isotretinoin and Pyoderma-Gangrenosum

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that may be caused by an adverse drug reaction. We discuss the clinical presentation and outcomes of 52 cases of drug-induced PG reported to date in the literature. We conducted our literature search for case reports of drug-induced PG using keywords on PubMed and Medical Subject Heading (MeSH) terms on MEDLINE and EMBASE. To assess the probability that each case of PG was related to drug therapy, we used the Naranjo criteria. We identified 44 studies in the literature, with a total of 52 cases of drug-induced PG. The mean Naranjo score for cocaine-induced PG (n = 13) was 9.4, indicating a definite adverse drug reaction, while the mean Naranjo scores for isotretinoin (n = 5), propylthiouracil (n = 5), and sunitinib (n = 5) were 6.2, 6.8, and 7.4, respectively, indicating probable adverse drug reactions. Drugs should be considered as a possible triggering event whenever PG is diagnosed, and clinicians should particularly consider this in patients taking isotretinoin, propylthiouracil, or sunitinib, as well as in patients with a history of cocaine use.

Topics: Cocaine; Drug Eruptions; Humans; Immunosuppressive Agents; Indoles; Isotretinoin; Propylthiouracil; Pyoderma Gangrenosum; Pyrroles; Sunitinib; Tumor Necrosis Factor-alpha; Withholding Treatment

A 19-year-old man with nodulocystic acne on baseline was treated with isotretinoin therapy. After 1 month on the medication, he developed pyoderma gangrenosum on his pubis area, arms and legs, and pathergy on a puncture site. Possible underlying diseases were excluded. The patient was started on steroids (prednisone 1 mg/kg/d) and isotretinoin therapy was withdrawn. Later the prednisone was tapered and dapsone 100 mg/daily was initiated. After 10 months of follow-up all lesions had healed and no underlying diseases developed.

Topics: Acne Vulgaris; Dapsone; Follow-Up Studies; Humans; Isotretinoin; Male; Prednisone; Pyoderma Gangrenosum; Risk Assessment; Severity of Illness Index; Treatment Outcome; Young Adult

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

Pyoderma gangrenosum (PG) is an unusual skin condition causing inflammation and sterile ulceration. It may occur in the context of a systemic disease or in otherwise healthy patients following trauma. Treatment is immunosuppression. Surgical debridement may worsen the disease. Post-surgical PG of the breast is rare and in previous reports has occurred within days or weeks of surgery. We report a highly unusual case of PG occurring at an incision site seven years after reduction mammoplasty.

Topics: Adult; Cicatrix; Dermatologic Agents; Drug Therapy, Combination; Female; Glucocorticoids; Humans; Isotretinoin; Mammaplasty; Prednisolone; Pyoderma Gangrenosum; Surgical Wound Infection; Time Factors

Topics: Adrenal Cortex Hormones; Adult; Aged; Anti-Bacterial Agents; Breast Diseases; Colchicine; Diabetes Mellitus, Type 2; Drug Resistance; Female; Granuloma; Humans; Hypertension; Immunosuppressive Agents; Isotretinoin; Male; Pyoderma Gangrenosum; Remission Induction; Tacrolimus

Topics: Aged; Dermatologic Agents; Humans; Isotretinoin; Male; Pyoderma Gangrenosum; Treatment Outcome

Topics: Acanthosis Nigricans; Acne Vulgaris; Adult; Dermatologic Agents; Female; Humans; Hyperandrogenism; Insulin Resistance; Isotretinoin; Pyoderma Gangrenosum; Syndrome

A 19 year old man with severe acne conglobata and ulcerated pyoderma gangraenosum-like skin lesions on the face was first treated with isotretinoin (Roaccutan((R))), then immunosuppressively with prednisolone, diaminodiphenylsulfone (Dapson-Fatol((R))) and mycophenolate mofetil (Cellcept((R))). Under a daily maintenance dose of immunosuppressive treatment with 2.5 mg prednisolone and 1 g mycophenolate mofetil, weakness, muscle and joint aches appeared. Due to proteinuria, haematuria and an elevated antineutrophil cytoplasmic antibody (cANCA) and the histological detection of a leukocytoclastic vasculitis, the diagnosis of cANCA positive vasculitis of the skin and kidneys was established. Therapy with cyclophosphamide (Endoxan((R))) along with prednisolone was effective. An exact classification of this disease process was not possible.

Topics: Acne Vulgaris; Adult; Antibodies, Antineutrophil Cytoplasmic; Cyclophosphamide; Diagnosis, Differential; Drug Therapy, Combination; Endothelium, Vascular; Facial Dermatoses; Humans; Immunosuppressive Agents; Isotretinoin; Kidney; Male; Neutrophils; Prednisolone; Pyoderma Gangrenosum; Vasculitis

Topics: Adolescent; Drug Eruptions; Humans; Isotretinoin; Keratolytic Agents; Male; Pyoderma Gangrenosum