Folliculocentric tinea versicolor: a case report.

BACKGROUND: Tinea versicolor is a very common condition. We reported a specific follicular manifestation and proposed that this particular presentation might be related to the patient's history of previous keratosis pilaris. CASE PRESENTATION: A 46-year-old Asian woman of Han ethnicity presented to the clinic with trunk lesions for over a year. On physical examination: multiple light brown patches of varying size centered on hair follicles in the axillae and trunk, with the patches on the back fusing together and scales visible on the surface of the patches. Finally, through fungal microscopy and pathological examination, the patient was diagnosed with folliculocentric tinea versicolor. CONCLUSIONS: Follicular tinea versicolor is a rare type of tinea versicolor. It is still not clear what causes tinea versicolor to become folliculocentric. This case may suggest that patients with a history of keratosis pilaris may have a tendency to develop follicular centration in the course of other diseases.

Pityriasis Rubra Pilaris in an Atopic Dermatitis Patient: A Case Report.

Pityriasis rubra pilaris (PRP) is a rare, chronic, inflammatory papulosquamous skin disease. Here, we report a case of a 43-year-old female with a known history of atopic dermatitis, bronchiectasis, and goiter who presented with a six-month history of persistent itchy skin lesions on her extremities. A skin examination revealed multiple diffuse, well-defined, fine, scaly erythematous patches with areas of spared skin over all four extremities, along with palmoplantar keratoderma. The trunk was spared. The differential diagnosis included atopic eczema, pityriasis rubra pilaris, dermatomyositis, mycosis fungoides, parapsoriasis, psoriasis, and drug-induced eczematous dermatitis. A skin biopsy revealed hyperkeratosis, acanthosis, spongiosis, follicular plugging, and mild perivascular lymphohistiocytic cellular infiltrates in the dermis. Based on the clinicopathological findings, the patient was diagnosed with atypical adult pityriasis rubra pilaris (PRP) (type II). She was started on narrowband UVB phototherapy (NBUVB). Two months after starting NBUVB treatment, all the skin lesions had cleared. She was put under periodic follow-up, and the lesions have remained clear for two years up to the time of this publication.

Tofacitinib for Pityriasis Rubra Pilaris: A Case Report.

Purpose: At present, we have entered the era of using biological agents and small molecule targeted drugs to treat diseases. Although there have been many reports of biological agents treating pityriasis rubra pilaris recently, the clinical application of the JAK inhibitors in the treatment of pityriasis rubra pilaris has been rarely reported, and there is a lack of evidence on the safety and efficacy of these drugs. We explore the use of the JAK inhibitor tofacitinib in the treatment of pityriasis rubra pilaris with significant efficacy and no significant side effects, providing new ideas for the clinical treatment of pityriasis rubra pilaris. Methods: We cover a case of pityriasis rubra pilaris treated with the JAK inhibitor tofacitinib, which showed significant efficacy without any adverse effects. Results: This case report showed that the JAK inhibitor tofacitinib had significant clinical efficacy in the treatment of pityriasis rubra pilaris. We speculated that the treatment of pityriasis rubra pilaris with the JAK inhibitors may be related to blocking the activation of the JAK/STAT pathway, thereby blocking the high expression of cytokines IL-17, IL-12/IL-23, IL-23, TNF-α. Conclusion: The JAK inhibitor tofacitinib can become a new option for treating pityriasis rubra pilaris.

Keratosis Follicularis Spinulosa Decalvans Associated With Woolly Hair: A Case Report.

Keratosis follicularis spinulosa decalvans X-linked (KFSDX) is part of the spectrum of a rare disorder known as keratosis pilaris atrophicans. Here, we report the case of a 14-year-old boy who presented with a history of abnormal hair since birth. He also had a history of skin lesions and hair loss. There was no similar condition in the family, and the parents were not consanguine. Scalp examination revealed woolly hair, a solitary scarring alopecia patch, and follicular papules. There were also patches of scarring alopecia on the lateral portion of the eyebrows and whole eyelashes bilaterally. His nose showed multiple, skin-colored, non-scaly follicular papules. The differential diagnosis included lichen planopilaris, Graham Little-Piccardi-Lassueur syndrome, KFSDX, keratosis follicularis spinulosa decalvans, and structural hair anomalies. Hair examination under light microscopy was normal. Skin biopsy from the follicular papule on the nose revealed follicular plugging with normal epidermis and dermis. Based on the above clinicopathological findings, the patient was diagnosed with KFSDX associated with woolly hair. He was reassured, but he did not show up for further treatment during the follow-up.

Efficacy and safety of non-cross-linked hyaluronic acid compound in the treatment of keratosis pilaris: A split-body randomized clinical trial.

BACKGROUND: Keratosis pilaris (KP) is a prevalent benign dermatological condition characterized by small bumps at the hair follicles alongside surrounding redness, significantly impacting both aesthetics and mental well-being. OBJECTIVE: This study investigated the potential benefits of a non-cross-linked hyaluronic acid (HA) compound for treating KP. METHODS: A split-body, investigator-blinded, randomized, intraindividual comparative clinical trial was conducted. The non-cross-linked HA compound was injected into KP-affected regions on both upper arms. The treatment was delivered across four sessions scheduled at 4-week intervals. Blinded physicians and patients assessed differences in erythema, skin roughness, and overall scores between treated and control areas at the final follow-up visit. At the 12th and 24th weeks post-treatment, a four-point scale was utilized to assess subjects' perceived treatment efficacy. Additionally, dermoscopic images, histological alterations, and adverse events were monitored. RESULTS: Physician assessments revealed a significant reduction in roughness and overall scores for treated areas compared to controls. Patient self-assessments also reflected improvements in roughness, redness, and overall scores for treated sides at the final visit, with 35.71% of patients demonstrating sustained improvement in redness and 71.43% reporting persistent improvements in roughness at 24th weeks post-treatment. The dermatoscopic examinations revealed a notable enhancement in both the quantity of follicular plugs and the extent of erythema among the subjects in the treatment group. Histopathological outcomes also demonstrated improvement. CONCLUSION: This study suggests that the non-cross-linked HA compound effectively improves skin roughness and promotes hair shaft growth in KP treatment, demonstrating a favorable safety profile. These findings position it as a potentially viable alternative therapy in clinical practice.

Emerging Role of Biologic Drugs Targeting IL-17 and IL-23: Pityriasis Rubra Pilaris.

Pityriasis rubra pilaris (PRP) is a rare, papulosquamous, inflammatory skin disease. PRP represents a therapeutic challenge. The rarity of this disease and its possible spontaneous remission makes the conduction and interpretation of therapeutic studies particularly difficult. Moreover, PRP not infrequently proves resistant to common topical and conventional systemic therapies. In this context, numerous biologic agents have been reported in PRP treatment. The aim of our manuscript was to review the current literature to evaluate the possible role of biologics targeting the IL17/23 axis in PRP management. Recent cases in the literature have highlighted the use of several promising drugs: IL-17 inhibitors, IL-23 inhibitors, and the IL-12/23p40 inhibitor ustekinumab. However, it should be noted that all these drugs are approved for moderate-to-severe plaque psoriasis and their use in PRP is off label. The treatment of PRP is based on clinical experience, case reports or case series reported in the literature, as randomized controlled trials are difficult to conduct due to the rarity of the condition. Despite data on the efficacy of drugs targeting IL-17 and IL-23 being promising, they are still limited. Certainly, further studies are desirable to better characterize PRP and establish shared guidelines.

Pityriasis Rubra Pilaris - a difficult path to optimal treatment. Case report.

Pityriasis Rubra Pilaris is a rare, chronic inflammatory dermatosis of unknown etiology, presenting with erythema and papular eruptions. Treatment is difficult due to the lack of causal therapy, guidelines and requires an individualized approach. The most common treatments are systemic retinoids, immunosuppressants, phototherapy and biological therapy. This article presents the case of a 73-year-old man suffering from type 1 pityriasis rubra pilaris. The patient was initially treated with acitretin, which was discontinued due to hypogammaglobulinemia. This rare side effect of acitretin has not been previously published. As a second-line treatment, the patient received methotrexate, but with no clinical improvement after 3 months and an increase in skin pruritus. Finally, the use of isotretinoin resulted in significant clinical improvement and was well tolerated.

Severe pityriasis rubra pilaris complicated with Kaposi's varicelliform eruption and cutaneous MRSA infection case report.

A 62-year-old woman presented to our hospital with erythroderma affecting 100 % of body surface area, skin scaling and a body temperature of 37.3o C. The lesions initially appeared on her scalp 6 months prior, then psoriasis was diagnosed. Topical corticosteroids were prescribed, which were ineffective. After 2 months the rash spread to the rest of the body, accompanied by nail changes and hair loss. The patient was subsequently admitted to the local hospital, where following clinical evaluation, oral methotrexate 10 mg once weekly was initiated for 6 weeks. Despite the administered treatment the patient's health and skin condition deteriorated, manifesting with an appearance of new lesions. By the time of admission to our hospital erythroderma affecting 100 % of body surface area covered with wide skin scales and punched-out erosions on the torso, lower eyelid ectropion, loss of scalp hair and thickened yellow nail plates were observed. Skin biopsy revealed histological changes consistent with pityriasis rubra pilaris diagnosis. Polymerase chain reaction test from erosions confirmed the presence of herpes simplex virus 1/2 and culture results identified methicillin-resistant Staphylococcus aureus. Given the considerations of pityriasis rubra pilaris, hematologic disorders and paraneoplastic syndrome, a comprehensive work-up for haematological and oncological disorders was conducted, which yielded no significant findings. The patient was treated with intravenous corticosteroids, antibiotics, and antiviral drugs. Isotretinoin was initiated following the histological confirmation of pityriasis rubra pilaris. By the time of discharge, the patient's condition improved. During a follow-up visit 43 weeks after the initiation of isotretinoin, the skin was almost clear. The described case highlights the rare possibility of developing Kaposi's varicelliform eruption in patients with pityriasis rubra pilaris and demonstrates that isotretinoin is a safe and effective treatment option for this condition.

Rapid clearance of extensive juvenile pityriasis rubra pilaris with ixekizumab.

Juvenile pityriasis rubra pilaris is a rare inflammatory skin disorder currently without any FDA-approved treatments, and lesions can be refractory to conventional treatment with topical corticosteroids, methotrexate, and oral retinoids. We herein present a case of a 6-year-old boy who attained clearance of extensive juvenile pityriasis rubra pilaris within 2 weeks of starting ixekizumab therapy. Therapeutic effect has been durable at 6 months, and patient continues on therapy without adverse effects. Our case highlights a new, rapidly effective treatment option for pediatric patients with this rare condition.

Role of IL-23 inhibitors including risankizumab and guselkumab in the treatment of pityriasis rubra pilaris.

Pityriasis rubra pilaris (PRP) is a rare and chronic inflammatory dermatologic condition characterized by hyperkeratotic salmon-colored plaques and palmoplantar keratoderma. Traditional therapeutic modalities have shown limited efficacy and often entail potential adverse effects, highlighting the need for alternative treatment options. Our review aims to summarize the current evidence on the off-label use of IL-23 inhibitors, risankizumab and guselkumab, in the treatment of PRP. These biologic agents have been approved for psoriasis, and their potential role in managing PRP has recently garnered interest. We conducted a comprehensive literature search on PubMed and Scopus databases, identifying relevant studies published in English up to June 2023 following PRISMA guidelines. A total of 10 studies were selected for data extraction and review. Results from the selected studies demonstrated encouraging outcomes with both risankizumab and guselkumab in managing PRP. Among 11 patients treated with risankizumab, 10 showed notable improvements in various disease manifestations, including pruritus, erythema, and affected body surface area. DLQI scores and BSA percentages reported a significant improvement before and after risankizumab treatment (p = 0.0322; p = 0.0216). However, two cases also reported symptom aggravation or even disease worsening. Patients treated with guselkumab exhibited ultimate improvement in all five cases, with complete clearance in three out of five cases. DLQI and BSA percentages also reported significant improvement with treatment with guselkumab (p = 0.0172; p < 0.0001). While most cases demonstrated positive outcomes, there were isolated instances of worsening symptoms, emphasizing the need for caution and further investigation. Further research with larger sample sizes and longer follow-up periods is necessary to establish the efficacy, optimal dosing, and long-term safety of risankizumab and guselkumab in treating PRP. Overall, we provide valuable insights into the potential use of IL-23 inhibitors, risankizumab, and guselkumab, as promising treatment options for PRP. These biologics have shown efficacy in improving symptoms in treatment-resistant cases, offering new avenues for clinicians to explore in the treatment of PRP.

Post-infectious pityriasis rubra pilaris in pediatric patients. / Pitiriasis rubra pilaris posinfecciosa en pacientes pediátricos.

Pityriasis rubra pilaris (PRP) is a rare skin condition. The etiology of PRP is unknown; however, it has been associated with infections, autoimmune diseases, and neoplasms. Here we describe the cases of 2 pediatric patients with PRP triggered by a respiratory syncytial virus infection concurrently with obstructive bronchial syndrome. PRP resolved after treatment with topical emollients, topical corticosteroids, and calcineurin inhibitors.

La pitiriasis rubra pilaris (PRP) es una enfermedad dermatológica poco frecuente. Se desconoce su etiología, sin embargo, se ha asociado a infecciones, enfermedades autoinmunes y neoplasias. Se describen los casos de dos pacientes pediátricos que presentaron PRP gatillada por una infección por virus sincicial respiratorio mientras cursaban un síndrome bronquial obstructivo. Los cuadros de PRP remitieron luego del tratamiento tópico con emolientes, corticoesteroides tópicos e inhibidores de la calcineurina.

Navigating Diagnostic Uncertainty: Frontal Fibrosing Alopecia Versus Keratosis Pilaris Atrophicans Faciei With Genetic Testing Insights.

Keratosis pilaris atrophicans faciei (KPAF) and frontal fibrosing alopecia (FFA) present diagnostic challenges due to their similar clinical characteristics. Dermatologists often employ overlapping treatment regimens, which may hinder accurate diagnosis and treatment expectations. Genetic testing offers promise for precise diagnosis and tailored treatment strategies, yet its utility in these conditions remains underexplored. This manuscript presents a unique case study of a 36-year-old male with symptoms of both KPAF and FFA, who underwent genetic testing. Despite testing negative for this mutation, the case underscores the potential of genetic testing to enhance diagnostic accuracy and optimize treatment outcomes.

Two Incidental Sibling Diagnoses of Netherton Syndrome in Separate Visits: A Case Report.

Netherton syndrome (NTS) is a genetic disorder that predominantly affects the hair and the skin, and it can have a wide variety of presentations. The genetic syndrome is more common with consanguineous parents. Given the rarity and varying presentation of the condition, a few cases have been reported in the literature. We present an unusual case of two incidental diagnoses of NTS in siblings of consanguineous parents, manifesting as erythroderma and other symptoms that were initially diagnosed as pityriasis rubra pilaris and psoriasis in separate visits. Physicians must maintain a high index of suspicion when faced with chronic skin conditions and hair shaft abnormalities that may have been present since childhood to avoid the sequela of inadvertent prolonged or misdiagnosis.

Diseases categorized as autoinflammatory keratinization diseases (AiKDs), and their pathologies and treatments.

Whole-exome and whole-genome sequencing have become widespread in approximately the last 15 years, and the predisposing factors and pathomechanisms of inflammatory keratinization diseases, which have been unknown for a long time, have gradually been revealed. Hence, various inflammatory keratinization diseases are recognized to cause innate immunity hyperactivation. Therefore, we have been advocating for the clinical entity, "autoinflammatory keratinization diseases (AiKDs)" since 2017. AiKDs are inflammatory keratinization diseases caused by autoinflammatory-related pathomechanisms in the skin. The aberrant activation of innate immunity and the resultant autoinflammation in the epidermis and the superficial dermis in AiKDs cause hyperkeratosis in the epidermis. Our initially proposed concept of AiKDs included generalized pustular psoriasis and related conditions, pityriasis rubra pilaris type V, and familial keratosis lichenoides chronica. Since then, the number of diseases known to be AiKDs has increased as previously unknown disease-causing factors and pathogenetic mechanisms of inflammatory keratinization diseases have been clarified one by one. To date, porokeratosis, hidradenitis suppurative, keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK) syndrome, and AiKDs associated with epidermal growth factor receptor (EGFR) deficiency or with hepatitis and autism have been recognized as AiKDs. The concept of AiKDs is considered extremely useful in our precise understanding of the pathogeneses behind inflammatory keratinization diseases and our appropriate treatment method selection. The number of AiKDs is expected to grow with the clarification of the pathomechanisms of further inflammatory keratinization diseases.

Biologics for Treatment of Pityriasis Rubra Pilaris: A Literature Review.

OBJECTIVE: To describe the published efficacy and adverse event rates associated with existing biologics for the treatment of pityriasis rubra pilaris (PRP). DATA SOURCES: A literature review using the PubMed database (January 1990-July 2023) was conducted. Multiple search combinations were conducted using "pityriasis rubra pilaris" and various biologics as keywords to identify relevant articles. STUDY SELECTION AND DATA EXTRACTION: Inclusion criteria included all study types that were published within the past 30 years in English and mentioned at least one biologic and PRP. A preliminary search yielded a total of 499 results. After screening using inclusion and exclusion criteria, 77 relevant articles (69 case reports, 5 case series, 2 clinical trials, and 1 retrospective analysis) were analyzed. DATA SYNTHESIS: TNF-α inhibitors have been evaluated and are effective in treating PRP. However, recent treatment with anti-interleukin (IL)-17 and anti-IL-23 therapies such as ustekinumab, secukinumab, and ixekizumab are emerging as new treatment options with a mean improvement in PRP Area and Severity Index scores, change in severity of erythema, scaling, and thickness of PRP lesions. From initial clinical trials, secukinumab and ixekizumab are promising treatment options for achieving remission. RELEVANCE TO PATIENT CARE AND CLINICAL PRACTICE: This review compares the efficacy for numerous biologics and a discussion to guide clinicians on benefits and risks in choosing a biologic for PRP patients. CONCLUSIONS: Biologics may be a favourable treatment option leading to greater patient adherence due to reduced dosing frequencies, improvement in quality of life, and reduction in frequency and severity of flares.