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In idiopathic cases, topical and oral corticosteroids, ultraviolet light therapies, and immunosuppressive/immunomodulating therapies have been used with variable results. Future studies are needed to further understand the disease process and to establish guidelines for diagnostic workup and treatment.Hidradenitis suppurativa (HS) is a chronic inflammatory disease usually involving the major skin folds characterized by a multifactorial pathogenesis and a wide spectrum of clinical manifestations. It can also rarely present in association with other diseases as complex clinical syndromes, causing additional diagnostic and therapeutic challenges. Different etiopathologic factors contribute to follicular inflammation and suppurative lesions of syndromic HS, including follicular hyperkeratinization and plugging, as well as activation of autoinflammatory pathways. Patients with syndromic HS frequently have a severe disease course, presenting with atypical skin involvement, signs of systemic inflammation, and resistance to conventional treatments. Systematic classification of syndromic HS is based on clinical, pathogenetic, and genetic factors, but it is constantly evolving due to increased disease awareness. Treatment of syndromic HS is difficult and should be personalized on a case-by-case basis. Investigating syndromic HS can lead to useful insights on genetics and pathogenesis, translating into new clinical approaches for sporadic hidradenitis. We review the classification, clinical presentation, disease associations, and therapeutic management of syndromic HS, focusing mainly on its autoinflammatory syndromes PASH, PAPASH, PsAPASH, and PASS.Cutaneous adverse drug reactions produce a significant clinical, financial, and psychological burden on our healthcare industry. The importance of considering a drug reaction in the cause of any dermatitis is underscored by the diversity of clinical manifestations and the prolific rate of drug discovery and approval. We present an update on the variety of drug reactions encountered in the inpatient and outpatient setting. Immunomodulatory drugs used in oncology will be reviewed separately as their clinical manifestations cross many reaction patters and morphologies.Cutaneous dermal mucinoses are a group of conditions characterized by abnormal deposition of mucin (hyaluronic acid and sulfated glycosaminoglycans) in the dermis. They can be classified either as localized or generalized forms and occur primarily or secondarily to systemic disorders. Obesity-associated lymphedematous mucinosis and pretibial stasis mucinosis are uncommon and relatively newly recognized disorders occurring in obese patients or in patients with venous insufficiency. Clinically, patients present with papules and nodules progressing to plaques and arising in an erythematous and edematous basis on the legs, especially the shins. Histopathologic examination indicates mucin deposition in the superficial portion of the dermis, angioplasia with an increase in small blood and/or lymphatic vessels, vertically running vessels, slight fibrosis, and no inflammation. Laboratory workup and histopathologic testing are helpful in differentiating these entities from pretibial myxedema (thyroid dermopathy), which represents the main differential diagnosis. Obesity-associated lymphedematous mucinosis and stasis mucinosis are not different entities but belong to a spectrum of mucinoses secondary to an increased body mass index, chronic lymphedema, and/or chronic venous insufficiency.Idiopathic lipoatrophic panniculitis of children is a rare disease of childhood, characterized by repeated attacks of tender subcutaneous nodules followed by the development of permanent lipoatrophy, often seen on the arms and legs, in association with fever, malaise, and other less common clinical manifestations such as abdominal pain or arthralgia. selleck kinase inhibitor The pathogenesis is unknown, and autoimmune origins, chromosomal alterations, and other causes have been proposed. The nosology of this condition is confusing in the literature, and the precise diagnosis still relies on a combination of clinicopathologic and laboratory assessments. Methotrexate may be recommended to minimize disease progression and morbidity.Adenopathy and an extensive skin patch overlying a plasmacytoma (AESOP) syndrome, first described 16 years ago, is characterized by a slowly expanding red or brown patch (classic variant) or plaque (morphea-like variant) overlying a solitary plasmacytoma of the bone. Early recognition can be life-saving, as it is an early presentation of polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome, which may be fatal. There are two forms the classic variant, which presents as a smooth, shiny macule with abnormal visibility of skin vessels, and the morphea-like variant, which presents as a plaque where the skin is folded, giving a "peau d'orange" appearance. The locations are frequently above the rib cage, above the sternum, or the scalp. Seventy-five percent of the patients develop regional lymphadenopathy within a few months. Histopathologic study reveals a capillary proliferation in the dermis. The diagnosis relies on subsequent identification of the plasmacytoma. At the time of a presumptive diagnosis, only 40% of patients have fulfilled the diagnostic criteria of POEMS. Treatment depends on the final hematologic diagnosis, but radiation of the plasmacytoma is required.Acral persistent papular mucinosis (APPM) is a subtype of localized lichen myxedematosus (LM) characterized by the chronic development of white to skin-colored papules and limited to the extensor surfaces of the hands and distal part of forearms, in the absence of systemic or laboratory manifestations. There is a strong predominance in women. Histopathology shows focal accumulation of mucin in the upper portion of the dermis sparing the grenz zone. The etiology is unknown. It is a benign condition, although dynamic changes occur with the progressive development of additional lesions. No specific treatment is required, and patients should be reassured about the prognosis.
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