10th July 2023

Skin in Systemic Disease

Respiratory

Sarcoidosis

Skin manifestations are seen in a quarter of those with systemic sarcoid, sometimes in the absence of systemic disease. Cutaneous manifestations include erythema nodosum, lupus pernio (see image below), scar sarcoid and scarring alopecia.

Lupus pernio

Image Source: Wikimedia Commons (2011). Lupus pernio 01, Licensed under a creative commons licence. Available for free at: https://commons.wikimedia.org/wiki/File:Lupus_pernio_01.jpg (Accessed on: 16 July 2022).

Tuberculosis

Tuberculosis is associated with scrofuloderma (breakdown of skin overlying TB focus), lupus vulgaris (50%), verrucosa cutis, scarring alopecia, and gumma.

Metabolic Disorders

The Porphyrias

The classic skin manifestations of porphyrias are blister formation, photosensitivity, scarring with milia and hypertrichosis.

Table 1: Types of porphyrias

Diabetes

Diabetes can often present with a wide range of cutaneous signs, such as necrobiosis lipoidica, granuloma annulare, diabetic rubeosis, diabetic dermopathy, candidiasis, vitiligo, and neuropathic foot ulcers.

• Diabetic dermopathy presents with dark red papules that eventually become atrophic and is associated with a long duration of diabetes mellitus.
• Necrobiosis lipoidica (waxy yellow lesions) mainly affects the shins and is associated with telangiectasia
• Granuloma annulare present as papules with central depression.

Acanthosis nigricans

Often described as dark, thick, velvety patches that present in skin folds, namely the underarms, neck, and groin. It often indicates an underlying health concern, such as internal malignancy, acromegaly, Cushing syndrome, obesity, insulin resistant diabetes or hypothyroidism. It can also be seen in those that take the oral contraceptive pill. Acanthosis nigricans can be familial.

Thyroid disease

Table 2: Thyroid disease and its cutaneous manifestations

Rheumatology

Rheumatoid arthritis

Specific cutaneous changes include:

• Rheumatoid nodules
• Vasculitis causing changes such as nail fold infarcts and ulcers
• Pyoderma gangrenosum

Dermatomyositis 

Skin manifestations include the classic heliotrope rash around the eyes, Gottron papules (red plaques on the extensor surfaces of finger joints), Gottron sign (erythema over knees and elbows), dilated nail fold capillaries, ragged cuticles, and nail fold infarcts. Please note that when a patient presents with dermatomyositis, this warrants an investigation for an underlying malignancy, namely bronchial, breast and ovarian.

Lupus Erythematosus

• Discoid lupus erythematosus is associated with scaly erythematous plaques with follicular plugging on sun-exposed areas of the skin. They tend to heal with scarring.
• Systemic lupus erythematosus (SLE) is commonly associated with cutaneous manifestations such as a malar rash (facial butterfly rash with purple hue), photosensitivity, vasculitis, Raynaud’s phenomenon, alopecia, and oropharyngeal ulceration.

Paraneoplastic Cutaneous Manifestations

Dermatological signs can be seen in various types of malignancy, with some being more common in certain types of neoplasia.

Table 3: Types of cutaneous manifestations in internal malignancies

Pyoderma Gangrenosum (PG)

PG presents clinically as painful, deep red necrotic ulcerating lesions with a violaceous border that mainly affects the lower extremities. Underlying medical disease is noted in approximately 50% of these patients, namely inflammatory bowel disease, rheumatoid arthritis, SLE and myeloid leukaemia. PG can characteristically cause peristomal complications in inflammatory bowel disease. Treatment involves oral steroid supplementation. PG is not typically associated with diabetes.

Table 4: Medical conditions associated with PG

Figure 72: Pyoderma gangrenosum 

Image Source: Wikimedia Commons (2008). Crohnie Pyoderma gangrenosum, Licensed under a creative commons licence. Available for free at: https://commons.wikimedia.org/wiki/File:Crohnie_Pyoderma_gangrenosum.jpg (Accessed on: 16 July 2022).

Gastrointestinal disorders

Hepatology

Table 5: Cutaneous manifestations of liver disease

Malnutrition

Please refer to the topic ‘Nutrition’ in the Gastroenterology section for more detail.