Dermatology

Lipodermatosclerosis (hypodermitis sclerodermiformis, sclerosing panniculitis, pseudoscleroderma)

Are You Confident of the Diagnosis?

What you should be alert for in the history

Complaints of erythema, pain, tenderness and induration on the medial leg proximal to the malleolus. The patient has not responded to one or more courses of antibiotics for suspected cellulitis. Usually has a history of stasis dermatitis and leg ulcers. No history of trauma to site.

Characteristic findings on physical examination

Acute: Diffuse or localized erythema. Can be bilateral. Tender plaque-like induration often with palpable well defined margins on the medial leg proximal to malleolus. Frequently pigmentary changes associated with stasis dermatitis. Ulceration rare in acute phase. Leg edema and varicosities (Figure 1).

Figure 1.

Acute lipodermatosclerosis

Chronic: Leg “inverted bowling pin” shape with bound down hard, scleroderma-like hyperpigmented skin on medial leg (Figure 2). Ulceration is often associated.

Figure 2.

Chronic lipodermatosclerosis

Expected results of diagnostic studies

Venous duplex ultrasound to evaluate the venous system with particular attention to saphenofemoral junction, the short saphenofemoral junction in the popliteal fossa, and possible incompetent perforators in area of pathology.

Diagnosis confirmation

Cellulitis: Painful erythema but patients complain of malaise, are often febrile and respond to antibiotic therapy. Cellulitis lacks the discrete induration of lipodermatosclerosis.

Erythema nodosum: Tender erythematous induration but usually multiple lesions that are not localized to the characteristic stasis area on medial leg.

Acute thrombophlebitis demonstrates a positive venous duplex ultrasound study for thrombosis.

Morphea is usually not erythematous and a medial leg location would be unusual. A biopsy would confirm the diagnosis.

Who is at Risk for Developing this Disease?

More common in women with a high body mass and a history of venous abnormalities.

What is the Cause of the Disease?

Etiology: Progressive venous disease

Pathophysiology

Knowledge is evolving, but incomplete. The problem begins with venous hypertension and extravasation of fluid from veins, and end result is skin hardening and fibrosis. The multifactorial intervening and sequence of events await clarification.

Systemic Implications and Complications

The main systemic complication associated with venous disease, ulcerations and lipodermatosclerois, is immobility leading to increased body mass and the diseases associated with increased weight, notably hypertension, diabetes.

Treatment Options

PHYSICAL MODALITIES

Compression wraps (Unna's boot, Profore)

Compression stockings

MEDICAL OPTIONS

Systemic

Pentoxyfylline

Anabolic steroids

Intralesional / topical

Intralesional steroid

Topical capsaicin

SURGICAL OPTIONS

Vascular consult for venous duplex ultrasound

Vascular (venous) surgery: endovenous laser or radiofrequency ablation

Optimal Therapeutic Approach for this Disease

Evaluation of venous system with venous duplex ultrasound with special attention to incompetent perforators underlying clinical pathology. Vascular surgery is recommended in selected positive cases.

Compression therapy, usually initially with Unna's boot, to reduce edema. The rationale as to why wraps preferred to compression stockings (even in absence of ulceration) is that compression stockings are removed nightly and thereby do not give 24/7 compression. In conjunction with intralesional steroid, most patients tolerate wraps.

Intralesional triamcinolone 10mg/cc, 1 to 2cc, injecting 0.2 to 0.3cc directly into the induration at approximately 0.5 to 1cm intervals. Repeat weekly into the residual indurated sites. Although the mechanism of intralesional steroids is still not certain, their effect is presumably related to reduction of inflammatory cytokines.

Pentoxyfylline 800mg 3 times a day x 8 weeks. The medication is used to supplement standard therapy if the response is slow. The proposed mechanism of pentoxyfilline is normalization of vascular endothelium. The side effect profile of pentoxyfilline is minimal as the drug is usually very well tolerated.

Anabolic steroids: Stanazolol is no longer available from US manufacturers. Danazol 100mg daily or oxandrolone 10mg twice daily x 8 weeks. The rationale is based on its presumed fibrinolytic action. The evidence is based on a few case reports as there are no randomized controlled trials to substantiate its use.

Patient Management

Patients are ideally seen weekly with repeat intralesional steroids into residual areas of painful induration and application of Unna's boot. When pain has subsided and edema controlled, compression stockings 20 to 30mm Hg are recommended. If patient cannot don the stockings, lesser manageable compressions are used.

Unusual Clinical Scenarios to Consider in Patient Management

“Sterile” wipe prior to intralesional therapy to avoid infection.

What is the Evidence?

Miteva, M, Romanelli, P, Kirsner, RS. "Lipodermatosclerosis". Dermatol Ther. vol. 23. 2010. pp. 375-88.

(Up-to-date comprehensive review of lipodermatosclerosis with complete references)

Bruce, AJ, Bennett, DD, Lohse, DN, Rooke, TW, Davis, MDP. "Lipodermatosclerosis: Review of cases evaluated at Mayo Clinic". J Am Acad Dermatol. vol. 46. 2002. pp. 187-92.

(Data on all aspects of lipodermatosclerosis over a 22-year span at Mayo Clinic)

Gniadecka, M, Karlsmark, T, Bertram, A. "Removal of dermal edema with class I and II compression stockings in patients with lipodermatosclerosis". J Am Acad Dermatol. vol. 39. 1998. pp. 966-70.

(Proposes comparable efficacy and better compliance of lighter compression stockings in the ongoing, but still inconclusive, discussion of the use and selection of compression stockings and wraps in venous disease.)

Herouy, Y. "Lipodermatosclerosis and compression stockings". J Am Acad Dermatol. vol. 42. 2000. pp. 307-8.

(Effects of compression therapy: prevention of extravasation of fluid into extravascular spaces not only as a mechanical aid, but also through decreasing vascular permeability by tightening the paracellular barrier)

Campbell, L, Miller, OF. "Intralesional triamcinolone in the management of lipodermatosclerosis". J Am Acad Dermatol. vol. 55. 2006. pp. 166-8.

(The Geisinger experience of successful treatment of lipodermatosclerosis with intralesional triamcinolone and wrap therapy)

Heymann, W. "Dialogues in dermatology, Lipodermatosclerosis". J Am Acad Dermatol. vol. 60. 2009. pp. 1022-3.

(A clear and incisive commentary after a Dialogue. Pathophysiology issues especially well summarized.)
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