isotretinoin and Acquired-Hyperostosis-Syndrome

Acne vulgaris is a chronic, inflammatory, skin condition that involves the pilosebaceous follicles and is influenced by a variety of factors including genetics, androgen-stimulation of sebaceous glands with abnormal keratinization, colonization with Cutibacterium acnes (previously called Propionibacterium acnes), and pathological immune response to inflammation. Acne can occur at all ages and this discussion focuses on the first three decades of life. Conditions that are part of the differential diagnosis and/or are co-morbid with acne vulgaris are also considered. Acne in the first year of life includes neonatal acne (acne neonatorum) that presents in the first four weeks of life and infantile acne that usually presents between 3 and 6 months of the first year of life with a range of 3 to 16 months after birth. Acne rosacea is a chronic, inflammatory, skin condition that is distinct from acne vulgaris, typically presents in adults, and has four main types: erythemato-telangiectatic, papulopustular, phymatous and ocular. Treatment options for acne vulgaris include topical retinoids, topical benzoyl peroxide, antibiotics (topical, oral), oral contraceptive pills, isotretinoin, and others. Management must consider the increasing impact of antibiotic resistance in the 21st century. Psychological impact of acne can be quite severe and treatment of acne includes awareness of the potential emotional toll this disease may bring to the person with acne as well as assiduous attention to known side effects of various anti-acne medications (topical and systemic). Efforts should be directed at preventing acne-caused scars and depigmentation on the skin as well as emotional scars within the person suffering from acne.

Topics: Acne Vulgaris; Acquired Hyperostosis Syndrome; Administration, Oral; Administration, Topical; Adolescent; Anti-Bacterial Agents; Awareness; Benzoyl Peroxide; Child; Contraceptives, Oral; Dermatologic Agents; Diagnosis, Differential; Female; Hidradenitis Suppurativa; Humans; Isotretinoin; Male; Propionibacteriaceae; Psychological Distress

Acne fulminans (AF) is a rare and severe form of inflammatory acne presenting clinically with an abrupt outburst of painful, hemorrhagic pustules and ulceration, that may or may not be associated with systemic symptoms, such as fever, polyarthritis, and laboratory abnormalities. It typically affects male teenagers with a pre-existing acne. Although the pathogenetic mechanism has not been established yet, a role of genetic, abnormal immunologic response, drugs intake, hormonal imbalance and viral infection, as causal factors, has been identified. AF may occur as a single disease or may be associated with other disorders. Traditionally, AF has been classified, on the basis of the presence of systemic involvement, in "acne fulminans" and acne fulminans "sine fulminans," when no systemic involvement is present. Recently, four clinical variants have been proposed: acne fulminans with systemic symptoms (AF-SS), acne fulminans without systemic symptoms (AF-WOSS), isotretinoin-induced acne fulminans with systemic symptoms (IIAF-SS), isotretinoin-induced acne fulminans without systemic symptoms (IIAF-WOSS). The diagnosis of AF is usually based on clinical history and physical examination. No specific laboratory abnormalities are generally found. In selected cases, biopsy and/or radiologic imaging are helpful for a correct diagnosis. The treatment significantly differs from severe acne according to severity of clinical presentation and possible systemic involvement. Currently, systemic corticosteroids (prednisolone) and retinoids (isotretinoin) represent the first choice of treatment. Dapsone, cyclosporine A, methotrexate, azathioprine, levamisole, and biological agents such as anakinra, infliximab, adalimumab may be considered as alternative therapies in selected cases. Adjunctive topical and physical therapies may also be considered.

Topics: Acne Vulgaris; Acquired Hyperostosis Syndrome; Adolescent; Adrenal Cortex Hormones; Adult; Androgens; Anti-Inflammatory Agents; Arthralgia; Combined Modality Therapy; Debridement; Dermatologic Agents; Diagnosis, Differential; Disease Progression; Female; Humans; Immunosuppressive Agents; Inflammation; Isotretinoin; Lasers, Dye; Low-Level Light Therapy; Male; Photochemotherapy; Propionibacteriaceae; Retinoids; Symptom Assessment; Young Adult

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes both dermatological and rheumatologic symptoms. Being a rare condition, the diagnosis is frequently late. The authors report a case of a 13-year-old boy diagnosed with synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome with unusual severe systemic repercussions. The patient presented with acne conglobata, inability to walk due to pain and weakness and weight loss. Bone scintigraphy was suggestive of sacroiliitis, and lumbar spine x-ray showed signs of hyperostosis. His clinical state improved after treatment with nonsteroidal anti-inflammatory drugs, methotrexate, clindamycin, and isotretinoin. A review of the clinical aspects of this syndrome is presented, emphasizing how this underdiagnosed syndrome can lead to severe weight loss and significant functional and psychological impairment at an early age.

Topics: Acquired Hyperostosis Syndrome; Adolescent; Anti-Inflammatory Agents, Non-Steroidal; Clindamycin; Delayed Diagnosis; Drug Therapy, Combination; Follow-Up Studies; Humans; Isotretinoin; Male; Methotrexate; Multimodal Imaging; Positron-Emission Tomography; Rare Diseases; Risk Assessment; Severity of Illness Index; Treatment Outcome

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome defines an association of inflammatory cutaneous disorders with osteoarticular manifestations and represents a clinical and therapeutic challenge. We report a case of severe SAPHO syndrome with acne conglobata and a diffuse involvement of the anterior chest wall and sacroiliac joints that required treatment with isotretinoin and adalimumab, a new fully human anti-tumor necrosis factor (TNF)-α monoclonal antibody. Combination treatment determined a complete clinical remission of cutaneous and osteoarticular manifestations after 48 weeks. Despite maintenance of clinical remission, follow-up imaging studies after 24 months of adalimumab monotherapy revealed osteoarticular disease progression, with features of inflammatory osteitis. TNFα antagonists have been used as third-line therapy for SAPHO syndrome in single case reports or case series, but these lack consistent long-term follow-up. SAPHO syndrome can present an intermittent-favorable course in the majority of cases as well as a chronic-progressive course, the latter requiring aggressive combination treatment with TNFα antagonists and conventional systemic agents.

Topics: Acquired Hyperostosis Syndrome; Adalimumab; Adult; Anti-Inflammatory Agents; Antibodies, Monoclonal, Humanized; Dermatologic Agents; Humans; Isotretinoin; Male; Tumor Necrosis Factor-alpha

Acne is the most common disease of the skin. It affects 85% of teenagers, 42.5% of men, and 50.9% of women between the ages of 20 and 30 years.96,97 The role of hormones, particularly as a trigger of sebum production and sebaceous growth and differentiation, is well known. Excess production of hormones, specifically androgens, GH, IGF-1, insulin, CRH, and glucocorticoids, is associated with increased rates of acne development. Acne may be a feature in many endocrine disorders, including polycystic ovary disease, Cushing syndrome, CAH, androgen-secreting tumors, and acromegaly. Other nonendocrine diseases associated with acne include Apert syndrome, SAPHO syndrome, Behçet syndrome and PAPA syndrome. Acne medicamentosa is the development of acne vulgaris or an acneiform eruption with the use of certain medications. These medications include testosterone, progesterone,steroids, lithium, phenytoin, isoniazid, vitamins B2, B6, and B12, halogens, and epidermal growth factor inhibitors. Management of acne medicamentosa includes standard acne therapy. Discontinuation of the offending drug may be necessary in recalcitrant cases. Basic therapeutic interventions for acne include topical therapy, systemic antibiotics,hormonal agents, isotretinoin, and physical treatments. Generally, the severity of acne lesions determines the type of acne regimen necessary. The emergence of drug-resistant P acnes and adverse side effects are current limitations to effective acne management.

Topics: Acne Vulgaris; Acquired Hyperostosis Syndrome; Acrocephalosyndactylia; Anti-Bacterial Agents; Behcet Syndrome; Dermatologic Agents; Drug-Related Side Effects and Adverse Reactions; Endocrine System Diseases; Hormones; Humans; Isotretinoin; Low-Level Light Therapy; Phototherapy

SAPHO (synovitis, acne, pustolosis, hyperostosis and osteitis) syndrome is a rare autoinflammatory chronic disorder, presenting with non-infectious osteitis, sterile joint inflammation and skin manifestations including palmoplantar pustolosis and severe acne. It could be often misdiagnosed for its heterogeneous clinical presentation. Treatment is challenging and, due to the rarity of this syndrome, no randomized controlled clinical trials have been conducted. Empirical treatments, including non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, antibiotics and bisphosphonates and disease-modifying anti-rheumatic drugs (DMARDs) could be quite effective. Anti-tumor necrosis factor-alpha (anti-TNF-α) agents and interleukin-1 (IL-1) antagonists have shown promising results in refractory patients. Isotretinoin, commonly used for severe acne, has been rarely described as possible trigger of osteo-articular manifestations, in particular sacroiliitis.. The case of a boy, affected by acne fulminans and depression, who presented with sacroiliitis after a 10-week treatment with isotretinoin is presented. After SAPHO diagnosis, NSAIDs therapy was started but the onset of bilateral gluteal hidradenitis suppurativa required the switch to a TNF-α antagonist (Adalimumab) with the achievement of a good control of the disease. Despite specific therapy with sertraline, the patient continued to complains severe depression.. Our case reports a temporal association between the onset of osteo-articular symptoms and the introduction of isotretinoin, as previously described. However, this timeline is not sufficient to establish a causal role of this drug into the pathogenesis of sacroiliitis. At this regard, further studies are required. The occurrence of hidradenitis suppurativa during SAPHO course supported the introduction of TNF-α blockers with a favourable result, as reported in a few cases in literature. The association between SAPHO syndrome and depressive mood disorders is already reported. Our patient experienced severe depression whose trend seems to be independent from the course of the main disease. Currently, it is not clarified if depression could be considered reactive to the underling disease or if it forms an integral part of the autoinflammatory disorder.

Topics: Acne Vulgaris; Acquired Hyperostosis Syndrome; Adalimumab; Adolescent; Anti-Inflammatory Agents; Depressive Disorder; Dermatologic Agents; Humans; Isotretinoin; Male

Topics: Acne Vulgaris; Acquired Hyperostosis Syndrome; Adolescent; Antirheumatic Agents; Drug Eruptions; Humans; Interleukin 1 Receptor Antagonist Protein; Isotretinoin; Male; Remission Induction; Severity of Illness Index

Topics: Acne Conglobata; Acquired Hyperostosis Syndrome; Adult; Arthralgia; Chest Pain; Drug Therapy, Combination; Etanercept; Humans; Isotretinoin; Male; Taiwan; Tumor Necrosis Factor-alpha

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome represents a spectrum of various dermatologic and musculoskeletal conditions. Thromboses have infrequently been reported in SAPHO syndrome, most often in the subclavian vein. There have been no reported cases of pulmonary emboli associated with SAPHO. We report a case of a young patient with SAPHO syndrome who later presented with extensive iliofemoral deep vein thromboses and seven pulmonary emboli.

Topics: Acquired Hyperostosis Syndrome; Adolescent; Etanercept; Humans; Immunoglobulin G; Isotretinoin; Male; Pulmonary Embolism; Receptors, Tumor Necrosis Factor

We report on a young woman suffering from SAPHO syndrome with back pain and arthritis of the sternoclavicular joints. This inflammatory disorder of the osteoarticular system (synovitis, osteitis, and hyperostosis) is associated with severe acne or palmoplantar pustulosis. The patient was treated with pamidronate, NSAID and physiotherapy which improved the musculoskeletal symptoms completely. The acne was treated with isotretinoin.

Topics: Acne Vulgaris; Acquired Hyperostosis Syndrome; Administration, Oral; Administration, Topical; Adult; Anti-Inflammatory Agents; Back Pain; Calcium Compounds; Combined Modality Therapy; Diagnosis, Differential; Diphosphonates; Dose-Response Relationship, Drug; Female; Follow-Up Studies; Humans; Infusions, Intravenous; Isotretinoin; Naproxen; Pamidronate; Physical Therapy Modalities; Sulfides; Thiosulfates

For 12 years, a 26-year-old man had acne conglobata and a non-suppurative diffuse sclerosing osteomyelitis of the mandible as part of a chronic recurrent multifocal osteomyelitis of the sternum, the pelvic bones, and the femoral head, and aseptic arthritis of the knee, the fibulotalar, and the sternoclavicular joints. This fulfills the formal criteria of the SAPHO syndrome. Repeated surgical and antibiotic treatment combined with hyperbaric oxygen caused partial improvement. Complete relief and partial disappearance of the scintigraphic lesions was achieved with long-term corticosteroids, non-steroidal anti-inflammatory drugs, minocycline, and isotretinoin.

Topics: Acne Vulgaris; Acquired Hyperostosis Syndrome; Adult; Amoxicillin-Potassium Clavulanate Combination; Anti-Inflammatory Agents; Dermatologic Agents; Drug Therapy, Combination; Humans; Isotretinoin; Male; Mandibular Diseases; Osteomyelitis; Prednisolone; Radionuclide Imaging

A patient with arthropathy associated with cystic acne, hidradenitis suppurativa, and perifolliculitis capitis abscedens et suffodiens who showed a dramatic response to isotretinoin is described. This, to our knowledge, is the first report documenting effective treatment of this condition, whose nosologic position with respect to other spondyloarthropathies associated with cutaneous disease is considered.

Topics: Acne Vulgaris; Acquired Hyperostosis Syndrome; Adult; Arthritis; Hidradenitis Suppurativa; Humans; Isotretinoin; Keratolytic Agents; Male; Scalp Dermatoses