Laboratory Data

Atrophie Blanche

What is the diagnosis?
Considering the age, gender and typical ivory-white scars, I think this is a case of Atrophie blanche (AB) or Milian's white atrophy.
What is the etiopathogenesis? Is it a vasculitis or a vasculopathic reaction?
"Atrophie blanche is common in large, community-based leg-ulcer clinics and is clearly related to venous insufficiency…” (1a)
“Livedo with ulceration (SYN. ATROPHIE BLANCHE) is an uncommon condition in which ulceration occurs, particularly in the summer, in association with livedo reticularis of legs..” (1b)

It seems that there is a controversy about the nature of this disease. Personally, I think that the original idea of Winkelmann is still correct about Atrophied Blanche and it is a localized vasculitis rather than a secondary phenomenon to venous insufficiency.(2)

What about treatment?

Till 6 months ago, the patient was good with conservative therapy and pentoxyphylline, but recently she developed painful ulcerations in spite of treatment.
There is a long list of drugs reported to be helpful in this condition. Nicotinic acid, low-molecular-weight dextran, danazol, stanazol, heparin, colchicine and antimalarials are among them. I would like to know which ones are your first and second choices. Do you approach and manage it as a true vasculitis?

1a. Ryan TJ, Burnand K. Diseases of the veins and arteries: Leg ulcers. In Champion RH, Burton JL, Burns DA, Breathnach SM (eds). Rook/ Wilkinson/ Ebling Textbook of dermatology. Oxford, Blackwell Science, 6th edn., 1998. P.2248-50

1b. Ryan TJ. Cutaneous Vasculitis. In Champion RH, Burton JL, Burns DA, Breathnach SM (eds). Rook/ Wilkinson/ Ebling Textbook of dermatology. Oxford, Blackwell Science, 6th edn., 1998. P.2209-10

2. Winkelmann RK et al. Clinical studies of livedo vasculitis (segmental hyalinizing vasculitis). Mayo Clin Proc 1974;49:746

Cesare Massone, MD, Dept of Dermatology, Medical University of Graz, Graz, Austria on Oct 10, 2004

This is a very interesting case. I agree with the diagnosis of Atrophie Blanche. In my estimation Atrophie Blanche is not a true vasculitis but a manifestation of a thrombogenic vasculopathy, being the primary event the formation of hyaline thrombi in the lumen of small vessels in the upper and mid dermis without vasculitis. AB appeared to result from a decreased fibrinolytic activity of the blood (JAAD 1996; 204), platelets shows increased tendency to aggregate and fibrinopeptide A may be elevated (Clin Exp Dermatol 1992; 4). Anticardiolipin antibodies and lupus-type anticoagulant may be present.

David Elpern M.D., Williamstown, MA, USA on Oct 10, 2004

Over the years, I have seen a number of these patients and have not helped most of them. I agree with Cesare. Some of these patients may have coagulopathies. If so, would they benefit from anticoagulation; but need to be worked up first. There are a few experts who can comment on this. I will pass along their names to Henry who will see if they can comment. With patient's like this it is important to remember the old saw: Sometimes, it is more important to treat the patient who has the disease, than it is to treat the disease the patient has." Although this is attributed to Osler, I think it is older than Osler. By the way, the photo of Rasht is beautiful.

Mark Pittelkow M.D., Professor of Dermatology, Biochemistry and Molecular Biology, Mayo Clinic, Rochester, MN, USA on Oct 10, 2004

This is a valuable teaching case to emphasize several points. Terminology is a critical component of dermatology clinical practice and education. A common terminology is important for effective communication and undertanding a specific disease process and its treatment, as well as to appreciate and validate the 'local' or 'teaching center' variability in the use and interpretation of these clinical terms and associated concepts .

This case represents a prime example of the evolving usage and 'migration' of terminologies over time. Milian's atrophie blanche (early 1900's) bore similarity to livedo reticularis with summer [winter] ulceration (Feldaker et al Arch Derm 1955 and Circulation 1956) and eventually with the terms livedo/livedoid vasculitis (Bard, Winkelmann, Arch Derm 1967; Schroeter et al, Arch Derm 1975). More recently, livedoid vasculopathy (Klein, Pittelkow Mayo Clin Proc 1992; MaCalmont et al Clin Exp Derm, 1992) has been added to the terminological synonymy. Efforts are underway to provide an open access, comprehensive dermatology lexicon, and dermatologists are encouraged to actively participate in development of the first version of the dermatology lexicon project (dlp) http://www.futurehealth.rochester.edu/dlp/ or http://www.dermatologylexicon.org

Most cases of livedoid vasculopathy (LV) do not demonstrate primary, frank necrotizing or lymphocytic vasculitis on biopsy. Segmental hyalinizing changes and microvascular thrombosis of vessels are principally observed along with fibrinogen/fibrin and complement deposition in the vessel, often showing a "donut" pattern of deposition on immunofluoresence examination.

Not surprisingly,therefore, with the variable terminologies describing this condition, numerous treatments have been used as therapy for LV. Nicotinates, aspirin-dypyridamole, pentoxifylline, danazol or stanozolol, heparin or warfarin, and parenteral fibrinolytic agents such as alteplase [tissue plasminogen activator] (Klein, Pittelkow Mayo Clin Proc, 1992) for more severe, recalcitrant cases have been reported. Most recently, IVIg has been evaluated as a therapy ( Kreuter et al JAAD, 2004).

LV has been associated with various inflammatory, vascular or pro-coagulant disease states, including venous incompetence-ulceration, antiphospholipid syndrome, protein C, S, Factor II (prothrombin G20210A) and Factor V Leiden-activated protein C resistance, as well as underlying cutaneous poly(peri)arteritis nodosa. Even case series of Sneddon Syndrome and scleroderma have been reported w/ LV or LV-like features (Zelger et al Arch Derm, 1993; Thomas, Winkelmann Arch Derm, 1983).

Other diseases or treatment complications manifest ulcers similar to LV, including thrombocythemia (Itin, Winkelmann JAAD, 1991) and prolonged hydroxyurea treatment (Best et al Ann Int Med, 1998) Often heparin or LMW heparin (Jetton, Lazarus JAAD 1983; Hairston et al, Arch Derm 2003) provides the most consistent and long term benefit to control more active/chronic LV. Following fibrinolytic therapy for recalcitrant LV, coumadin/ASA seems to prevent recurrence of microvascular thrombosis and ulceration. Various persisting systemic or local vascular inflammatory or pro-coagulant abnormalities may play significant roles in perpetuating the underlying pathology causing the chronicity that is typically observed for LV.

In addition, it is important to have the patient discontinue all tobacco use and use a high quality elastic compression (30-40 mm Hg) garment to minimize swelling and venous stasis that aggravates LV.

Victoria P Werth, M.D., Professor of Dermatology, University of Pennsylvania School of Medicine, Philadelphia, PA on Oct 11, 2004

I agree with the comments that have been made relating to livedoid vasculopathy (LV). In a young person such as this, there is likely some hypercoagulable factor responsible for the ulcers, although often even extensive work-ups don't yield the etiology. LV is more a vessel plugging phenomenon (vasculopathy) than a true vascultiis. I agree with the therapeutic suggestions already made and would echo the benefits of good leg compression, as with surgical support stockings.