Generalized Pustular Psoriasis and Systemic Organ Dysfunctions.

This review explores the intricate relationship between generalized pustular psoriasis (GPP) and various systemic diseases, shedding light on the broader impacts of this severe skin condition beyond its primary dermatological manifestations. GPP is identified as not only a profound contributor to skin pathology but also a significant risk factor for systemic diseases affecting cardiovascular, hepatic, renal, pulmonary, and skeletal systems, as well as associated with an increased incidence of anemia, depression, anxiety, and arthritis. The research highlights the complex interplay of cytokines, particularly IL-17 and IL-36, which are central to the pathophysiology of GPP and implicated in the exacerbation of systemic conditions. Key findings indicate a higher incidence of cardiovascular events in GPP patients compared to those with other severe forms of psoriasis, notably with a stronger correlation between myocardial infarction history and GPP development. Liver disturbances, frequently reversible upon psoriasis remission, suggest a cytokine-mediated link to hepatic health. Renal dysfunction appears elevated in GPP sufferers, with IL-17 and IL-36 potentially driving renal fibrosis. Similarly, interstitial lung disease and osteoporosis in GPP patients underscore the systemic reach of inflammatory processes initiated in the skin. The associations with anemia, depression, anxiety, and arthritis further complicate the clinical management of GPP, requiring a multidisciplinary approach. The study concludes that managing GPP effectively requires a holistic approach that addresses both the cutaneous and systemic dimensions of the disease, advocating for continued research into the mechanisms that connect GPP with broader health implications to refine therapeutic strategies.

Spesolimab in the Management of Generalized Pustular Psoriasis With Concurrent Bullous Pemphigoid and Psoriasis.

Autoimmune diseases often co-occur due to shared immunological mechanisms, necessitating strategic treatment approaches to manage overlapping conditions without exacerbating each other. A 75-year-old male with a history of psoriasis vulgaris and bullous pemphigoid (BP) developed new-onset pustular psoriasis under systemic corticosteroid therapy, which is known to potentially worsen psoriasis into its pustular form. Histological examination confirmed the diagnosis, showing features typical of pustular psoriasis. The patient was successfully treated with spesolimab, an anti-IL-36 neutralizing antibody, achieving complete remission without aggravating the BP. This case highlights the necessity of cautious treatment selection in patients with multiple autoimmune disorders and underscores the potential role of IL-36 in exacerbating inflammatory responses in BP. Further research into the interaction between IL-36 and BP may provide deeper insights into managing such complex clinical scenarios.

A Case of Pan-TRK Positive Dermatofibrosarcoma Protuberans Located on the Nose.

Dermatofibrosarcoma protuberans (DFSP) is a rare and infiltrative soft tissue tumor. Our report details a distinctive case of DFSP with pan-TRK positivity in the right nasal dorsum of a 46-year-old female. Histological analysis identified NTRK fusion gene involvement in this patient, detectable through pan-TRK immunostaining. The case underscores the significance of comprehensive management for pan-TRK-positive DFSP in challenging facial locations, indicating the potential efficacy of TRK inhibitors.

Congenital Langerhans Cell Histiocytosis With the Skin and Lung Involvement: A Case and Literature Review.

Langerhans cell histiocytosis (LCH) is a clonal proliferative disease of immature Langerhans cells that expand in various organs, leading to organ and tissue dysfunction. Although LCH is most commonly seen in children under the age of three, a small number of cases of congenital LCH have been described. With a review of the literature on congenital LCH with lung and skin lesions, we present a case of congenital LCH with involvement of skin and lung, which was effectively treated with chemotherapy without recurrence for 3 years during the observational period. In addition, we summarized previously published case studies of congenital LCH with skin and lung involvement.