isotretinoin and Ulcer

For over two decades, the acronym PAPA syndrome has been used to describe an autoinflammatory condition caused by missense mutations in the PSTPIP1 (proline-serine-threonine phosphatase interacting protein 1) gene and clinically characterized by the presence of pyogenic arthritis, pyoderma gangrenosum (PG), and acne (1,2). Due to the involvement of the PSTPIP1 gene in the regulation of innate immunity, mutations of this gene cause abnormal activation of inflammasomes, complexes of NLRP3/ASC/caspase-1 proteins. As a result, production of interleukin-1β, a key molecule that triggers synthesis of cytokines necessary for the recruitment of neutrophils, is significantly increased (2,3). Additionally, the levels of other pro-inflammatory cytokines, such as tumor necrosis factor-α (TNF-α), interferon-γ (INF-γ) and interleukin 17 (IL-7) are also elevated, which further disrupts inflammatory mechanisms in the microenvironment (4). Since hyperproduction of IL-1 and other involved cytokines is the predominant event in the pathogenesis, these molecules are promising targets in the treatment of PAPA syndrome. Corticosteroids and biologics are currently the most commonly used agents for inducing and hastening remission of symptoms (5). A substantial step forward in the treatment of PAPA syndrome has been the introduction of medications blocking the cytokines crucial in the pathogenesis of this disorder, with TNF-α and IL-1 inhibitors being the most frequent choice of such biological therapy (6). We report the case of a 22-year-old male patient with PAPA syndrome who was referred to our department 18 months ago due to exacerbation of skin changes. Initial presentation and subsequent evolution of disease in this patient matched the typical clinical pattern of PAPA syndrome. The first symptoms occurred at the age of two in the form of unspecific joint disease that was diagnosed as juvenile idiopathic arthritis. Subsequently, in the early adolescence the patient presented with new skin changes manifesting as severe acne and persistent pyoderma gangrenosum-like ulcers. At the same time, severity of joint involvement gradually decreased. After the characteristic phenotype of the disease had fully developed, suspicion of possible syndromic origin of symptoms arose. For this reason, genetic analysis was performed as requested by attending pediatricians at the University Clinical Center in Sarajevo, and E250Q mutation of the PSTPIP1 gene was detected. Thus, the diagnosis of PAP

Topics: Acne Vulgaris; Adalimumab; Adolescent; Adrenal Cortex Hormones; Adult; Biological Products; Cicatrix; Cytokines; Etanercept; Humans; Immunologic Factors; Infliximab; Interleukin 1 Receptor Antagonist Protein; Isotretinoin; Male; Prednisone; Pyoderma Gangrenosum; Quality of Life; Retrospective Studies; Tumor Necrosis Factor-alpha; Ulcer; Young Adult

Acne fulminans is a severe form of acne with a sudden onset, rapid ulceration of the lesions, and some systemic symptoms, with or without systemic symptoms. These papulopustular lesions, whose pathogenesis is not fully understood, may exacerbate with systemic isotretinoin or may be a component of some autoinflammatory syndromes. It is recommended to control these sudden pustular attacks with systemic steroids or anti-inflammatory drugs. Here, in 12 patients with acne fulminans who developed exacerbation during systemic isotretinoin treatment and whose cytological examination revealed hemophagocytosis, colchicine treatment was started in addition to low-dose isotretinoin treatment, and a very good response was obtained to this treatment approach that did not contain systemic corticosteroids.

Topics: Acne Vulgaris; Anti-Inflammatory Agents; Colchicine; Humans; Isotretinoin; Ulcer

Topics: Adult; Dermatologic Agents; Esophageal Diseases; Female; Humans; Isotretinoin; Ulcer

Acne fulminans is an ulcerative form of acne with an acute onset and systemic symptoms. It most commonly affects adolescent boys.. Clinical and laboratory findings and treatment results of patients with acne fulminans were reviewed to obtain a better understanding of the clinical course and outcome of the disease.. Data of patients with severe acne were collected from the Dermatology Departments of Finnish hospitals during the years 1970 to 1991.. Twenty-four patients with acne fulminans are described. All patients had ulcerative acne with acute onset. In 22 patients acne was associated with high fever for at least 1 week. All patients had musculoskeletal pain. Increased uptake in bone scan or radiographic findings compatible with an infectious origin were detected in 17 patients. Eight patients were treated with antibiotics alone, but the response was poor; three patients had a relapse of musculoskeletal symptoms. Ten patients were given systemic steroids in addition to antibiotics. In this group the response was rapid, but acne and musculoskeletal symptoms tended to relapse when the steroid dosage was reduced. Four patients were treated with a combination of antibiotics, systemic steroids, and isotretinoin; all responded well, but one of these patients also had a relapse.. Musculoskeletal symptoms are common in patients with acne fulminans. Systemic steroid treatment rapidly controls the skin lesions and systemic symptoms. The duration of steroid treatment should be 2 to 4 months to avoid relapses. Therapy with isotretinoin, antibiotics, or both was often combined with steroids, but the role of these agents is still uncertain.

Topics: Acne Vulgaris; Adolescent; Adult; Anti-Bacterial Agents; Anti-Inflammatory Agents, Non-Steroidal; Blood Sedimentation; Dermatitis, Atopic; Female; Fever; Glucocorticoids; Humans; Isotretinoin; Joints; Leukocytosis; Male; Muscles; Osteolysis; Pain; Retrospective Studies; Tetracycline; Ulcer