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The imbalance
between metalloproteinases and their tissue inhibitors is involved
in the pathogenesis of dermatitis herpetiformis.
Mediators
Inflamm. 2005 Dec 14;2005(6):373-9.
Dermatitis
herpetiformis (DH) is a subepidermal autoimmune disease
characterized by skin and intestinal lesions consistent with coeliac
disease. There are also some data that metalloproteinases (MMPs) are
involved in the development of skin lesions in DH, however their
exact role in this process is not fully understood. The aim of the
study was to investigate whether MMPs and their inhibitors are
involved in pathogenesis of DH. Skin biopsies were taken from 13
patients with active DH and from 10 healthy subjects. The
localization and expression of MMPs and TIMPs were examined by
immunohistochemistry. MMPs expression was detected in basal
keratinocytes and in the whole epidermis in all of the DH subjects.
Neutrophils in microabscesses and in blister fluid were also
positive for MMPs. Expression of TIMPs was moderate or weak in all
examined biopsies. Our results allow us to conclude that imbalance
between these enzymes takes an important role in the pathogenesis of
DH.
Subepidermal
blistering disorders: a clinical and histopathologic review.Semin
Cutan Med Surg. 2004 Mar;23(1):10-8.
The
subepidermal blistering disorders are comprised of a number of
unrelated disorders with a diverse clinical presentation and
pathogenic basis that share in common the presence of blister
formation beneath the epidermis. Many of the disorders are both
debilitating and potentially fatal. Timely and accurate diagnosis
facilitates their appropriate management. The etiologic, clinical,
and pathologic attributes as well as the treatment of these
disorders including bullous pemphigoid, epidermolysis bullosa
acquisita, dermatitis herpetiformis, linear IgA dermatosis,
cicatricial pemphigold, herpes gestationis,and porphyria cutanea
tarda are described.
Dermatitis
herpetiformis.Dermatol
Online J. 2003 Oct;9(4):8.
Dermatitis
herpetiformis is a chronic, lifelong disease characterized both by
symptomatic, characteristic skin lesions and by a gluten-sensitive,
often asymptomatic, enteropathy. Cutaneous findings include
intensely pruritic vesicles on symmetrical extensor locations.
Treatment for disease may be pharmacologic agents or a gluten-free
diet. Evidence for the genetic basis of disease has been elucidated.
Dermatitis
herpetiformis.Baillieres
Clin Gastroenterol. 1995 Jun;9(2):371-93
Dermatitis
herpetiformis (DH) is a relatively rare skin disorder with an
estimated incidence of 1:10,000 in the UK. It is characterized by
urticarial plaques and blisters on the elbows, buttocks, and knees,
although other sites may also be involved. The eruption tends to be
persistent: only 10-15% of patients have spontaneous remission over
a 25-year study period. The disease is characterized by the presence
of IgA deposits in the upper dermis of uninvolved skin and the
diagnosis should not be made in the absence of these deposits.
Two-thirds of patients have a small intestinal enteropathy with
villous atrophy as seen in coeliac disease (CD). However, the
remaining third also show evidence of a gluten sensitivity in the
intestine, as judged by increased lymphocytic infiltration of the
epithelium. Villous atrophy also ensues after gluten challenge in
those patients with previous normal villous architecture. The
initial treatment of the rash is with one of the following three
drugs, dapsone, sulphapyridine or sulphamethoxypyridazine. However,
the rash also clears with gluten withdrawal. It must be stressed,
however, that the average time to achieve significant reduction in
drug requirements is 6 months and it can be over 2 years before
drugs are no longer required. On re-introduction of gluten the
eruption recurs. Patients with DH have a high incidence of
auto-immune disorders, thyroid disease, pernicious anaemia, and
insulin-dependent diabetes, and should be screened for those
diseases on a yearly basis. As with coeliac disease there is also an
increased incidence of lymphoma and a gluten-free diet appears to
protect patients from this complication. The mechanism by which
gluten causes the skin lesions has still to be elucidated, but
current investigations implicate lymphocytes and cytokines in the
pathogenesis. The original hypothesis of an antigen-antibody
reaction in the skin with complement activation causing the skin
lesions, may not be correct.
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