Nursing Center - Journal Issue

Keywords: Lipodermatosclerosis, Morphea, Myxedema, Nephrogenic Fibrosing Dermopathy, Scleredema, Systemic Sclerosis

Abstract

ABSTRACT: An 80-year-old male patient reported an 8-month history of a red, flat rash on his bilateral anterior lower extremities, accompanied by swelling of the ower extremities. He complained that his skin "felt thick" but was otherwise asymptomatic. One month after being diagnosed with lipodermatosclerosis by his primary care provider, he presented to dermatology. The differential diagnoses for this patient's presentation are discussed here and include nephrogenic fibrosing dermopathy, systemic sclerosis, scleredema, myxedema, and morphea.

An 80-year-old Caucasian gentleman presented to dermatology for a rash on his bilateral anterior lower extremities. The patient reported that his skin changes began 8 months before as a red, flat rash, accompanied by swelling of the lower extremities. He also complained that his skin "felt thick" but was otherwise asymptomatic, and he had not treated it with anything before presenting to dermatology. His primary care provider had diagnosed him with lipodermatosclerosis 1 month before presentation in dermatology.

The patient had no history of deep venous thrombosis, vascular insufficiency, superficial thrombophlebitis, lower extremity claudication, varicose veins, or stasis dermatitis.

He was in good health, with a past medical history significant only for hypercholesterolemia and an abdominal hernia 3 years before. He had no history of thyroid disorder, diabetes, renal disease, or known exposure to gadolinium.

On presentation, he was a well-appearing, older gentleman, in no acute distress. On bilateral lower extremities, from knee to ankle, he had ill-defined hyperpigmented plaques with central hypopigmentation. The skin felt thick and bound down. There were smaller erythematous, bound-down plaques on the thighs. On the forearms, he had small, discrete bound-down violaceous plaques.

The initial workup included liver profile, basic metabolic profile, complete blood count, rheumatoid factor, and protein electrophoresis, which were all within normal limits. His antinuclear antibody was elevated at 1:160 and was speckled, and his C-reactive protein was elevated at 7.

No biopsy was performed; diagnosis was made based on clinical findings. He was started on clobetasol (0.05%) ointment and calcipotriene (0.005%) cream, both twice daily to all affected areas. One week later, his disease was no worse but had not improved with topical treatment.

It was recommended that oral therapy be initiated. A test dose of methotrexate (MTX; 10 mg) by mouth once a week for 2 weeks was given. His liver profile was reassessed during this trial treatment and remained normal.

After 2 weeks of methotrexate and continued topical therapy, the plaques on his arms were improving. After 1 month of treatment, the erythema around the plaques on both arms and legs had resolved, and all lesions were less indurated and firm than at initial presentation.

At the time of publication the patient was continuing to see improvement in skin thickness, firmness, and erythema and was currently tolerating the methotrexate and topical treatments without laboratory abnormalities or other problems.

WHAT'S YOUR DIAGNOSIS?

A. Lipodermatosclerosis

B. Nephrogenic fibrosing dermopathy

C. Systemic sclerosis

D. Scleredema

E. Myxedema

F. Morphea

Lipodermatosclerosis

Lipodermatosclerosis is a form of panniculitis or inflammation of the subcutaneous fat, which is seen in patients with chronic venous insufficiency (Moschella & Hurley, 1992). Patients usually present with skin induration, hyperpigmentation, swelling, erythema, and narrowing of the distal lower leg near the ankle, which is known as inverted champagne bottle or bowling pin appearance (Bolognia, Jorizzo, & Rapini, 2008; Walsh & Santa Cruz, 2010). There may be small, white, scarred areas, known as atrophie blanche (Bolognia et al., 2008). The overlying skin can be warm, tender, and scaly, leading to the frequent misdiagnosis of cellulitis (Moschella & Hurley, 1992).

Lipodermatosclerosis usually occurs in people over 40, without inciting illness or trauma (Moschella & Hurley, 1992). The cause is not completely understood but is related to venous hypertension and venous insufficiency, and it occurs more commonly in people who are obese. Some patients present with recurrent episodes of painful inflammation of the lower extremities, often just proximal to the ankle (Bolognia et al., 2008).

The diagnosis can usually be made clinically but can be confirmed with a skin biopsy. Primary management of lipodermatosclerosis consists of compression therapy to correct venous stasis. Other components of therapy and management include sclerotherapy, weight reduction, oral pentoxifylline to increase blood flow, topical steroids or intralesional steroids to reduce inflammation, and topical capsaicin for pain relief (Bolognia et al., 2008).

Nephrogenic Fibrosing Dermopathy

Nephrogenic fibrosing dermopathy is a fibrosing disease of the skin and internal organs, distinct from scleroderma and scleromyxedema (Bolognia et al., 2008). It is only seen in patients with renal insufficiency who have received imaging studies with gadolinium, a contrast dye. Gadolinium can be found in tissue samples of patients with NSD (Pryor et al., 2007).

NSD usually has a gradual onset with development of induration, thickening, and hardening of the skin, with hyperpigmentation. The skin is often shiny, with a woody consistency, and the classic peau d'orange appearance (Bolognia et al., 2008).

Skin changes are most readily appreciated on the extremities, followed by the trunk; the face is almost never involved. Yellow palmar papules have been reported, as have yellow scleral plaques (Pryor et al., 2007).

NSD is a debilitating and sometimes fatal disease. Patients complain of burning, itching, swelling, and hardening and tightening of the skin. Joint complaints are common, with difficulty moving or straightening the arms, hands, legs or feet (Bolognia et al., 2008). Patients may have muscle weakness and bone pain, especially involving the hipbones or ribs (Bolognia et al., 2008; Pryor et al., 2007).

The most successful treatment appears to be extracorporeal photopheresis (Bolognia et al., 2008); however, for most patients, the disease is progressive and slow to respond to treatment (Schieren et al., 2009).

Systemic Sclerosis

Systemic sclerosis is an autoimmune systemic connective tissue disease whose pathogenesis is only partially understood (Bolognia et al., 2008). There are three main changes in systemic sclerosis: induration and thickening of skin (scleroderma), abnormalities of both microvasculature (telangiectasia) and large vessels (Raynaud's phenomenon), and fibrotic degenerative changes in various organs, such as heart, lungs, kidneys, and gastrointestinal tract (Bolognia et al., 2008).

Systemic sclerosis can have many different presentations. Patients may have involvement of the skin only, internal organs only, or both skin and internal organs. Because of the various presenting symptoms, diagnosis is often difficult at the onset (Bolognia et al., 2008).

Raynaud's phenomenon is the most common initial complaint; the second most common complaint is symmetric painless edema or thickening of the skin of the digits, known as sausaging of the digits (Moschella & Hurley, 1992). Patients may also complain of pruritus, difficulty in swallowing, shortness of breath, palpitations, joint pain or swelling, or myositis (Bolognia et al., 2008; Moschella & Hurley, 1992).

No definite therapy has been shown to slow or stop progression of skin or visceral disease. Treatment is tailored to organ involvement. Pruritus can be ameliorated by topical camphor or menthol, emollients, psoralen plus UVA treatment, or UVA-1 phototherapy (Bolognia et al., 2008).

Numerous oral therapies have been proposed and tested, many without effect. d-Penicillamine, interferon, photopheresis, corticosteroids, methotrexate, chlorambucil, cyclosporine, tacrolimus, thalidomide, intravenous cyclophosphamide, statins, immunosuppressive drugs with stem cell transplantation, and imatinib have been investigated. Results are varied, and no one therapy has shown consistent benefit. Whichever therapy is initiated, care should be multidisciplinary, including dermatology, pulmonology, nephrology, radiology, cardiology, and orthopedic surgery (Bolognia et al., 2008).

Scleredema

Scleredema is an uncommon disorder of progressive, nonpitting induration of the skin. Although it does not typically affect the arms and legs, it is discussed here because it presents with tightening and induration of the skin, similar to the complaints of our patient. Scleredema usually occurs on the face, neck, and upper back, and patients report difficulty smiling, opening the mouth, and wrinkling the forehead (Moschella & Hurley, 1992).

Scleredema may be associated with an acute antecedent febrile illness, most often streptococcal infection, diabetes mellitus, blood dyscrasia, and, rarely, multiple myeloma. Although generally considered to be a benign, self-limited skin condition, scleredema may be persistent and can involve the viscera (Bolognia et al., 2008).

Primary lesions are ill-defined, woody, nonpitting, indurated plaques that may clinically mimic edema. There may be erythema, hyperpigmentation, and/or peau d'orange appearance. The epidermis overlying affected areas wrinkles when pinched (Bolognia et al., 2008).

Streptococcal throat culture and antistreptolysin-O titer should be obtained, in addition to fasting glucose, glycosylated hemoglobin, and serum protein electrophoresis (Bolognia et al., 2008).

Although the name implies sclerosis and edema, neither is present on histology. Instead, there is mucin deposition between dermal collagen bundles, seen mostly in the deep dermis (Moschella & Hurley, 1992).

Treatment with appropriate antibiotic therapy should be initiated for patients with documented or suspected infection. Evaluation and treatment for blood dyscrasia and diabetes should be completed (Bolognia et al., 2008).

Myxedema

Myxedema is a primary disorder of mucin, in which there is deposition of hyaluronic acid in the dermis and subcutis, usually because of thyroid disease. This diagnosis should be considered in a patient presenting with skin induration of the pretibial area (Moschella & Hurley, 1992).

Patients present with sharply circumscribed nodules or plaques over the anterior lower legs, usually bilaterally (Bolognia et al., 2008). Lesions can extend to any part of the legs and lower abdomen. Lesions are skin-colored to yellow-brown, nonpitting, and waxy. Follicular pores may be dilated, resulting in the peau d'orange appearance. Small plaques are asymptomatic, but as lesions enlarge, they become painful (Bolognia et al., 2008; Moschella & Hurley, 1992). Cosmesis may be a concern.

Onset of disease is usually within 1-2 years of diagnosis of Graves' disease. Myxedema in the absence of Graves' disease is rare (Bolognia et al., 2008; Moschella & Hurley, 1992).

Workup should include thyrotropin levels, which may be abnormally high or low, and long-acting thyroid stimulator, which is elevated in 50% of patients (Bolognia et al., 2008). Skin biopsy shows deposition of mucin in the reticular dermis and separation of collagen bundles into individual fibers (Bolognia et al., 2008; Moschella & Hurley, 1992).

First line treatment is usually compression wraps or stockings with 20-40 mm Hg of pressure and topical corticosteroids under occlusion. If topical corticosteroids are ineffective, intralesional corticosteroids may be helpful. Ideal management includes collaboration between dermatology and endocrinology (Bolognia et al., 2008).

There is little evidence in the literature to support the use of hydroxychloroquine, but broadband UVA, long-wavelength UVA-1, and psoralen plus UVA have shown marked clinical improvement in morphea. Case reports of treatment with extracorporeal photopheresis, photodynamic therapy, and pulsed dye laser seem promising, but larger studies are needed.