edgepedia

Metabolic and systemic dermatoses

16 articles

Acquired ichthyosis

Acquired ichthyosis, secondary or paraneoplastic ichthyosis, is a rare nonhereditary adult-onset scaling skin disorder linked to Hodgkin lymphoma, HIV, hypothyroidism, sarcoidosis, malabsorption, and drugs, resolving when the cause is treated.

Acral persistent papular mucinosis

Acral persistent papular mucinosis (APPM), a rare subtype of localized lichen myxedematosus, causes 2–5 mm flesh-colored papules on the backs of hands, wrists, and distal forearms; benign, skin-limited, female-predominant, no systemic links.

Atypical lichen myxedematosus

Atypical lichen myxedematosus, an intermediate form of lichen myxedematosus (papular mucinosis), is a rare cutaneous mucinosis not meeting criteria for scleromyxedema or localized LM, with about 20 reported cases and unpredictable course.

Benign acanthosis nigricans

Benign acanthosis nigricans is a skin condition with dark, thick, velvety patches in body folds such as the neck and armpits, caused by insulin resistance and obesity rather than cancer.

Cutaneous mucinosis

Cutaneous mucinosis (mucinoses) is a group of uncommon skin disorders with abnormal mucin (hyaluronic acid) deposition in dermis or follicles, classified as primary or secondary, dermal or follicular, including scleromyxedema, follicular mucinosis, and focal mucinosis.

Diabetic skin lesions

Diabetic skin lesions (ICD-11 EB90.0) are skin changes caused by diabetes mellitus, including infections, diabetic dermopathy, necrobiosis lipoidica, and foot ulcers; pooled prevalence is about 70% of diabetic patients.

Discrete papular lichen myxedematosus

Discrete papular lichen myxedematosus is a rare, chronic skin condition with small, symmetrical, flesh-colored mucinous papules on the limbs and trunk, without paraproteinemia or thyroid disease.

Eruptive xanthoma

Eruptive xanthoma, ICD-11 EB90.22, is a skin marker of severe hypertriglyceridemia: sudden crops of 1–5 mm yellow papules on extensor surfaces and buttocks, with dermal foam cells on biopsy, resolving as triglycerides fall.

Lichen amyloidosis

Lichen amyloidosis, also called lichen amyloidosus, is a rare chronic primary localized cutaneous amyloidosis with intensely itchy hyperkeratotic papules on the shins, keratin-derived amyloid in the papillary dermis, OSMR/IL-31RA links, and refractory treatment.

Macular amyloidosis

Macular amyloidosis (macular cutaneous amyloidosis) is a rare, chronic primary localized cutaneous amyloidosis: pruritic gray-brown macules in a rippled pattern on the upper back and arms, without systemic amyloid involvement.

Nephrogenic systemic fibrosis

Nephrogenic systemic fibrosis (NSF), formerly nephrogenic fibrosing dermopathy, is a rare gadolinium-triggered fibrosing disease of patients with severe renal impairment after MRI contrast, causing indurated skin, contractures, and sometimes organ fibrosis.

Nodular cutaneous amyloidosis

Nodular cutaneous amyloidosis (PLCNA), the rarest form of primary cutaneous amyloidosis: AL light-chain amyloid from local plasma cells forms waxy 5–20 mm nodules on acral sites, with about 7–10% risk of systemic progression.

Nodular lichen myxedematosus

Nodular lichen myxedematosus (atypical tuberous myxedema of Jadassohn-Dosseker), ORPHA:90393, is a rare localized cutaneous mucinosis with skin-coloured mucinous nodules on limbs and trunk, without monoclonal gammopathy or thyroid disease.

Pretibial myxoedema

Pretibial myxoedema (thyroid dermopathy, localized myxoedema) is a rare Graves' disease skin manifestation with hyaluronic acid buildup on the shins, treated first with corticosteroids, emerging teprotumumab.

Scleredema

Scleredema of Buschke (scleredema adultorum) is a rare scleromucinous skin disease of unknown cause causing woody skin induration starting on the neck, in postinfectious, paraproteinemia-associated, and diabetes-associated types.

Tuberous xanthoma

Tuberous xanthoma (xanthoma tuberosum, ICD-11 EB90.21) is a firm, painless red-yellow nodule of foam cells over pressure areas such as elbows, knees, and heels, usually signaling type III hyperlipoproteinemia or familial hypercholesterolemia.