Cutaneous Manifestations of Systemic Diseases

Acanthosis nigricans (AN)
(Figure 3.48A)

Velvety hyperpigmentation (body folds)

Most common: GI adenocarcinoma (gastric)

Acquired angioedema
(Figure 3.50C)

Angioedema without urticaria

Lymphoproliferative diseases

Diamond-shape scale (often legs)

Lymphoma (Hodgkin and NHL)

Cicatricial localized loss of hair

Breast cancer (metastatic)

Primary amyloidosis
(Figure 3.49A)

Periorbital ‘pinch’ purpura

Multiple myeloma (one fourth of cases)

Bazex sign
(Acrokeratosis neoplastica)
(Figure 3.50A)

Psoriasiform plaques on palms, soles, nose and ear helices

Upper aerodigestive tract carcinoma

Head/neck flushing, pellagra-like dermatitis, erythema

{↑ 5-HIAA levels (hydroxyindoleacetic acid), a serotonin metabolite}

Mid-gut tumors (w/ liver metastases), gastric/bronchial carcinoid tumors

Purpura, acrocyanosis, livedo reticularis

Lymphoplasmocytic disorders

Gottron’s papules, poikiloderma

GI and ovarian cancer

Erythema gyratum repens
(Figure 3.50D)

Gyrate polycyclic plaques with trailing scale

Various malignancies, bronchogenic carcinoma most common

Sudden growth of soft, downy hair in adult

Lung and colon cancer

Metastases, cutaneous
(Figure 3.49F)

Pink/violaceous papules/nodules

Most common: breast and lung

Multicentric reticulohistiocytosis
(Figure 3.49D)

Erythematous papules mainly over face and dorsal hands; arthritis

Various malignancies (30% with underlying cancer)

Erythematous patches with bullae over face, groin and abdomen (severe intertrigo)

Pancreatic carcinoma (α-cell tumor)

{Glucagonoma syndrome: glucagon-secreting carcinoma, NME, weight loss, glossitis, DM}

Yellow plaque commonly seen periorbitally

Paraproteinemia, occasionally myeloma

Subcutaneous nodules (legs)

Pancreatic carcinoma

Paget’s disease
(Figure 3.49B)

Eczematous to psoriasiform plaque

Adnexal, breast, GU or GI cancer

Erosive disease of mucous membranes

Lymphoma, CLL

Localized or generalized

Hodgkin’s lymphoma mainly

Pyoderma gangrenosum (Figure 3.49E)

Rapidly expanding ulceration with undermined border

Hematologic malignancy (especially atypical bullous form)

Suddent onset of multiple seborrheic keratoses

Various malignancies: carcinoma (gastric, colon, breast) and lymphoma

Erythematous, pseudovesicular papules, nodules and plaques

Acute myelogenous leukemia, less commonly lymphoma

Tripe palms
(Acanthosis palmaris)
(Figure 3.49C)

Thickened, velvety palms witih pronounced dermatoglyphics

Lung cancer (if only palms), gastric cancer (if palms + AN)

Yellow, thin plaques favoring trunk, periorbital and body folds

Monoclonal gammopathy including multiple myeloma

– Acanthosis nigricans
– Bullous diabeticorum: (Figure 3.48B) tense noninflammatory blisters on lower extremities
– Diabetic dermopathy: atrophic yellow to brown macules on lower legs
– Disseminated granuloma annulare
– Necrobiosis lipoidica diabeticorum
– Scleredema of Buschke
– Eruptive xanthomas (Figure 3.48F)

– Coarse dry skin
– Generalized myxedema: boggy and edematous
– Dull, brittle hair
– Alopecia of lateral 1/3 eyebrows (madarosis)
– Onycholysis

– Velvety, smooth or moist skin
– Hyperpigmentation (localized or generalized)
– Pretibial myxedema: (Figure 3.48E) yellow-brown waxy papules on lower extremities
– Fine hair
– Mild but diffuse alopecia
– Koilonychia
– Onycholysis

– Hyperpigmentation

– Spider angiomas
– Palmar erythema
– Gynecomastia
– Terry’s nails

– Generalized hyperpigmentation

– Pruritus
– Eruptive/planar xanthomas

– Kayser-Fleischer rings
– Blue lunulae

– Mixed cryoglobulinemia
– Porphyria cutanea tarda
– Lichen planus (particular oral) (Figure 3.48C)
– Pruritus
– Necrolytic acral erythema
– Polyarteritis nodosa

– Erythema nodosum (Figure 3.48D)
– Pyoderma gangrenosum (also oral pyostomatitis vegetans)

– Pruritus
– Uremic frost
– Calciphylaxis
– Acquired perforating disorder (Figure 3.50B)
– Nephrogenic systemic fibrosis