Unusual case of Anetoderma with Leukocytoclastic Vasculitis

presented by

Arash Radfar MD, PhD *

Elizabeth McLeish MD **

Boston, MA, USA

on May 14, 2006

*Dermatopathologist, Assistant Professor of Dermatology, Department of Dermatology, Boston University School of Medicine, Boston, MA, USA

**Dermatologist, Boston, MA, USA

Abstract The patient is a 16-year-old female who has the clinical picture of anetoderma and biopsy findings of leukocytoclastic vasculitis
16 y.o. female
3 months
trunk and extremities

The patient is a 16-year-old female with new onset inflammatory papules on the trunk and extremities which resolve to become atrophic macules.

Physical Examination

Generalised scattered thin atrophic erythematous macules on the trunk and extremities.


Laboratory Data



Skin biopsy shows features of leukocytoclastic vasculitis. There are occasional plasma cells in the infiltrate.

Diagnosis Anetoderma
Reasons Presented

This is a case of a 16-year-old young woman who has been developing thin atrophic macules for the last 3 months which clinically look like anetoderma but yet the biopsy showed leukocytoclastic vasculitis


The purpose of this is a) what is the diagnosis? b) what laboratory
investigations would be appropriate? and C) what treatment would anyone

References Hellwich M, Nickolay-Kiesthardt J. A case report of Jadassohn anetoderma Z Hautkr. 1986 Nov 15;61(22):1638-40, 1645.[Article in German]

Itchy pink plaques have been developing on the trunk of a 29-year-old patient for two years. After a few weeks, these plaques usually change into atropic scars. Both types of efflorescence occur simultaneously. Histological examination of a fresh lesion showed leukocytoclastic vasculitis. These micromorphological changes are no longer visible in the atrophic scars. The clinical picture and histological findings are identical with those seen in Jadassohn's anetoderma. The histological changes correspond with the degenerative-inflammatory process first described by Cramer in 1963.

Keywords anetoderma, leukocytoclastic vasculitis
Comments from Faculty and Members

Rick Sontheimer MD, Professor and Vice-Chairman, Dept. of Dermatology University of Oklahoma Health Sciences Center, Oklahama City,OK, USA on May 15, 2006

Any other clinical or lab evidence of SLE and/or antiphospholipid antibody syndrome?

Hussain Mahdi MD, Senior Resident, Dermatology Unit, SMC, Manama, Bahrain on May 15, 2006

Recently Lyme disease has been suspected to be one cause of secondary anetoderma. Atypical forms of acrodermatitis chronica atrophicans under the clinical picture of anetoderma have to be considered in secondery anetoderma.
I guess the patient should be investigated for lyme disease and connective tissue diseases.

Andrew Carlson MD, Professor, Divisions of Dermatopathology and Dermatology, Albany Medical College, Albany, NY, USA on May 15, 2006

There are some focal changes of vasculitis, but there is also a diffuse neutrophilic infiltrate (Sweet's-like, albeiti sparse than most cases). Anetoderma has been describe in the setting of neutrophilic dermatoses.; Therefore, the pathogenesis of the anetoderma/elastolysis is likely related to this neutrophilic dermal infiltrate. Determining exactly what the primary inflammatory process is the first step in management as the anetoderma is a phenomenon secondary to the inflammation.

Leibowitz, M. R., J. J. Rippey, et al. (1982). "Unusual aspects of febrile neutrophilic dermatosis (Sweet's syndrome). Case reports." S Afr Med J 62(11): 375-8.

Two patients with febrile neutrophilic dermatosis (FND) of Sweet's syndrome are described. One patient had acute myeloblastic leukaemia and FND antedated any changes in the peripheral blood. The second patient had bullous lesions which healed, with clinical cutis laxa (acquired anetoderma). In this patient FND had persisted for 8 years and histological examination of the skin lesions showed inflammation of the subcutaneous fat. To our knowledge this represents the first report of panniculitis due to FND.

Christensen, C. C. and F. Gonzalez-Crussi (1983). "Postinflammatory elastolysis and cutis laxa: report of a case with aortitis." Pediatr Pathol 1(2): 199-210.

A 17-month-old black female manifested an acute febrile dermatosis followed by the development of cutis laxa and aortitis. The neutrophilic, acute inflammatory nature of the disease is emphasized. Pathologically, both the skin and the aorta were affected by a lesional process that shared common morphologic attributes and resulted in extensive elastolysis. However, the disease appears to differ from other entities characterized by generalized degradation of elastic fibers.

Lewis, K. G., S. W. Dill, et al. (2004). "Mid-dermal elastolysis preceded by acute neutrophilic dermatosis." J Cutan Pathol 31(1): 72-6.

BACKGROUND: Mid-dermal elastolysis is a rare idiopathic elastic tissue disorder that is characterized by localized patches of finely wrinkled skin and a "band-like" loss of elastic tissue in the mid-reticular dermis. Lesions may be preceded by erythema and/or urticaria, and histological examination of inflamed lesional skin may demonstrate lymphohistiocytic dermal infiltration. CASE REPORT: We report a case of mid-dermal elastolysis in a 31-year-old woman who developed multiple erythematous and urticarial plaques on the arms and trunk. Histologic examination of a representative lesion revealed a neutrophilic infiltrate and a normal pattern of elastic tissue. Several months later, the erythema and urticaria was noted to have resolved, leaving soft, pendulous plaques with overlying finely wrinkled skin. A follow-up biopsy at this time showed minimal lymphocytic inflammation but almost complete absence of elastic tissue in the mid-reticular dermis. CONCLUSIONS: To our knowledge, acute neutrophilic dermatosis resulting in mid-dermal elastolysis has not been previously described. This observation lends support to an emerging theory that the pathogenesis of mid-dermal elastolysis may be inflammatory.

Verhagen, A. R. and M. J. Woerdeman (1975). "Post-inflammatory elastolysis and cutis laxa." Br J Dermatol 92(2): 183-90.

Post-inflammatory elastolysis and cutis laxa (Marshall, Heyl & Weber, 1966) is a skin disease in African infants which appears to be comparatively common in at least two countries. Destruction of elastic tissue and atrophy are preceded by urticarial or by annular erythematous-popular lesions and result in severe disfigurement. The clinical features are intermediate between anetoderma (macular atrophy) and acquired cutis laxa, but sufficiently typical and characteristic to constitute a distinctive syndrome, which might represent an abnormal reaction to the bite of an arthropod.

Muster, A. J., S. Bharati, et al. (1983). "Fatal cardiovascular disease and cutis laxa following acute febrile neutrophilic dermatosis." J Pediatr 102(2): 243-8.

Acute neutrophilic dermatosis (Sweet syndrome) is a benign self-limited disease in adults. A child with apparent evolution of acute neutrophilic dermatosis to postinflammatory cutis laxa and elastolysis then developed fatal vascular involvement. One other patient with postinflammatory cutis laxa with aortic regurgitation and sudden fatal unrecognized occlusive coronary arterial disease is discussed. If cardiovascular symptoms or signs develop during the course of Sweet syndrome or postinflammatory cutis laxa, a thorough investigation is warranted to rule out potentially fatal coronary arterial disease. Coronary bypass surgery may be the only effective treatment for the severely fibrosed proximal coronary arterial system.

Jeffrey Callen MD, Professor of Medicine (Dermatology), Chief, Division of Dermatology, Department of Medicine, University of Louisville School of Medicine, Louisville, KY, USA on May 19, 2006

The etiology of anetoderma is unclear, and although I suspect that the biopsy demonstrates a loss of elastic fibers along with vascular inflammation, there are multiple etiologies that might be possible in this case.

I agree with Rick Sontheimer, that an evaluation for LE and antiphospholipid antibodies is indicated. In addition, a thorough evaluation such as might be performed in a patient with cutaneous vasculitis is needed including testing of pANCA, cANCA, Hepatitis B surface antigen, Hepatitis C antibody, serum protein electrophoresis, HIV testing, ANA, anti-nDNA, anti-U1RNP and anti-Ro/SS-A.

Treatment should be aimed at an identified association should one be found, absent that the therapy of neutrophilic dermatoses or cutaneous small vessel vasculitis becomes empiric as there are no high quality RCT to guide us. I like colchicine or dapsone as potential initial therapies with appropriate screening and monitoring. I sometimes combine them. I prefer to use immunosuppressive agents over moderate to high dose corticosteroids.

Of course if evidence of antiphospholipid antibodies or other thrombotic disease is found, then treatment is otherwise directed.

It will be of interest for those of us reading Virtual Grand Rounds to hear follow-up from Drs. Rafdahr and McLeish after they have had an opportunity to further evaluate and treat this patient.

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