Dermatoliposclerosis I83.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Callosity of the lower leg; Congestive dermatitis; Dermatolipofasciosclerosis; Dermatosclerosis; Edema sclerosis; indurated hypodermitis; Lipodermatosclerosis; sclerodermatitis sclerodermiformis; sclerosing panniculitis

History
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Huriez, 1955

Definition
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Etiologically unexplained, chronic, inflammatory reaction of dermis, subcutis (and fascia) with varying degrees of pain, nodular and plate-like, after proximally sharply defined indurations of the lower legs, channel-like embedded varicose veins(canyon veins) and extensive pigmentation of the skin (S.a. venous diseases)

Etiopathogenesis
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Unexplained; many factors indicate that venous hypertension is the decisive pathogenetic principle in CVI ( chronic venous insufficiency). This is supported by the fact that the clinical picture improves with sufficient compression therapy. Dermatoliposclerosis is increasingly observed with Protein S-deficiency and Protein C-deficiency.

Manifestation
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Predominantly occurring in women of middle to higher age (40-70 years).

Localization
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Distal lower leg (one or both sides).

Clinical features
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Acute dermatoliposclerosis: In the acute phase mostly proximal arc-shaped or tongue-shaped flat erythema; moderate oedematous induration of the distal lower leg with moderate to distinct spontaneous and pressure pain; usually accompanied by oedema of varying extent.

Chronic Dermatoliposclerosis: The chronic stage which follows after months or years shows a pronounced, board-hard dermatosclerosis with extensive brown-red or brown-black discoloration, atrophy of fatty tissue and muscles and miniaturization of the lower leg contour (bottle leg). There is also moderate to marked pain, which increases after physical exertion. In areas with lipodermatosclerosis, cardiac edema typically does not develop. They are found distally and proximally. In particular, skin-coloured, bulging, doughy edema cuffs can develop proximally.

Laboratory
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Inconspicuous. Clarify protein S and C deficiency.

Histology
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Intralobular lipomembranous (membranocytic) fat necrosis and pronounced septal sclerosis.

Early lesions show an atrophic epidermis as well as mild lobular necrobiosis with micro- and macropseudo-lipid cysts of varying sizes. Lymphatic infiltrates and hemosiderin deposits in the edematized septa.

In the advanced stage, broad sclerosed septums with bulbous, English wall thickened capillaries and venules with PAS-positive perivascular fibrin sheaths, as well as macrophages and fibroblasts are found. Intralobularly, the fully developed picture of lipomembranous fat necrosis is shown with irregularly large fat or oil cysts which are bounded by thickened, homogeneously eosinophilic and PAS-positive membranes and in places also form papillary vegetation protruding into the lumen. These membranes probably originate from degenerated cell membranes of lipocytes.

Dermal changes: Perivascular superficial and deep sclerosing dermatitis with thickened vessels.

Differential diagnosis
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Clinical:

  • Acute dermatoliposclerosis:
    • Erysipelas (acute erysipelas is accompanied by hyperthermia, fever, painful lymphadenitis, leukocytosis and elevation of CRP); in chronic recurrent erysipelas the acute inflammatory symptoms may be absent.
    • Erythema nodosum (nodular, highly acute lower leg field dermatosis; localized on the stretching side; painfulness much more pronounced; clear inflammatory parameters).
  • Chronic dermatoliposclerosis:
    • Circumscript scleroderma (no signs of CVI, atypical localization, usually further scleroderma areas detectable).
    • Necrobiosis lipoidica (localization of the lower leg on the extensor side, no signs of CVI, symmetrical infestation in the US would be atypical).
    • Erythema induratum (preferably located on the back of the US; size 2.5 cm, rarely up to 10 cm; often deep-reaching painful meltdowns).

Histological:

Complication(s)
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Therapy
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Treatment of the underlying chronic venous insufficiency by adequate compression therapy. In addition, acetylsalicylic acid (e.g. ASS) 3 times/day 500 mg p.o. (if necessary 3 times/day 1 g p.o.) until the skin symptoms subside. Systemic glucocorticoids (short-term 5-20 mg prednisone equivalent/day p.o.) are second choice therapeutics.

Literature
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  1. Bruce AJ et al (2002) Lipodermatosclerosis: review of cases evaluated at Mayo Clinic. J Am Acad Dermatol 46: 187-192
  2. Huriez C (1955) Ulceres des jambes et troubles trophiques d`origine veineuses. Rev Pract 5: 2703-2721
  3. Kasperczak J et al (2013) Definition, classification and diagnosis of chronic venous insufficiency - part II]. Ginekol Pol 84:51-55

  4. Kucukguven A et al (2013) Matrix metalloproteinases as potential targets in the venous dilation associated with varicose veins. Curr drug targets 14:287-324

  5. Martin LK et al (2003) Ulcers caused by bullous morphea treated with tissue-engineered skin. Int J Dermatol 42: 402-404
  6. Morton LM et al (2013) Venous eczema and lipodermatosclerosis. Semin Cutan Med Surg 32:169-76

  7. Naschitz JE et al (1999) Acute lipase-induced panniculitis in rats with ligated veins of the hindlimb: a contribution to the role of acute panniculitis as a precursor of lipodermatosclerosis of venous disease. J Dermatol Sci 19: 9-16
  8. Stacey MC et al (2000) Pericapillary fibrin deposits and skin hypoxia precede the change of dermatoliposclerosis in limbs at an increased risk of developing a venous ulcer. Cardiovasc Surgery 8: 372-380

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Last updated on: 29.10.2020