Histiocytoses are rare disorders characterized by the accumulation of cells derived from macrophages, dendritic cells or monocytes in various tissues. There is a broad spectrum of disease manifestations with some subtypes commonly showing skin lesions, while in others, the skin is rarely involved. Here, we describe cutaneous manifestations of histiocytoses belonging to the Langerhans group (L group), the group of cutaneous and mucocutaneous histiocytoses (C group) and the group of Rosai-Dorfman disease (RDD) and related histiocytoses (R group) according to the current classification. Characteristic clinical presentations noted were a rust-brown colour or xanthomatous aspect in many cases of histiocytoses. Histological criteria for differentiation are described. Immunohistochemistry shows positivity for S100 and CD1a in Langerhans-cell histiocytoses (LHCH) of the L group, while CD68 positivity with S100 and CD1a negativity are typical in histiocytoses of the C group of cutaneous and mucocutaneous histiocytoses. RDD in the R group shows positivity for S100 and CD68, while CD1a is negative. We further review the pathogenesis of LHCH based on insights on the central role of the mitogen-activated protein (MAPK) kinase pathway. Common mutations in various histiocytic populations of diverse ontogeny and at different stages of differentiation may be responsible for the diverse clinical picture of this neoplastic entity. For histiocytoses of the C group and R group, a reactive origin is discussed with the exception of the disseminated form of juvenile xanthogranuloma. We suggest exploring the role of an origin from skin residing histiocytes for the isolated cutaneous manifestation in some types. With regard to therapeutic options, skin-directed therapies are the first choice in limited disease, while systemic chemotherapy has traditionally been used in extensive disease. In Langerhans-cell histiocytoses and related entities, therapy by BRAF inhibition has led to a breakthrough especially in patients with an activation of the MAPK pathway.

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The physical properties of the SLLL compression stocking kit correspond to the characteristics of a strong stocking at rest and exercise (DSI). The donning success rate is excellent (100%). A further potential advantage is that the SLLL leg compression kit is dose adjustable, according to indication or patient tolerance. Wearing comfort over periods of several days and clinical effectiveness need to be investigated in future trials.

SummaryIn acute and chronic wounds, pain represents a common and central medical problem. Wound pain can be caused by multiple factors, such as macro- or microvascular as well as inflammatory processes.The basic concept of pain management is based on the WHO pain ladder. Often this concept of pain therapy remains insufficient. Apart from the conventionally used anal-getics, there are not yet established guidelines for the use of alternative substances.Ultimately the adequate wound pain therapy must be adjusted to each patient and his comorbidities.

Summary Introduction: Complex regional pain syndrome (CRPS) is a relatively rare disorder, but one that is extremely serious for the affected patient. It usually occurs in the area distal to a primary limb injury. The clinical symptoms and the pain are out of all proportion to the inciting event and in approximately 10 % of CRPS patients, there is no triggering event at all. CRPS leads to long-term disability and high treatment and follow-up costs In about half of those affected.Clinical symptoms: Two forms exist. In CRPS type 1, no nerve lesions are present, whereas in CRPS type 2, injury has occurred to a nerve or the main branch of a nerve. However, in terms of their clinical course, there is no difference between the two forms. Approximately 90 % of all cases involve CRPS type 1, formerly known as “Sudeck’s atrophy”. The cardinal symptom is pain. In addition, trophic disturbances, such as swelling, local skin discolouration or asymmetric skin temperatures, can also occur. Impaired mobility and function of the affected limb also occur frequently and are very difficult to treat. Diagnosis: Initially, it can be difficult to distinguish between CRPS and a normal post-traumatic course. Subsequently, the severe symptoms are out of all proportion to the inciting event. The diagnosis of CRPS is based mainly on the clinical symptoms. The Budapest criteria help to confirm the diagnosis. Therapy: Early and interdisciplinary rehabilitation is of crucial importance in CRPS treatment. Occupational therapy and physiotherapy are supplemented by good analgesic management and psychological support, if required. Analgesia should be based on the WHO pain ladder. Methadone is of proven efficacy in cases of severe hyperalgesia and gabapentin or pregabalin are used to treat refractory pain. Bisphosphonates have shown a good analgesic effect, particularly in patients with confirmed bone lesions. Chronic oedema and inflammation may require short-term steroid administration. A further clinical goal is the avoidance of sequelae, such as osteoporosis. Patients with suspected CRPS should be referred to a multidisciplinary treatment team, preferably one with considerable experience in treating this clinical presentation. One physician should coordinate the patient’s treatment. The earlier the treatment is started, the better the prognosis.