Female Genital Herpes In Women : Dermatology in India is very affordable-Complete Information

Female Genital Herpes In Women : Dermatitis herpetiformis (DH ) is an autoimmune blistering disorder associated with a gluten-sensitive enteropathy (GSE). The disease was described and named in 1884 by Dr. Louis Duhring at the University of Pennsylvania. It is characterized by grouped excoriations; erythematous, urticarial plaques; and papules with vesicles. These are located on the extensor surfaces of the elbows, knees, buttocks, and back. It is exquisitely pruritic, and the vesicles are often excoriated to erosions by the time of physical examination. Diagnosis requires direct immunofluorescence of a skin biopsy specimen showing deposition of immunoglobulin A (IgA) in a granular pattern in the upper papillary dermis. Although most patients are asymptomatic, more than 90% have an associated GSE upon endoscopic examination. Among patients with celiac disease, 15-25% develop DERMATITIS HERPETIFORMIS . The mainstays of treatment are dapsone and a gluten-free diet.

DERMATITIS HERPETIFORMIS  is a disease of the skin caused by the deposition of IgA in the papillary dermis, which triggers an immunologic cascade, resulting in neutrophil recruitment and complement activation. It has been hypothesized that DERMATITIS HERPETIFORMIS  is the result of an immunologic response to chronic stimulation of the gut mucosa by dietary gluten with subsequent activation of cutaneous endothelial cells and circulating inflammatory cells, including neutrophils.

An underlying genetic predisposition to the development of DERMATITIS HERPETIFORMIS  has been demonstrated. Both DERMATITIS HERPETIFORMIS  and celiac disease (CD) show an increased expression of HLA-A1, HLA-B8, HLA-DR3, and HLA-DQ2 haplotypes. Environmental factors are also important; monozygotic twins may have DERMATITIS HERPETIFORMIS , CD, and/or GSE with variable symptomatology.

Evidence is mounting that epidermal transglutaminase 3 (TGase3), a cytosolic enzyme involved in cell envelope formation during keratinocyte differentiation, is the autoantigen of DERMATITIS HERPETIFORMIS . Theoretically, DERMATITIS HERPETIFORMIS  is caused by dermal deposition of circulating immune complexes containing both IgA and TGase3. This is supported by the finding that precipitates of skin-bound IgA from DERMATITIS HERPETIFORMIS  lesions contain TGase3. In addition, it has been demonstrated that serum from DERMATITIS HERPETIFORMIS  patients contains high-affinity anti-TGase IgA autoantibodies. Skin and gut TGases are both highly homologous, and serum from patients with GSE, with or without skin disease, contains IgA antibodies to both skin and gut types. The target autoantigen of TGase3 has not been demonstrated in normal papillary dermis, suggesting it is part of the circulating complex that is deposited in the papillary dermis, rather than originating from the papillary dermis.

The leading theory for DERMATITIS HERPETIFORMIS  is that a genetic predisposition for gluten sensitivity, coupled with a diet high in gluten, leads to the formation of IgA antibodies to gluten-TGase complexes. These antibodies cross-react with TGase3, and IgA/TGase3 complexes deposit within the papillary dermis to cause the lesions of DERMATITIS HERPETIFORMIS . These IgA deposits can disappear after long-term (up to 10 y) avoidance of dietary gluten.

Cutaneous IgA deposits in DERMATITIS HERPETIFORMIS  have been shown to function in vitro as a ligand for neutrophil migration and attachment. Although IgA deposition is pivotal for disease, an increased serum IgA is not necessary for pathogenesis; in fact, case reports describe DERMATITIS HERPETIFORMIS  in patients with a partial IgA deficiency. When the disease is active, circulating neutrophils have a higher level of CD11b and an increased ability to bind IgA. In fact, the characteristic histologic finding of DERMATITIS HERPETIFORMIS  is neutrophil accumulation at the dermoepidermal junction, frequently localizing to the papillary tips of the basement membrane zone.

Collagenase and stromelysin 1 may be induced in basal keratinocytes by either cytokines released from neutrophils or by contact with keratin from damaged basement membrane matrix. Stromelysin 1 may contribute to blister formation.

One study found levels of E-selectin mRNA expression in normal-appearing skin of patients with DERMATITIS HERPETIFORMIS  to be 1271 times greater that that of controls. Additionally, the same study observed increased soluble E-selectin, IgA antitissue transglutaminase antibodies, tumor necrosis factor-alpha, and serum interleukin 8 levels in patients with DERMATITIS HERPETIFORMIS , providing further evidence of endothelial cell activation and a systemic inflammatory response as part of the pathogenic mechanism of the disease. Mild local trauma may also induce the release of cytokines and attract the partially primed or activated neutrophils, which is consistent with the typical location of DERMATITIS HERPETIFORMIS  lesions on frequently traumatized areas, such as the knees and elbows.

Deposits of C3 also may be present in a similar pattern at the dermoepidermal junction. The membrane attack complex, C5-C9, also has been identified in perilesional skin, although it may be inactive and not contribute to cell lysis.

Hormonal factors may also play a role in the pathogenesis of DERMATITIS HERPETIFORMIS , and 2 recent reports describe DERMATITIS HERPETIFORMIS  induced by treatment with leuprolide acetate, a gonadotropin-releasing hormone analog. Androgens have a suppressive effect on immune activity, including decreased autoimmunity, and androgen deficient states may be a potential trigger for DERMATITIS HERPETIFORMIS  exacerbation.

Apoptosis may contribute to the pathogenesis of epidermal changes in DERMATITIS HERPETIFORMIS , and recent research demonstrates a markedly increased apoptotic rate within the epidermal compartment in DERMATITIS HERPETIFORMIS . In addition, Bax and Bcl-2 proteins were increased in the dermal perivascular compartment and Fas proteins showed epidermal staining in DERMATITIS HERPETIFORMIS  lesions.

Most patients with DERMATITIS HERPETIFORMIS  have histologic evidence of enteropathy, even in the absence of symptoms of malabsorption. In one recent study, all DERMATITIS HERPETIFORMIS  patients had increased intestinal permeability (as measured by the lactulose/mannitol ratio) and up-regulation of zonulin, a regulator of tight junctions. Thus, increased expression of zonulin may be involved in the pathogenesis of enteropathy in patients with DERMATITIS HERPETIFORMIS .

United States

The only US study showed a prevalence of 11.2 cases per 100,000 population.

International

Prevalence has been reported as high as 10 cases per 100,000 population.

Patients with DERMATITIS HERPETIFORMIS  were followed (152 total) from the date of diagnosis to the end of 1989 for mortality and from 1971 or the date of diagnosis (if later) to 1986 for cancer incidence. Death occurred in 38 patients younger than 85 years, slightly fewer than expected on the basis of national general population rates. Cancer incidence was significantly increased. Cancer of the small intestine caused 1 death, and lymphoma caused 1 death. A 30-year population-based study of 1147 CD and DERMATITIS HERPETIFORMIS  patients in Finland also revealed an overall good prognosis for patients with DERMATITIS HERPETIFORMIS . The total occurrence of malignancies was equal to that of the general population in both CD and DERMATITIS HERPETIFORMIS , but an increased incidence of non-Hodgkin lymphoma was noted among both CD and DERMATITIS HERPETIFORMIS  patients with standardized incidence ratios of 3.2 and 6.0, respectively. Overall mortality was actually decreased in DERMATITIS HERPETIFORMIS  patients compared to that in the general population.

DERMATITIS HERPETIFORMIS  lesions are extremely pruritic. Morbidity results from scarring, discomfort, and insomnia due to itching. Secondary infection may also develop, requiring antibiotic therapy.

DERMATITIS HERPETIFORMIS  occurs more frequently in individuals of Northern European ancestry and is rare in Asians and persons of African descent. It is most common in Ireland and Sweden. This can be attributed to the shared HLA associations of DERMATITIS HERPETIFORMIS  and CD including DQA1*0501 and B1*-02, which encode HLA-DQ2 heterodimers.

US studies show a male-to-female ratio of 1.44:1, but international studies have demonstrated a male-to-female ratio up to 2:1. In one study of patients with GSE, 16% of the men and 9% of the women had DERMATITIS HERPETIFORMIS .

Typically, the onset of DERMATITIS HERPETIFORMIS  is in the second to fourth decade; however, persons of any age may be affected.

Patients typically present with a waxing and waning, pruritic eruption on the arms, knees, and buttocks. Small vesicles may have been noted. They may have associated worsening of disease with dietary intake of gluten. Many do not report any GI symptoms until prompted.

The diagnosis is suspected based on the distribution of the eruption.

DERMATITIS HERPETIFORMIS  has recently been proposed as a cutaneous manifestation of asymptomatic-to-mild CD. The genetic predisposition to the development of gluten sensitivity underlies the disease.

Differential Diagnoses

Bullous PemphigoidErythema MultiformeLinear IgA DermatosisNeurotic ExcoriationsScabiesTransient Acantholytic Dermatosis

Other Problems to Be Considered

EczemaPapular urticaria

Workup

Laboratory Studies

Procedures

Histologic Findings

Biopsy specimens of lesional skin reveal neutrophils in the dermal papillae, with fibrin deposition, neutrophil fragments, and edema. Eosinophils may be present. Papillary microabscesses form and progress to subepidermal vacuolization and vesicle formation. Vesicles form in the lamina lucida, the weakest portion of the dermoepidermal junction, due to neutrophil lysosomal enzymes

The histologic differential diagnosis of early skin lesions includes bullous lupus erythematosus, bullous pemphigoid, epidermolysis bullosa acquisita, and linear IgA disease. The histologic differential diagnosis of late skin lesions includes bullous drug eruption, bullous pemphigoid, erythema multiforme, and herpes gestationis.

Granular IgA deposits in dermal papillae of perilesional skin observed by direct immunofluorescence is the criterion standard of diagnosis. Inflammation in lesional skin degrades the immunoreactants and is usually negative for the diagnostic granular pattern. Because deposits are found throughout normal-appearing skin, the standard practice is to obtain biopsy specimens from normal-appearing perilesional skin for direct immunofluorescent staining.

Treatment

Medical Care

Control of the skin disease can be achieved with medications, dietary avoidance of gluten, or both.

Consultations

Diet

Dietary intake of gluten causes the disease, and elimination improves it.

Follow-up

Further Outpatient Care

Complications

Prognosis

Patient Education

Miscellaneous

Medicolegal Pitfalls

Special Concerns

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