlin, Berlin, Germany

¹⁹Interdisciplinary Group Molecular Immunopathology, Dermatology/Medical Immunology, Charité—Universitätsmedizin Berlin, Berlin, Germany

²⁰Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel

Edited by: Devinder Mohan Thappa, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), India

Reviewed by: Hiroshi Koga, Kurume University School of Medicine, Japan; Reinhart Speeckaert, Ghent University Hospital, Belgium; Takashi Hashimoto, Osaka City University, Japan

^✉

*Correspondence: Ralf J. Ludwig ralf.ludwig@uksh.de

This article was submitted to Dermatology, a section of the journal Frontiers in Medicine

PMCID: PMC9218547 PMID: 35755063

Abstract

An estimated 20–25% of the population is affected by chronic, non-communicable inflammatory skin diseases. Chronic skin inflammation has many causes. Among the most frequent chronic inflammatory skin diseases are atopic dermatitis, psoriasis, urticaria, lichen planus, and hidradenitis suppurativa, driven by a complex interplay of genetics and environmental factors. Autoimmunity is another important cause of chronic skin inflammation. The autoimmune response may be mainly T cell driven, such as in alopecia areata or vitiligo, or B cell driven in chronic spontaneous urticaria, pemphigus and pemphigoid diseases. Rare causes of chronic skin inflammation are autoinflammatory diseases, or rheumatic diseases, such as cutaneous lupus erythematosus or dermatomyositis. Whilst we have seen a significant improvement in diagnosis and treatment, several challenges remain. Especially for rarer causes of chronic skin inflammation, early diagnosis is often missed because of low awareness and lack of diagnostics. Systemic immunosuppression is the treatment of choice for almost all of these diseases. Adverse events due to immunosuppression, insufficient therapeutic responses and relapses remain a challenge. For atopic dermatitis and psoriasis, a broad spectrum of innovative treatments has been developed. However, treatment responses cannot be predicted so far. Hence, development of (bio)markers allowing selection of specific medications for individual patients is needed. Given the encouraging developments during the past years, we envision that many of these challenges in the diagnosis and treatment of chronic inflammatory skin diseases will be thoroughly addressed in the future.

Keywords: medical need, skin, inflammation, atopic dermatitis, psoriasis, alopecia areata, chronic spontaneous urticaria, hidradenitis suppurativa

Chronic, Non-Communicable Inflammatory Skin Diseases

Chronic, non-communicable skin inflammation can be caused by many different diseases. Herein, we categorized these into (i) chronic inflammatory diseases (atopic dermatitis, psoriasis, lichen planus, chronic prurigo, and hidradenitis suppurativa), (ii) autoimmune diseases (alopecia areata, vitiligo, chronic spontaneous urticaria, pemphigus, bullous pemphigoid, mucous membrane pemphigoid, and epidermolysis bullosa acquisita), (iii) autoinflammatory diseases (cryopyrin-associated periodic syndrome and Schnitzler's syndrome), and (iv) rheumatic diseases (cutaneous lupus erythematosus, dermatomyositis, and systemic sclerosis). This categorization is based on the main driving pathomechanism(s) of each disease. However, a clear classification of the pathologic driver is challenging as in lichen planus and psoriasis autoreactive T- and B- cells potentially contribute to disease pathogenesis (1, 2). This classification is also expected to change over time, as it will need to adopt and consider new data on disease pathogenesis. Alternatively, to the here used classification, chronic inflammatory skin diseases may be categorized based on key driving molecules. For example, Janus kinases (JAK) in atopic dermatitis, alopecia areata, vitiligo, and cutaneous lupus erythematosus. Furthermore, as detailed below, for many chronic skin inflammatory diseases the clinical presentation varies greatly even within the same disease, as with psoriasis or bullous pemphigoid (3, 4). With the emerge of multidimensional datasets, it has been proposed to classify inflammatory skin diseases based on molecular patterns (5). The increasing understanding of (molecular) disease pathogenesis and availability of appropriate biomarkers for their identification, we expect a more complex, but more tailored categorization of molecular disease pathogenesis is leading to the emergence of potential biomarkers, and a more categorization of chronic, non-communicable skin inflammatory diseases. These diseases are a major medical burden because of their high and, in many cases, increasing prevalence (Table 1), diagnostic challenges, lack of curative treatments, co-morbidity, as well as significant economic impact. We here selected 17 chronic, non-communicable skin inflammatory diseases that collectively affect 15–20% of the population (Table 1). For each disease, the current diagnostic and therapeutic challenges are outlined. Furthermore, a perspective is given on how these challenges may be met in the future.

Table 1.

Epidemiology of selected chronic inflammatory skin diseases.

Disease	Prevalence rate	Sex distribution	Ethnic/geographic predisposition	Notable trends	References
Atopic dermatitis	10–30% in children and 2–10% in adults	Almost equal sex distribution	Higher in high-income countries	Two- to three-fold increase over the past several decades	(6–8)
Psoriasis	2–3%	Equally prevalent in both sexes	Most common in populations of northern Europe and least common in eastern Asia	An apparent upward trend is observed in several countries	(9–11)
Prurigo nodularis	0.1%	Higher among females	None	Increasing incidence over time	(12, 13)
Lichen planus	0.2–1.3%	Equally prevalent in both sexes	• CLP: equally prevalent in both sexes • MLP: more frequent in the female population • LPP: more frequent in the female population	NA	(14, 15)
Hidradenitis suppurativa	0.1–1.3%	Overall almost equal distribution, but varies between races	Higher in African Americans	NA	(16–18)
Alopecia areata	2%	Slightly higher among females	Higher in African American and Hispanics	The incidence is increasing over time	(19)
Vitiligo	0.2–1.8%	Higher among females	Higher prevalence in African nations	Constant or decreasing frequency in the past decades	(20)
Chronic spontaneous urticaria	0.1–1.4%	Slightly higher among females	Higher prevalence in Asian nations	Increasing incidence over time	(21)
Pemphigus	Orphan	Higher among females	Higher in Ashkenazi Jewish and Mediterranean population	Inconsistent findings	(22, 23)
Bullous pemphigoid	Orphan	Higher among females	None	1.9- to 4.3-fold rise over the past two decades	(24)
Mucous membrane pemphigoid	Orphan	Higher among females	None	NA	(23)
Epidermolysis bullosa acquisita	Orphan	Equally prevalent in both sexes	HLA-DR2 and HLA-DRB1*15:03-associated susceptibility among Africans	NA	(23, 25, 26)
Cryopyrin-Associated periodic syndrome	Orphan	Equally prevalent in both sexes	None	NA	(27, 28)
Schnitzler's syndrome	Orphan	Higher among males	None	NA	(29, 30)
Cutaneous lupus erythematosus	Orphan	Higher among females	Higher in Māori/Pacific population	NA	(31, 32)
Dermatomyositis	Orphan	Higher among females	Higher among Africans and Hispanics	Increasing incidence over time	(33, 34)
Systemic sclerosis	Orphan	Higher among females	Higher among Africans and Hispanics	Increasing incidence over time	(34, 35)

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Orphan, Disease prevalence 5/10,000 or less; NA, not applicable.

Chronic Inflammatory Skin Diseases

Atopic Dermatitis

Atopic Dermatitis (AD) or atopic eczema is a common, chronic, relapsing inflammatory disease, affecting up to 30% of the pediatric population and 2–10% of adults (36). While most commonly symptoms start in the first 5 years of life, it is now recognized that onset can occur at any age. There can be a significant effect on patient's quality of life and sleep due to itch and pain (37). There are also significant effects on patients' mental health with higher incidence of depression and suicide (38). The high burden of disease can interfere with work productivity, not only from the baseline disease but particularly from flares (39). Patient also have many out-of-pocket costs, including cleaning products, clothing, moisturizers, and other expenses (40). In AD, there is an interplay between barrier dysfunction, immune dysregulation, and the microbiome (41). Both genetics and environmental factors play a role in the pathogenesis (42). In AD, the stratum corneum, composed of the terminally differentiated enucleated keratinocytes called corneocytes, is often compromised. Among European Caucasians, filaggrin mutations are associated with early-onset and severe AD (43). Filaggrin is broken down into compounds that constitute natural moisturizing factor which is important for appropriate hydration, desquamation, plasticity, acidity, and the commensal microbiome (44, 45). Patients with AD have a higher burden of Staphylococcus aureus which contributes to the inflammation (46). As allergens penetrate the defective skin barrier in AD, pro-inflammatory cytokines are released. While a type 2 immune response with elevated levels of IL-4 and IL-13 predominate in the acute phase, chronically, a mixed response of Th1, Th17, and Th22 immune cells is observed (47). IL-31 is particularly implicated in pruritus (48).

Diagnosis

AD is usually diagnosed based on clinical experience (Figure 1A). There are diagnostic criteria, but no simple test for definitive diagnosis (49). When patients manifest in atypical locations, develop lesions later in life, have uncommon morphologies or other overlying skin diseases the diagnosis can be challenging. AD is heterogenous and can show racial variation (50). Asians may manifest with well-demarcated lesions and skin of color patients may have increased xerosis, follicular eczema, and post-inflammatory pigmentation changes (51). Allergic contact dermatitis may overly AD, so patch testing should be considered in those with recalcitrant atopic dermatitis. AD is notably associated with other atopic disorders such as asthma, allergic rhinitis, and food allergies. There also has been an association with obesity, malignancy, and cardiovascular disease (52–54). For a precision medicine approach, validated and reliable biomarkers are needed to individually tailor treatment (55).

Clinical images of patients with chronic skin inflammatory diseases. **(A)** Blurry erythema and lichenification at the inside the bend of the elbows and arms of a patient with atopic dermatitis. **(B)** Sharply demarked, scaling, erythematous plaques at the back of a patient with psoriasis vulgaris. **(C)** Generalized erythema and scaling in a patient with psoriasis. **(D)** Erythematous nodules, partially excoriated, in a patient with prurigo nodularis. **(E)** Erosions of the lower gums in a patient with mucosal lichen planus. **(F)** Polygonal, scaling, reddish-violet plaques at the wrist of a patient with cutaneous lichen planus. **(G)** Scaring, nodules and pustules located at the sub-axillary region of a patient with hidradenitis suppurativa. **(H)** Sharply demarked hair loss at the back of the head in a patient with alopecia areata. **(I)** Sharpley demarked white maculae at the hands of a patient with vitiligo. **(J)** Wheals at the back of a patient with chronic spontaneous urticaria. **(K)** Brown macules and erosions at the back of a patient with muco-cutaneous pemphigus vulgaris. **(L)** Tense blisters on erythematous skin on the legs of a patient with bullous pemphigoid. **(M)** Oral erosions in a patient with mucous membrane pemphigoid. **(N)** Tense blisters on erythema on the arm of a patient with inflammatory/non-mechano-bullous epidermolysis bullosa acquisita. **(O)** Tense blister and scaring on the hand of a patient with predominant mechano-bullous epidermolysis bullosa acquisita. **(P)** Wheals at the leg of a patient with cryopyrin-associated periodic syndrome. **(Q)** Urticarial exanthema at the lower back of a patient with Schnitzler's syndrome. **(R)** Alopecia and erythema at the head of a patient with cutaneous lupus erythematosus. **(S)** Erythema and depigmentation at the arm of a patient with cutaneous lupus erythematosus. **(T)** Gottron papules in a patient with dermatomyositis. **(U)** Shortening of the sublingual frenulum in a patient with systemic sclerosis. **(V)** Raynaud's phenomenon (anemic color of the fingers) and necrosis of the index finger in a patient with systemic sclerosis.

Treatment

Treatment is mainly aimed at restoring the skin barrier and modulating the abnormal immune response. Education on skin hygiene strategies is important for all patients, ideally with written action plans. There is uncertainty as to ideal bathing recommendations as it may improve skin hydration and provide some symptom relief while use of detergents may have a dehydrating effect. Emollients are a cornerstone of treatment and can lead to a decrease in the amount of prescription topical agents needed to treat AD (56). However, it is not known the optimal amount or frequency of emollient application. Additionally, there are some moisturizers that may irritate the skin of individual patients. Besides emollients, topical agents including corticosteroids are first-line therapy. Non-steroidal options such as topical calcineurin inhibitors (TCIs) are useful for areas of sensitive skin such as face, neck, and genitals. Calcineurin inhibitors can also be used as maintenance twice a week to reduce the frequency and severity of flares (57). While there was initial concern regarding the use of TCIs and the risk of malignancy, post-marketing research has been reassuring as to the safety of these treatments (58). In patients who fail topical treatment, phototherapy, oral immunosuppressants, and targeted biologics are indicated. In particular, the anti-IL4 receptor alpha inhibitor dupilumab has changed the way we treat AD in both pediatric and adult patient. While sedating antihistamines for short-term use can assist with sleep disturbance caused by pruritus, there is a lack of evidence to support the use of non-sedating and sedating antihistamines for generalized, extended use. Due to cumulative side effects, oral corticosteroids should be avoided in the long-term and in children. There are many exciting new mechanisms of action in development (or very recently approved) to treat atopic dermatitis including aryl hydrocarbon receptor agonists, commensal bacteria, JAK inhibitors (JAKi), and new biologics that target IL-13, IL-31, IL-33, and OX-40 (59, 60).

Perspectives

Atopic dermatitis is one of the most common inflammatory skin disorders and there are still multiple unmet needs and educational gaps. Instructing patients and caregivers regarding skin hygiene with liberal use of emollients is essential for all. Additionally reassuring fears of corticosteroids is an important task of providers. There is no generally accepted goal of treatment, so currently plans are individualized for patients with a need for biomarkers and research into personalized medicine. Adherence to therapy remains a long-term challenge as care of atopic dermatitis can be quite time consuming and costly. There are an increasing number of therapeutic options that are being developed due to our improved understanding about the pathogenesis of AD and with it, improved hope at helping more patients who suffer from atopic dermatitis.

Psoriasis

Over the past three decades, psoriasis has become a model disease for the study of chronic inflammatory diseases. Several new drugs have been and are being developed first for psoriasis and then extended to other indications (61). Central to our current understanding of the pathogenesis of psoriasis is a close interaction between components of the innate and adaptive immune systems (62, 63). For example, the former branch is represented by macrophages, neutrophilic granulocytes, and (plasmacytoid) dendritic cells; the latter by T lymphocytes, primarily Th17 cells. Communication between these immune cells is mediated by various cytokines including TNFα, IL-17, and IL-23 which have become targets of multiple biologic therapies (64, 65).

Diagnosis

Psoriasis is diagnosed based on history and clinical presentation—only rarely a biopsy is needed to confirm the diagnosis. Comorbidity, especially psoriatic arthritis should be excluded at diagnosis and during follow-up (62). However, psoriatic disease is not a uniform disease entity (Figures 1B,C). Although current drugs were developed and approved for the so-called chronic plaque psoriasis, we encounter psoriasis in the clinic as a spectrum ranging from acute exanthematous to chronic stable, from classically scaly and sharply demarcated plaques to highly inflammatory, pustular or erythrodermic forms, or from forms restricted to a few predilection sites to generalized or inverse forms. Specific underlying genetic patterns have now been identified for some of these manifestations; e.g., in IL36RN (3, 66). The involvement of other organ systems (comorbidity) and provoking factors in psoriasis as a systemic disease also influence the disease process. To account for the increased inflammation throughout the organism with (possible) systemic impairment of several other organ systems, we now tend to refer to it as psoriatic disease.

Treatment

There are well-defined guidelines for the treatment of psoriasis (67, 68). The following drugs are FDA or EMA approved for psoriasis and/or psoriatic arthritis: TNFα inhibitors etanercept, infliximab, adalimumab, certolizumab pegol, and golimumab; IL-17a inhibitors secukinumab and ixekizumab; IL-17A and IL-17F dual inhibitor bimekizumab; IL-17 receptor A/C inhibitor brodalumab; IL-12 and IL-23 inhibitor, ustekinumab; and IL-23 inhibitors guselkumab, tildrakizumab, and risankizumab (69–72). Very recent developments also include mirikizumab and netakimab (73). The development of specific therapeutics against essential cytokines in the IL-23/IL-17 axis is a good illustration of how basic and translational immunological research has led to the development of highly potent drugs that can effectively and safely treat most patients with at least moderate psoriasis. These therapies have been and continue to be included in current guidelines (67, 68, 74, 75). In addition, small-molecule drugs have been and are being developed, such as apremilast against phosphodiesterase 4 (PDE4), deucravacitinib against tyrosine kinase 2 (Tyk2) and piclidenoson, a Gi protein-associated A3 adenosine receptor agonist (73). Compared to the era before biologics, the impact has been so great that it is fair to label these treatments revolutionary. Thus, we are now in a fairly comfortable position with regard to the treatment psoriatic disease: there are now numerous effective and well-tolerated preparations available and due to competitive pressure and the increasing availability of biosimilars, the price will (hopefully) come down in such a way that more and more patients can be treated with these systemic therapeutics. Still, there are still unmet medical needs for psoriasis that related to diagnostics and treatment. Specifically, in terms of personalized and precision medicine, however, there is definitely still a need for development here, since by no means do all of our patients meet the “standard” of chronic plaque psoriasis, which is usually considered in registration studies. On this basis, later extensions of indications are also conceivable for diseases that have similar pathogenetic features and for which, due to their relative rarity, large prospective clinical trials are usually not conducted (76). The categorization and characterization of inflammatory and autoimmunity patterns, which is already under development, may help in this regard (77). There is also not yet enough data on combination therapies for psoriasis (78). In particular, combinations of modern and conventional therapies could in some cases increase effectiveness and reduce costs. Similar considerations apply to individualized dosing regimens and terminations of therapies.

Perspectives

Perspectives

Due to the long delay in diagnosis, the enormous impairment of the quality of life caused by HS and the limited range of evidence-based therapies, patients with HS have an enormous unmet medical need. To change this situation, first and foremost the time interval between first symptoms and diagnosis must be significantly shortened. This is extremely important because of the progressive nature of the disease that over time leads to irreversible skin destruction. To this end, the patients must be pharmacologically treated as soon as the first symptoms appear. Training of doctors such as general physicians, dermatologists, surgeons, and gynecologists, as well as programs to raise/create the awareness of the disease within large parts of the society are needed. It is gratifying that many clinical trials with a focus on new systemic pharmacological treatment are currently being carried out. However, these are often without a well-founded scientific rationale and a better understanding of disease mechanisms is needed. Thus, we need extensive, well-founded translational research into the pathogenesis of the HS as the basis for the development of targeted systemic therapies for HS. A further aspect is a holistic view of the patient to include awareness of systemic inflammation evaluation, treatment of systemic comorbidities and pain, and psychological care for the patient. The last aspect is to motivate and support the patient in changing lifestyle factors that can contribute to the persistence of HS, such as smoking and obesity. Structured patient counseling that provides information about these associations, including referral to smoking cessation programs and weight loss might be helpful.

In a metanalysis of over 1,000 EBA cases, use of the CD20 antibody rituximab or high dose intravenous immunoglobulin G (IVIG) were, compared to all other treatments, more often associated with the induction of remissions (270). These observations are a basis to establish protocols for clinical trials in EBA, evaluating the impact of either rituximab or IVIG. In addition, this also indicates that drugs targeting the B cells, such as the BTK inhibitor PRN1008, or compounds modulating the half-live of IgG, such as FcRn inhibitors, could be also effective in EBA (234). In addition, use of pre-clinical model systems has identified and validated a number of novel therapeutic targets in EBA (272–276). Based on these findings in pre-clinical EBA models, controlled clinical trials are currently performed—albeit in bullous pemphigoid patients (234).

Autoinflammatory Diseases

Cryopyrin-Associated Periodic Syndrome

Cryopyrin-associated periodic syndrome (CAPS) comprises a group of rare diseases that, despite certain clinical similarities, were previously considered separate disorders. These include Familial Cold Urticaria Syndrome (FCAS) which was first described in 1940 (277), Muckle-Wells Syndrome (MWS), and Chronic Infantile Neurologic Cutaneous and Articular or Neonatal Onset Multisystem Inflammatory Disease (CINCA/NOMID). Its prevalence is about 1–2 per million inhabitants in Europe and the USA.These diseases are characterized by an attack-like course with fever episodes lasting up to a few days and an enormous increase of inflammatory laboratory parameters, usually an accompanying urticaria-like exanthema, conjunctivitis, joint and muscle pain as well as hepatomegaly and splenomegaly (Figure 1P). In severe cases, cartilage growth leads to joint dysfunction. In MWS and NOMID/CINCA, central nervous involvement with mental retardation, epilepsy and hearing loss is found. The expression of the disease pattern varies from individual to individual. A long-term complication is the development of AA amyloidosis, which affects multiple organs, most prominently the heart and the kidney. As the cause for these diseases, heterozygous monogenetic deficiency in the NLRP3 (278), NLRP12 (279), PLCG2 (280), and NLRC4 (281) genes have been identified. However, a considerable number of cases are caused by mosaicism in the respective genes, such as in NLRP3 (282).

Diagnosis

The diagnosis is based on a careful history and observation of the clinical course, especially of the inflammatory parameters, as well as genetic testing, preferably requiring NGS panel diagnostics. It may be necessary to assess whether the disease can be influenced by corticosteroids, NSAIDs, and ultimately IL-1b inhibitors in an individual patient. The additional use of clinical scores, such as the EUROFEVER/PRINTO score (283), is helpful, although, given the rarity of the disease, a systematic approach is needed to differentiate it from other diseases. A special difficulty are cases where the disease is caused by somatic mosaicism or by a not yet identified unknown genetic defect. In such cases the diagnosis may not be made satisfyingly, leading to delay in efficient, but often expensive, treatment options. In addition to diagnosis, monitoring of disease activity is also of high importance. While ESR and CRP are routine diagnostics, the measurement of calprotectin levels or amyloid A in the serum, for example, are helpful but much less available. Another problem is the early diagnosis of AA amyloidosis and determination of its extent. Here, nuclear medicine methods such as a PET-CT scan with 18F-florbetaben have been described (284) but are also not yet routine.

Treatment

The IL-1β inhibitors anakinra and canakinumab are approved and available for the treatment of CAPS. However, other cytokines such as IL-18 have been described to be important in autoinflammatory diseases (285, 286). Hence, breakthrough attacks carried by IL-18 are not inhibited by current treatments. On the long run, secondary AA amyloidosis poses a challenge. Although it can be indirectly alleviated by inhibition of IL-1β signaling, targeted resolution of amyloid deposits is not possible to date (287).

Perspectives

Over the past 20 years, genetic defects have been identified for a variety of autoinflammatory diseases. Especially challenging are cases in which no classical germline mutation is present. In such cases, classical genetic methods reach their limits. The development of third generation sequencing methods such as nanopore sequencing with the simultaneous development of bioinformatics and advances in IT infrastructures could provide the solution for these cases as well (288). In addition to inhibition of secondary proinflammatory messengers such as IL-1β, inhibition of NLRP3 by small-molecule agents may also show promise (289, 290). To inhibit the action of IL-18, which is important in addition to IL-1β and plays a role in macrophage activation in particular, a promising drug might be available in the form of IL-18 binding protein (tadekinig alfa) (291).

Schnitzler's Syndrome

Schnitzler's syndrome is a late-onset autoinflammatory diseases that has been described first in 1972 by Schnitzler (292). The disease is characterized by the combination of urticaria-like exanthema (neutrophilic urticarial exanthema) and gammopathy, associated with fever, joint, muscle or bone pain, elevated inflammation markers, morphologic bone changes, hepato-splenomegaly, and palpable lymph nodes (Figure 1Q) (29). It is considered to be a rare entity—with only about 100 patients described in the literature—although a retrospective database search for urticarial exanthema associated with dysproteinemia led to the identification of 16 patients at Mayo Clinic (293), pointing toward a much higher incidence. The etiology and pathogenesis of the disease is unknown. A somatic mutation is assumed (293), which is comparable to the pathogenesis of mastocytosis. In the case of mastocytosis, even low frequencies of mutant cKit that are barely detectable by means of digital PCR can lead to pronounced symptoms (294). The possible causal relationship between gammopathy and Schnitzler syndrome is also unclear.

Diagnosis

The diagnosis of Schnitzler's syndrome should be considered in patients with gammopathy and urticarial exanthema, especially those without itching, increased inflammation markers and fever (295). On the other hand, chronic spontaneous urticaria (CSU) is a much more common diagnosis, which renders the differentiation very difficult. Especially, pressure urticaria may present with systemic symptoms such as fever and myalgia (296). On the other hand, CSU is such a much more common disease than Schnitzler syndrome that not every gammopathy in combination with urticarial exanthema should be misdiagnosed as Schnitzler syndrome. Unfortunately, the diagnosis of chronic urticaria is not established by specific biomarkers that would allow differentiation from other entities including allergic forms. Nevertheless, a lack of response to antihistamines, biologics such as omalizumab, or even corticosteroids may serve as further evidence of an autoinflammatory syndrome. The Strasbourg criteria are helpful in establishing the diagnosis, although they are still considered provisional and specificity and sensitivity have not been adequately determined (297). The dermatohistopathological differentiation between neutrophil-rich infiltrates in Schnitzler syndrome (298), urticarial vasculitis, and urticaria is not straightforwardly possible in practical settings, although a morphologic criterion, neutrophilic epidermotropism, might be specific for autoinflammatory diseases such a Schnitzler's syndrome. Moreover, the gammopathy strictly required by the Strasbourg criteria may be not that absolutely necessary, as cases that appear to be clearly late-onset autoinflammatory diseases may present without it (299, 300). Gammopathy may also develop later in the course of the disease. Hence, establishment of validated diagnostic criteria for Schnitzler's syndrome is needed.

Treatment

IL-1β inhibiting treatment with anakinra and also canakinumab is highly efficient and can lead to resolution of symptoms within a few hours (301). Other treatment modalities such as colchicine, hydroxychloroquine, pefloxacin (29), and IL-6 inhibition with tocilizumab (302) are described. Like CAPS, Schnitzler's syndrome may lead to AA amyloidosis (303). AL amyloidosis due to gammopathy occurs, although rarely (304). Both types are difficult to treat, and no specific amyloid resolving treatment is known.

Perspectives

At the time being, only 9 controlled studies for Schnitzler's syndrome are listed in ClinicalTrials.gov, 5 of which that are using established IL-1β and IL-6 inhibitors have the status of being completed. A novel IL-1β inhibitor with affinity to IL-1α and IL-1Ra is recruiting, and a study that tests the histone deacetylase inhibitor ITF2357 (305) has an unknown status. Drugs that target the inflammasome may be able to prevent the activation of the autoinflammatory cascade (290). A pilot study using the NLPR3 inhibitor dapansutrile is listed as recruiting. Interestingly, a clinical observation of a resolution of Schnitzler's syndrome after haematopoietic stem cell transplantation may hint at the pathogenesis, e.g., a somatic mutation in bone marrow cells (306). This observation may also hint at a similar pathogenic pattern as in systemic mastocytosis, where myeloablative conditioning followed by (allogenic) stem-cell transplantation is used for treatment (307).

Despite the progress made in deciphering the pathogenesis of SSc in recent years, the triggers of the disease and the mechanisms that lead to the heterogeneous disease manifestations and disease severity remain poorly understood. However, since an understanding of these mechanisms is the basis for the identification of key molecules in pathogenesis and thus new therapeutic options, deciphering the disease-driving mechanisms of SSc is urgently needed. A key requirement for this is the enrollment of patients in international registries as well as a close collaboration between patients, clinicians, and scientists.

Perspectives

In conclusion, there are a multitude of challenges for the diagnosis and treatment of chronic skin inflammation. These are, however, different for each disease: At a generalized glance, for the common inflammatory skin diseases, especially psoriasis, atopic dermatitis and lichen planus, disease heterogenicity and the identification of biomarkers that allow to predict treatment responses are at the forefront of the medical needs. We assume that with the advent of more and more detailed molecular data from these patients, a stratification allowing personalized treatment options are on the horizon (63).

For the rare and orphan chronic skin inflammatory diseases, medical practitioners from all specialties need to be made aware of these diagnoses, for example pemphigus or dermatomyositis. Patient organizations, such as the International Pemphigus & Pemphigoid Foundation (IPPF), that educate patients and medical practitioners alike are key for this. This will then also lead to an earlier and more validated diagnosis, which are both essential to start the appropriate treatments. Regarding these, there is a high need to develop more selective, and potentially causal, treatments for chronic skin inflammation. The use of the chimeric antigen receptor (CAR)-T cell technology to selectively deplete autoreactive B cells in pre-clinical models of pemphigus is a milestone in reaching this goal (370).

These differences in unmet medical needs across the here discussed chronic, non-communicable inflammatory skin diseases, do, however, not allow to determine which of these diseases has the “most” or the “highest” unmet medical need. In a generalized manner, one could approach this open issue by a systematic and longitudinal assessment of patient reported outcomes across a wide range of chronic inflammatory skin diseases. This would allow to determine the burden of individual diseases at diagnosis, as well as at a time point where treatment should have had a positive impact on both objective and subjective disease symptoms.

Another challenge that is observed across almost all chronic (skin) inflammatory diseases is comorbidity. At the forefront of these are metabolic syndrome, (cardio)vascular and mental health diseases (348, 371–374). One hypothesis is that chronic skin inflammation drives the associated comorbidity (279, 375). By contrast, others provided evidence that the environment is a key driver for the observed comorbidity in chronic (skin) inflammation (376).

Thus, in perspective, we believe that we will observe significant changes how chronic skin inflammation is diagnosed and treated during the next years. Overall, this will improve the quality of life of patients. We also envision the emerge of curative treatments for those autoimmune skin diseases, where culprit cells can specifically be targeted, i.e., autoreactive B cells.

Author Contributions

RL: conceptualization. KBi (lead), HU, DR, MS, SS, MMe, MMa, DT, ES, CC, KA, DD, MH, AR, HG, AH, AS, GR, GS, JD, DZ, TS, AC, KW, RS, KK, VW, and RL: visualization. All authors: writing–original draft and review and editing. All authors contributed to the article and approved the submitted version.

Funding

This work has been financially supported by the Cluster of Excellence Precision Medicine in Chronic Inflammation (EXC 2167) and the Collaborative Research Center Pathomechanisms of Antibody-mediated Autoimmunity (SFB 1526), the Research Units PruSearch (FOR 2690) and PEGASUS (FOR 2497), the Individual Research Grant RI/1056-11, all from the Deutsche Forschungsgemeinschaft; the Schleswig-Holstein Excellence-Chair Program from the State of Schleswig Holstein, an EADV research fellowship grant 2019, the Department of Veterans Affairs Veterans Health Administration, Office of Research and Development, Biomedical Laboratory Research and Development, National Institutes of Health (National Institute of Arthritis and Musculoskeletal and Skin Diseases) R01AR071653 (VPW) and R01AR076766 (VPW), and Sinergia Unravel principles of self-organization in injured tissue (CRSII5_202301/1) from the Swiss National Science Foundation.

Conflict of Interest

HU has received research grants from JB, Otsuka, Taiho, Boehringer Ingelheim, Kyowa Kirin, Kaken, Sun Pharma, Shionogi, Teijin, Mitsubishi Tanabe, Nihon Zoki, Eisai, Torii and Tokiwa, consultant fees from Ono, Nihon-Pharmaceutical, Sun Pharma, argenx and Ishin Pharma, and speaker's fees from Nihon-Pharmaceutical, Maruho, Eli Lilly, Abbie, Eisai, Sanofi, Janssen, Kyowa Kirin, Ono, UCB, Novartis, Sun Pharma, Torii, Taiho, Mitsubishi Tanabe, and Boehringer Ingelheim during the last 3 years, DR has received honoraria or research support from AbbVie, Abcuro, AltruBio, Amgen, Boehringer-Ingelheim, Bristol Meyers Squibb, Celgene, Concert, CSL Behring, Dermavant, Dermira, Galdrema, Incyte, Janssen, Kyowa Kirin, Lilly, Merck, Novartis, Pfizer, Regeneron, Sanofi, Sun Pharmaceuticals, UCB, and VielaBio during the last 3 years, MS has received consulting/speakers' fees, or grant support from AbbVie, Almirall, Amgen, Biogen, BMS, Janssen, Novartis, and UCB during the last 3 years, SS has received funding and personal fees from Celldex, Clexio, Dermasence, Galderma, GSK, Kiniksa, Menlo, Trevi, Novartis, Sanofi (investigator all), Abbvie, Almirall, Beiersdorf, Bellus Health, Benevolent, Bionorica, Cara, Celgene, CelloHealth, Clexio, DS Biopharma, Eli Lilly, Escient, Galderma, Grünenthal, Kiniksa, Klinge Pharma, Menlo, Sanofi, Sienna, Trevi, P.G. Unna Academy, Perrigo, Pfizer, Vanda, Vifor, WebMD (Consultancy/Advisory board) and Almirall, Eli Lilly, Sanofi, Galderma, Menlo, Omnicuris, Beiersdorf, Leo Pharma, Novartis, P. G. Unna Academy, Pfizer, Pierre Fabre (speaker) during the last 3 years, MMe has received honoraria as a speaker and/or consultant for Amgen, Aralez, argenx, Bayer, Beiersdorf, Celgene, Escient, Galderma, GSK, Menlo, Moxie, Novartis, Pharvaris, Pfizer, Roche, Sanofi, Siennabio, and Uriach, MMa was a speaker and/or advisor for and/or has received research funding from Allakos, Amgen, Aralez, ArgenX, AstraZeneca, Celldex, Centogene, CSL Behring, FAES, Genentech, GIInnovation, Innate Pharma, Kyowa Kirin, Leo Pharma, Lilly, Menarini, Moxie, Novartis, Roche, Sanofi/Regeneron, Third HarmonicBio, UCB, and Uriach during the last 3 years, is a consultant, advisory board member, and/or investigator for AbbVie, Almirall, Amgen, Beiersdorf, BMS, Boehringer Ingelheim, Eli Lilly, Galapagos, Janssen-Cilag, LEO Pharma, MorphoSys, Novartis, Pfizer, Regeneron Pharmaceuticals, Inc., Samsung, Sandoz, Sanofi, Sun Pharma, and UCB, ES has received research grants from UCB, Incyte, Biotest, ArgenX, Dompe, Fresenius Medical Care, Bayer, AstraZenca, and Euroimmun and honoraria from Biotest, Thermo Fisher, ArgenX, Fresenius Medical care, Topas, Leo, Chugai, AstraZenca, and Almirall during the last 3 years, MH has received honoraria from Novartis, Sanofi, Celgene, and unrestricted grants from Biotest, Janssen Cilag and Topas durimg the last 3 years, KBi has received research funds from ArgenX during the last 3 years, DZ has received support for research and development work, lecturing and consulting from Euroimmun AG, UCB Pharma, ArgenX, Biotest, Abbvie, Janssen, Sanofi in the last 3 years, KW has received research grants, travel grants, consulting honoraria or lecturer's honoraria from Amgen, Bristol Myers Squibb, Celgene, Charité Research Organization, Flexopharm, Janssen-Cilag, Novartis Pharma, Sanofi-Aventis, and Trial Form Support during the last 3 years, RS has received research grants or honoraria for participation in advisory boards, clinical trials, or as speaker for one or more of the following: AbbVie, Amgen, Bayer, Boehringer Ingelheim Pharma, Bristol Myers Squibb, Celgene, Charité Research Organization, CSL Behring, Dr. Willmar Schwabe, Flexopharm, Incyte, Janssen-Cilag, La Roche-Posay Laboratoire Dermatologique, Novartis Pharma, Parexel International, Sanofi–Aventis, TFS, and UCB Biopharma during the last 3 years, VW has received grants from Celgene, Amgen, Janssen, Biogen, Gilead, Viela; Horizon therapeutics, Pfizer, Corbus, CSL Behring, consulted Astra-Zeneca, Pfizer, Biogen, Celgene, Resolve, Janssen, Gilead, Lilly, BMS, Nektar, Abbvie, Viela, GSK, EMD Serona, Sanofi, Anaptysbio, Amgen, Merck, Pfizer, Janssen, Neovacs, Idera, Octapharma, CSL Behring, Corbus, Novartis, Romefor during the last 3 years, RL has received honoraria for speaking or consulting or has obtained research grants from Novartis, Lilly, Bayer, Dompe, Synthon, Argen-X, and Incyte during the last 3 years. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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PERMALINK

Unmet Medical Needs in Chronic, Non-communicable Inflammatory Skin Diseases

Hideyuki Ujiie

David Rosmarin

Michael P Schön

Sonja Ständer

Katharina Boch

Martin Metz

Marcus Maurer

Diamant Thaci

Enno Schmidt

Connor Cole

Kyle T Amber

Dario Didona

Michael Hertl

Andreas Recke

Hanna Graßhoff

Alexander Hackel

Anja Schumann

Gabriela Riemekasten

Katja Bieber

Gant Sprow

Joshua Dan

Detlef Zillikens

Tanya Sezin

Angela M Christiano

Kerstin Wolk

Robert Sabat

Khalaf Kridin

Victoria P Werth

Ralf J Ludwig

Abstract

Chronic, Non-Communicable Inflammatory Skin Diseases

Table 1.

Chronic Inflammatory Skin Diseases

Atopic Dermatitis

Diagnosis

Figure 1.

Treatment

Perspectives

Psoriasis

Diagnosis

Treatment

Perspectives

Prurigo Nodularis

Diagnosis

Treatment

Perspectives

Lichen Planus

Diagnosis

Treatment

Perspectives

Hidradenitis Suppurativa

Diagnosis

Treatment

Perspectives

Autoimmune Skin Diseases

Alopecia Areata

Diagnosis

Treatment

Perspectives

Vitiligo

Diagnosis

Treatment

Perspectives

Chronic Spontaneous Urticaria

Diagnosis

Treatment

Perspectives

Pemphigus

Diagnosis

Treatment

Perspectives

Bullous Pemphigoid

Diagnosis

Treatment

Perspectives

Mucous Membrane Pemphigoid

Diagnosis

Treatment