Category Dermatology

Graft-versus-Host Disease

Key features ▪ Currently, over 25 000 allogeneic hematopoietic stem cell transplants (HSCTs) are performed worldwide each year ▪ Trends in the use of unrelated donors, non-myeloablative conditioning protocols, and donor lymphocyte infusions are impacting the incidence and presentation of graft-versus-host…

Nutritional Diseases

Key features ■ Nutritional deficiencies may affect any organ system, including the skin, which may provide clues to the diagnosis ■ The two classic protein–energy malnutrition syndromes are kwashiorkor and marasmus ■ In addition to inadequate oral intake, there are…

Calcifying and Ossifying Disorders of the Skin

Synonyms ▪ Calcinosis cutis: cutaneous calcification ▪ Osteoma cutis: cutaneous ossification ▪ Calciphylaxis: calcific uremic arteriolopathy; calcifying panniculitis; uremic gangrene syndrome ▪ Subepidermal calcified nodule: solitary congenital nodular calcification; Winer's nodular calcinosis Key features ▪ Calcium regulates several key physiologic…

Porphyria

Key features ▪ The porphyrias result from dysfunction of enzymes involved in heme biosynthesis ▪ The different types can be classified into either acute versus non-acute or cutaneous versus non-cutaneous forms ▪ Cutaneous symptoms exclusively involve sun-exposed areas of the…

Deposition Diseases

Introduction The cutaneous deposition disorders are a heterogeneous group of conditions characterized by the presence of primarily endogenous substances within the dermis or the subcutis. Deposition disorders can be associated with localized or generalized cutaneous findings, and skin involvement is…

Amyloidosis

Synonyms ▪ Primary systemic amyloidosis – AL amyloidosis ▪ Secondary systemic amyloidosis – AA amyloidosis Subtypes ▪ Primary cutaneous amyloidosis – macular amyloidosis, friction amyloidosis, lichen amyloidosis (lichen amyloidosus), biphasic amyloidosis, dyschromic amyloidosis, nodular amyloidosis Key features ▪ Characteristic properties…

Mucinoses

Key features ▪ The cutaneous mucinoses are a heterogeneous group of disorders in which an abnormal amount of mucin accumulates in the skin ▪ The etiopathogenesis of cutaneous mucinoses is unknown ▪ The cutaneous mucinoses are divided into two groups:…

Other Rheumatologic Disorders and Autoinflammatory Diseases

Although all the disorders in this chapter represent inflammatory systemic diseases, there are two major groups based upon underlying pathomechanisms. Disorders such as systemic-onset juvenile idiopathic arthritis (Still disease) and inherited autoinflammatory diseases are related to abnormalities in the innate…

Morphea and Lichen Sclerosus

Morphea and lichen sclerosus are inflammatory skin diseases that ultimately evolve into two distinct modes of cutaneous sclerosis. In morphea, there is inflammation of the dermis that may extend into subcutaneous structures. Lichen sclerosus is primarily a disease of the…