Systemic conditions that affect skin

It is important to remember that the skin can act as a window to a patientâââ€Å¡Ã‚¬Ãƒ¢Ã¢€Å¾Ã‚¢s general health. Changes in the skin can signal underlying disease.

A physician writing in the journal Medicine, says it is incumbent upon dermatologists to conduct a thorough examination of the skin, nails, mucosal surfaces, and hair so as to not overlook clues that might help arrive at a diagnosis of a systemic condition.

The article, by Ruth C. Lamb, a consultant dermatologist at St. Georgeâââ€Å¡Ã‚¬Ãƒ¢Ã¢€Å¾Ã‚¢s NHS University Hospital Trust in London, outlines systemic conditions that can affect the skin.¹ Dr. Lamb stresses that by looking at the skin and noticing any changes in the skin, clinicians can potentially identify a condition that has remained undiagnosed.  

LUPUS ERYTHEMATOSUS

Lupus erythematosus represents a group of inflammatory conditions that have involvement of the skin. The criteria for the classification of systemic lupus erythematosus underwent a revision that increased sensitivity and specificity.²

Subtypes of lupus erythematosus are divided into subacute cutaneous lupus erythematosus (SCLE) and chronic cutaneous lupus erythematosus, with the latter including discoid lupus erythematosus. Depending on the variant of SCLE, it can present as psoriasiform plaques.  

Photoprotection is vital in the management of all skin symptoms associated with lupus erythematosus subtypes. Treatment is topical and takes the form of topical corticosteroids or calcineurin inhibitors. Systemic lupus erythematosus can require immunosuppressants to be effectively treated.

DERMATOMYOSITIS

When a condition is rare, such as dermatomyositis, skin changes can be an avenue to diagnosis. Some of the skin symptoms that are associated with dermatomyositis include the presence of Gottronâââ€Å¡Ã‚¬Ãƒ¢Ã¢€Å¾Ã‚¢s papules, an eruption of erythema on the upper eyelids, and changes in nail folds including periungual erythema ragged cuticles, and abnormal capillaries at the proximal nail fold.

Several investigations have to be conducted to arrive at a diagnosis of dermatomyositis. Taking a skin biopsy and taking blood samples should be done to identify the presence of any antinuclear antibodies, which assess the presence of autoimmune disorders. Testing for extractable nuclear antigen antibodies may follow testing for antinuclear antibodies.

Identifying muscle weakness, a symptom which also characterizes dermatomyositis, requires imaging as well as blood tests to measure creatinine kinase and inflammatory markers. Other inflammatory skin conditions like lupus erythematosus, psoriasis, and eczema make up the differential diagnoses of dermatomyositis.

Management of dermatomyositis will vary depending on the extent of muscle involvement, but the skin symptoms can be treated with topical agents like corticosteroids or systemic agents like hydroxycholoroquine or methotrexate. An association between dermatomyositis and has pointed to an elevated risk of malignancy in adults with dermatomyositis, necessitating investigations to exclude possible malignancy.³

SKIN CHANGES ASSOCIATED WITH GASTROENTEROLOGICAL CONDITIONS

Skin changes can also occur when patients have gastroenterological conditions, such as inflammatory bowel disease. In a minority of cases (15%), patients with inflammatory bowel disease can experience skin changes such as erythema nodosum, mucosal surface changes like episcleritis, pyoderma gangrenosum, and psoriasis. Cutaneous manifestations, such as erythema nodosum and episcleritis, parallel activity of the bowel disease, but other cutaneous manifestations, such as pyoderma gangrenosum, can present independent of the bowel disease activity.

Another condition that can occur with IBD is Sweetâââ€Å¡Ã‚¬Ãƒ¢Ã¢€Å¾Ã‚¢s syndrome, a condition which may not require treatment for it can spontaneously resolve. If treatment is initiated, oral glucocorticoids form the basis of treatment.

MALIGNANCIES

If an individual has an internal malignancy, skin changes can present, particularly paraneoplastic skin disease. If a malignancy such as squamous cell carcinoma develops, paraneoplastic skin changes can occur in conjunction, taking the form of acrokeratosis paraneoplastica, a rare acral psoriasiform dermatosis.

Psoriasiform plaques typically develop in sites such as the nose and ears in acrokeratosis paraneoplastica. The pathophysiology of acrokeratosis paraneoplastica is not well-understood, but it has been put forth that it arises as a result of cross-reactivity between skin and tumor antigen, or the function of growth factors produced by the tumor,⁴ or even nutritional deficiency of zinc. Less than 200 cases have been reported in the literature, and men have made up the vast majority of cases that have been diagnosed. Some cases diagnosed in women have been associated with gynecological cancers.⁵

The skin signs of acrokeratosis paraneoplastica, such as an acral skin finding, can precede the diagnosis of internal malignancy, highlighting the role of the dermatologist in diagnosing internal malignancy.⁶

REFERENCES

¹Lamb RC. Skin manifestations of systemic disease. Medicine. 2017;45(7):399-404.

²Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982 Nov;25(11):1271-7.

³Barnes BE, Mawr B. Dermatomyositis and malignancy. A review of the literature. Ann Intern Med. 1976; 84: 68-76

⁴Amano M, Hanafusa T, Chikazawa S, et al. Bazex Syndrome in Lung Squmaous Cell Carcinoma: High Expression of Epidermal Growth Factor Receptor in Lesional Keratinocytes with Th2 Immune Shift. Case Rep Dermatol. 2016 Dec 8;8(3):358-362.

⁵Squires B, Daveluy SD, Joiner MC, Hurst N, Bishop M, Miller SR. Acrokeratosis Paraneoplastica Associated with Cervical Squamous Cell Carcinoma. Case Rep Dermatol Med. 2016; 2016: 7137691.  

⁶Gul ÃÆ’Ã…â€Å“. Acral manifestations of paraneoplastic and collagen vascular diseases. Clin Dermatol. 2017 Jan âââ€Å¡Ã‚¬Ãƒ¢Ã¢‚¬Ã…“ Feb;35(1):50-54.