The porphyrias represent a group of metabolic disorders in which there is dysfunction of the enzymes involved in heme synthesis ( Fig. 41.1 ). With the exception of acquired porphyria cutanea tarda (PCT), the underlying etiology is monogenetic mutations. Porphyrins…
A heterogeneous group of disorders in which there are deposits, usually of endogenous materials, within the skin. Entities belonging to this group that are covered in other chapters include mucinoses ( Ch. 38 ), amyloidosis ( Ch. 39 ), porphyrias…
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In this group of disorders, there is deposition of glycosaminoglycans, previously referred to as mucopolysaccharides (“mucin”), within the skin, especially the dermis. Most often, the deposit is composed of hyaluronic acid. While several of the entities are idiopathic, underlying disorders…
Systemic-Onset Juvenile Idiopathic Arthritis (SoJIA; Still Disease) and Adult-Onset Still Disease (AoSD) Among the major types of juvenile idiopathic arthritis (JIA; formerly juvenile rheumatoid arthritis [JRA]), cutaneous manifestations are most common in SoJIA, psoriatic arthritis (see Ch. 6 ), and…
Morphea (Localized Scleroderma) An uncommon fibrosing disorder that is limited to the skin, subcutaneous tissues, and occasionally the underlying bone; if present on the face and/or scalp, it can be associated with underlying CNS abnormalities. It is distinct from limited…
The spectrum of sclerosing skin disorders is outlined in Fig. 35.1 . Systemic Sclerosis (SSc, Scleroderma) An uncommon autoimmune connective tissue disease (AI-CTD) that affects the skin, blood vessels, and various internal organs (e.g. kidney, lung, gastrointestinal tract, heart). Etiology…
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General A multisystem AI-CTD disorder that prominently affects the skin. Broadly divided into systemic lupus erythematosus (SLE), cutaneous lupus erythematosus (CLE), and drug-induced lupus erythematosus (DI-LE) ( Fig. 33.1 ). CLE is further classified into specific and nonspecific skin lesions,…
Eccrine and apocrine glands represent the two major types of sweat glands (see Fig. 91.1 ). Eccrine Glands Functional from birth and activated by thermal stimuli via the hypothalamic sweat center; while their major function is thermoregulation by evaporative heat…