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
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
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