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Guidelines for the management of dermatitis herpetiformis

  • Bahria Medical and Dental College


Dermatitis herpetiformis is a rare autoimmune bullous disorder characterized by intensely pruritic polymorphic lesions over extensor surfaces of body, associated with gluten sensitive subclinical or clinical enteropathy. Dermatitis herpetiformis is often misdiagnosed and there is a paucity of guidelines for the management of dermatitis herpetiformis in literature. The present guidelines have been prepared for physician and dermatologists. It reflects highest evidence based data available at the time of formulating the guidelines. The diagnosis of dermatitis herpetiformis is made clinically, histologically, immunopathologically and supported by serology. A gluten free diet is the treatment of choice for all patients with dermatitis herpetiformis. Dapsone should be used during the acute presentation until the gluten free diet is effective. In conclusion, the present guidelines provide evidence based guidance for management of dermatitis herpetiformis.
Journal of Pakistan Association of Dermatologists 2013; 23 (4): 428-435.
Review Article
Guidelines for the management of dermatitis
Muhammad Irfan Anwar, Ajmal Rashid, Moizza Tahir, Afsheen Ijaz
Dermatology Department, PNS SHIFA, Karachi, Pakistan
Dermatitis herpetiformis is a rare autoimmune bullous disorder characterized by intensely pruritic
polymorphic lesions over extensor surfaces of body, associated with gluten sensitive subclinical or
clinical enteropathy. Dermatitis herpetiformis is often misdiagnosed and there is a paucity of
guidelines for the management of dermatitis herpetiformis in literature. The present guidelines have
been prepared for physician and dermatologists. It reflects highest evidence based data available at
the time of formulating the guidelines. The diagnosis of dermatitis herpetiformis is made clinically,
histologically, immunopathologically and supported by serology. A gluten free diet is the treatment
of choice for all patients with dermatitis herpetiformis. Dapsone should be used during the acute
presentation until the gluten free diet is effective. In conclusion, the present guidelines provide
evidence based guidance for management of dermatitis herpetiformis.
Key words
Dermatitis herpetiformis, guidelines, management.
Dermatitis herpetiformis (DH), initially
described by Louis A. Duhring, is a chronic,
intensely itchy polymorphic skin disease which
rarely presents as a clear bullous dermatosis.1-3
DH can appear at any age,4 but onset is most
frequent in the third decade.5 It is very rare in
children under 3 years of age.6 DH is slightly
more common in men than in women.6 While all
patients with DH are sensitive to gluten, the
majority of patients do not develop digestive
symptoms.7 The finding of granular deposits of
IgA along the dermo-epidermal junction is a
pathognomonic finding of dermatitis
herpetiformis. Treatment of dermatitis
herpetiformis is based on a lifelong, gluten-free
diet, which improves all clinical manifestations
of gluten sensitivity, and dapsone, that is only
effective for the skin manifestations.
The pathogenesis of DH involves a complex
relationship between autoimmune factors, such
as human leukocyte antigen (HLA)
predisposition, genetics and environmental
factors. A strong genetic predisposition to
dermatitis herpetiformis exists among affected
family members.HLADQ2 and HLADQ8 are
associated with dermatitis herpetiformis. The
immunopathogenesis of DH is linked with the
pathogenesis of gluten sensitivity in Celiac
disease.8,9 Tissue transglutaminase (tTG) is the
major autoantigen for celiac disease.10 The tTG
protein is primarily a cytoplasmic calcium-
dependent enzyme that catalyzes cross-linkage
between glutamine and lysine residues leading
to stabilization of the cytoskeleton.
Transglutaminases play a key role in the
pathogenesis of gluten intolerance. First, tTG
modifies the alcohol soluble fraction of gluten
known as gliadin into an efficient autoantigen,
resulting in T cell stimulation leading to
inflammatory response. In addition, protein-
Address for correspondence
Brig. Dr. Ajmal Rashid,
Dermatology Department, PNS SHIFA,
Karachi, Pakistan
Journal of Pakistan Association of Dermatologists 2013; 23 (4): 428-435.
protein cross-linking results in tTG-gliadin
complex formation that also generates an
autoantibody response, further squealing into
However, in patients of DH epidermal
transglutaminase (eTG) appears to be the
dominant autoantigen as it colocalizes with IgA
deposition in the skin. eTG is homologous to
tTG.14 The main function of eTG in the
epidermis involves cross-linking of cornified
envelope.15 Whether the IgA anti-eTG antibodies
play a central role in the pathogenesis of DH is
still in doubt. Passive transfer of sera from
diseased patient to the healthy substrate does not
lead to the production of disease, and
furthermore the presence of IgA deposits does
not seem to correlate with disease activity.16
However, serum anti-eTG titres strongly
correlate with the gluten free diet. Future
research studies may elaborate the exact
pathogenesis in this regard.
Cutaneous manifestations
Primary lesions of DH are grouped
erythematous papules and vesicles. Due to the
intense itching associated with this condition,
patients often scratch all the vesicles and may
present with erosions and excoriations.17 The
lesions are symmetrically distributed over the
extensor surfaces of the upper and lower limbs,
elbows, knees, scalp and buttocks (Figure 1).
The face and groin may also be involved. Mostly
lesions heal without scarring and usually
associated with postinflammatory hyper- or
hypopigmentation. An uncommon cutaneous
manifestation is palmoplantar purpura. This
finding is more common in children, but a
number of cases in adults have been described.18-
21 Clinically, small purpuric lesions are present
on the palms or soles. But, there is no dorsal
hand involvement reported. Mucosal
involvement is rarely seen in DH.22 Dental
abnormalities have been elaborated in patients
with DH.23,24,25 Enamel defects in permanent
teeth, horizontal grooves, defects in enamel
color, and large enamel pits are the most
common dental findings in patients with DH.26,27
Figure 1 Excoriated papules over extensor surfaces.
Figure 2 Dermal papillary tip abscess in DH.
Figure 3 Granular IgA deposition at dermoepidermal
Associated diseases
Journal of Pakistan Association of Dermatologists 2013; 23 (4): 428-435.
While 100% patients with DH are sensitive to
gluten, the majority do not develop GI
symptoms.28 Only 13% had digestive
symptoms,29 namely diarrhea, abdominal pain, or
growth retardation in case of children. In the
remaining 87%, gluten sensitive enteropathy
(GSE) was diagnosed after small bowel biopsy.
DH and celiac disease are frequently associated
with other autoimmune disorders, e.g.
autoimmune thyroiditis, diabetes mellitus, SLE,
Sjogren syndrome, vitiligo and pernicious
anemia.30 Approximately 20% of patients with
GSE have antithyroid antibodies and antiparietal
cell antibodies. So, it is recommended to
measure these autoantibodies in addition to
antinuclear antibodies, fasting blood glucose
levels, and thyroid function test in patients with
DH. Gluten sensitivity may present with ataxia
known as gluten ataxia.24% percent of
idiopathic cerebellar ataxia is a gluten induced
ataxia. This the single most common cause of
the disease.31
The diagnosis of DH is based on a constellation
of findings on physical examination, routine
histopathology, immunofluorescence studies,
and serological testing.
Biopsy specimens for histopathology should
contain an intact vesicle. Skin biopsy shows
neutrophilic microabscesses in the dermal
papillary tips and variable degree of
inflammatory infiltrate in the papillary dermis
often with subepidermal clefts (Figure 2). These
findings, however, are not specific and can also
be found in other autoimmune blistering
Direct immunofluorescence studies
The detection by direct immunofluorescence
(DIF) method of granular deposits of IgA with
or without complement factor (C3) at the
dermoepidermal junction of healthy perilesional
substrate is pathognomonic and gold standard
for the diagnosis of DH (Figure 3).
Serological tests
Serologic testing is a useful adjunct to tissue
based assays. Circulating IgA antibodies against
gliadin, endomysium, tissue transglutaminase
(tTG) and epidermal transglutaminase (eTG) are
detected in DH. Serologic testing has the
advantages of entailing a lower cost and being
less invasive than a skin biopsy.
Other investigations
Genetic testing to determine a patient’s HLA
type is useful in cases where DH cannot be
excluded. However, because the prevalence of
these alleles in the general population is rather
high, a positive test is not sufficient to diagnose
DH. Therefore, HLA typing is not recommended
as part of the routine work up of DH. A biopsy
specimen of the small bowel is usually not
necessary in DH work up. However, should
clinical signs of GI disease or malignancy be
evident on physical examination, further
investigations, imaging and work up may be
indicated. Because, a significant number of DH
patients have other autoimmune diseases,
screening for such disorders is indicated. In
particular, patients should be evaluated for
thyroid disease by checking both thyroid
stimulating hormone and antithyroid peroxidase
antibody titers.32,33 Measuring of fasting blood
glucose level for diabetes is also recommended.
Finally, screening for other autoimmune
connective tissue diseases should be done, if
Journal of Pakistan Association of Dermatologists 2013; 23 (4): 428-435.
Box 1 Diagnostic work up for the diagnosis of DH
1. Skin biopsy
i .Histopathology of the lesional skin:
neutrophilic microabscesses in dermal
papillary tips, subepidermal blister
ii. Direct immunofluorescence study of
healthy perilesional skin: granular
immunoglobulin (Ig) A deposits in the
basement membrane (Gold Standard)
2. Immunology study
i .Serum IgA antiendomysial/antigliadin
ii. Serum IgA antitransglutaminase
antibodies (anti eTG)
3. Class II HLA typing (optional and if available)
i. DQ2
ii. DQ8
4. Autoimmune screening (if clinically indicated)
Free T4, TSH levels, fasting blood sugar levels, ANA
5. Digestive system study (optional)
i. Malabsorption studies (Serum iron, B12,
folic acid)
ii. Small bowel biopsy (to see villous
clinically indicated, especially in patients with
suspicious symptoms, such as joint pain or
Differential diagnosis
The differential diagnosis should include
scabies, atopic eczema, contact eczema, chronic
prurigo, urticaria, popular urticaria and other
autoimmune blistering diseases such as linear
IgA dermatosis and bullous pemphigoid. In
general, a diagnosis of DH can be made with
ease and certainty based on histopathology and
DIF findings.
The cornerstone of DH management is the
gluten free diet (GFD). Strict adherence to a
GFD leads to the resolution of skin disease and
an improvement in bowel pathology.34,35,36,37
Gluten free diet
A gluten free diet (GFD) is the treatment of
choice in DH and absolutely essential in all
patients with DH (Table 1). It is effective
against skin manifestations and digestive
symptoms if these exist, although improvement
may not be seen until 1 to 2 years after the
elimination of gluten from the patient’s diet.
Wheat, barley, and rye and all kind of products
made from them must be eliminated completely,
but rice, corn, and pure oats are allowed.38
Consultation with a dietician is recommended,
because the maintenance of a GFD can be
difficult and requires a great deal of commitment
on the part of the patient. The diet must be
maintained for life as the vast majority of
patients with DH experience a resurgence of
symptoms when they reintroduce gluten into
their diet. Strict long-term adherence to a gluten
free diet has the following benefits: a sensation
of general well-being, a reduced need for
medication, a reduced risk of intestinal
lymphomas, and resolution of the skin rash and
signs and symptoms of enteropathy.39
Strict GFD does not lead to a rapid resolution of
itching and inflammatory condition of skin. It
may take months to year to cause its
symptomatic effects and addition of a
medication is necessary to achieve a rapid
control over symptoms. Sulfones, such as
dapsone, sulfapyridine or sulphasalazine, rapidly
suppress the symptoms of DH. Dapsone has
remarkable anti-inflammatory properties and
inhibits neutrophil recruitment via chemotaxis
and also suppresses neutrophil mediated tissue
injury.41 Symptoms of DH usually resolve within
Journal of Pakistan Association of Dermatologists 2013; 23 (4): 428-435.
Table 1 Diet in dermatitis herpetiformis [40]
Food items allowed Food items to avoid
Rice, rice flour
Corn, corn flour
Pure oat
Soya bean
Fresh vegetable
Fresh Fruits
Fresh brewed coffee
Apple cider
Pure chocolate
Canned food item (preservatives)
Instant coffee and instant tea
Ice cream cones
Packed rice mix
Shampoo containing wheat germ oil
Dental adhesive and tooth paste containing offending grains
Fish rich in iodine contents (Shellfish)
Iodized salt
Box 2 Adverse effects of dapsone therapy
1. Toxic (mostly dose dependent)
Hemolytic anemia
2. Idiosyncratic
Dapsone hypersensitivity syndrome
Peripheral neuropathy
Exanthematous eruption
Stevens Johnson syndrome
Toxic Epidermal Necrolysis
Optic nerve atrophy,
Psychosis, headache, nervousness, lethargy,
Nephropathy: nephritis, renal failure
GI effects like nausea, vomiting, diarrhea
Box 3 Monitoring of dapsone therapy
1. Baseline monitoring
Complete blood count
Reticulocyte count
Glucose-6-phosphate dehydrogenase level
Liver function studies
Urinalysis and renal function studies
2. Follow-up monitoring
Complete blood count every 2 weeks for 12
weeks; then once every 3 months
Reticulocyte count every 2-4 weeks for 12
weeks; then once every 3 months
Renal and liver function studies every 3
Methemoglobin levels if patient becomes
symptomatic (dyspnea, headache, dizziness)
Journal of Pakistan Association of Dermatologists 2013; 23 (4): 428-435.
days of starting dapsone therapy. Dosages of 25 mg to 100 mg daily are usually enough to control
symptoms.42 Although dapsone effectively decreases itching and inflammatory lesions, patients taking this
drug should be strictly monitored because of the possibility of severe adverse effects ( Box 2 and 3). The
commonest adverse effect of dapsone is hemolysis, specially, in patients suffering from glucose-6-
phosphate dehydrogenase deficiency. So patients should be seen within 2 weeks after starting drug with a
complete blood count. Adverse effects are usually dose dependent and more common in patients with
comorbid conditions. In few patients, dapsone can induce a severe hypersensitivity syndrome, consisting
of fever, rash, malaise, lymphadenopathy, and visceral involvement, 3-6 weeks after the beginning of
treatment. As dapsone does not therapeutically alter the underlying enteropathy, the significance of a GFD
must repeatedly be elaborated to the patient.
Sulfasalazine and sulphamethoxypyridazine
Sulfasalazine and sulphamethoxypyridazine may provide an alternative to dapsone, when it fails to
control the disease or the therapy is complicated by adverse effects of dapsone. The suggested dosages are
of 1-2 g/day for sulfasalazine and of 0.25-1.5 g/day for sulphamethoxypyridazine. Both drugs share
common adverse effects, including hypersensitivity reactions, hemolytic anemia, proteinuria and
crystalluria. So, a full blood count and urine microscopy should be carried out prior to starting treatment
and then monthly for the first 3 months of therapy, and thereafter, once every 6 months. However, the
most common adverse effects are nausea, anorexia and vomiting, which can be avoided by the use of
enteric coated forms of the drugs.
Orally administered corticosteroids give poor results while application of potent or very potent topical
steroids e.g. clobetasol propionate can be useful to decrease pruritus
Although their efficacy is not very high in the treatment of dermatitis herpetiformis, third generation
antihistamines e.g. desloratadine and fexofenadine, with specific activity on eosinophilic granulocytes,
may also be used to control itching.
Prognosis and complications
Dermatitis herpetiformis is a treatable condition with a favorable prognosis, especially for those who
adhere with a strict gluten free diet for life. The disease runs a very long course with exacerbations and
remissions .Overall, 10 to15 years survival rates in patients with DH do not appear to differ from those of
the general population. The association between DH and development of lymphomas is a subject of
debate. Unlike in patients with celiac disease, where the association between enteropathy and
hematological and GI malignancies has been well established, the incidence of lymphomas in patients
with DH is lower than 2% according to literature published to date. A possible explanation is that patients
with DH have a lower level of gastrointestinal inflammation than those with celiac disease.
Journal of Pakistan Association of Dermatologists 2013; 23 (4): 428-435.
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Second National Conference
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... 3,4 Etiopatogenesis DH didasari pada beberapa temuan klinis dan pemeriksaan penunjang yang berhubungan dengan CD, yaitu hubungan predisposisi genetik HLA genotip DQ, derajat gluten sensitive enteropathy (GSE) yang ditemukan pada biopsi usus halus, deposisi IgA granular di sepanjang stratum papilare jaringan dermis pada pemeriksaan imunofluoresensi, dan infiltrasi neutrofil pada papila dermis pada pemeriksaan histopatologi. 1,4,8 A. Predisposisi Genetik Gen spesifik HLA yang berinteraksi dengan reseptor sel-T diperkirakan mempunyai spesifisitas antigen yang sama pada pasien CD dan DH. Gen yang mengkode HLA-DQ2 dan DQ8 terdapat baik pada pasien CD maupun DH. 4,9 Meskipun terdapat kemiripan gen asal, faktor lingkungan juga berpengaruh terhadap terjadinya penyakit ini. 1 ...
... Sel-T CD4+ yang berinteraksi dengan residu tersebut juga menstimulasi sel-B, yang akan mengalami diferensiasi menjadi sel plasma dan memproduksi autoantibodi IgA terhadap tTG. 1,8,10 Autoantibodi dalam sirkulasi tersebut bereaksi silang dengan eTG pada kulit yang membentuk kompleks imun dan mengalami deposit pada stratum papila dermis. Kompleks imun tersebut menyebabkan proses inflamasi lokal yang didominasi oleh infiltrasi neutrofil. ...
... Mortalitas pasien DH yang berhubungan dengan limfoma juga ditemukan lebih rendah pada pasien yang menjalani diet bebas gluten. 2,4,8 SIMPULAN Dermatitis herpetiformis merupakan penyakit kulit autoimun bersifat kronik-residif akibat proses sekunder hipersensitivitas terhadap zat gluten. Manifestasi klinis berupa lesi ruam papulo-vesikular atau papul eskoriasi yang didominasi rasa gatal. ...
Full-text available
p>Dermatitis herpetiformis (DH) merupakan penyakit kulit autoimun kronik-residif akibat proses sekunder hipersensitivitas terhadap gluten. Kejadian DH tinggi pada populasi dengan predisposisi genetik HLA-DQ2 atau DQ8. Manifestasi klinis DH berupa lesi polimorfik ruam papulo-vesikular atau papul-eskoriasi didominasi rasa gatal. Baku emas diagnosis DH adalah pada pemeriksaan DIF didapatkan deposit imunoglobulin (Ig)-A granular di stratum papila dermis. Diet bebas gluten merupakan tata laksana utama. Dapson menjadi obat pilihan pertama. Prognosis baik dengan diagnosis dan tata laksana yang tepat. Dermatitis herpetiformis (DH) is a chronic-recurrent autoimmune skin disease caused by secondary hypersensitivity to gluten. The incidence of DH is high in population with genetic predisposition to HLA-DQ2 or DQ8. Clinical manifestations of DH are polymorphic lesions, papulo-vesicular rash or papules-excoriations, dominated by itching. The gold standard for diagnosis is the presence of granular immunoglobulin (Ig)-A deposits in the stratum papilla dermis on DIF examination. A gluten-free diet is the mainstay of treatment. Dapsone is the drug of choice. Prognosis is good with proper diagnosis and treatment. Prayogi Miura Susanto. Diagnosis and Management of Dermatitis Herpetiformis</p
... Due to intense itching associated with the disease, patients often scratch the vesicles and this results in an appearance that appears only to be erosion and excoriation. Examples of primary and secondary efflorescence in patients with dermatitis herpetiformis can be seen in ( Figure 1) [1,4,8,9]. A. Plaque and papules on the elbow, excoriation and veiscles. B. Edema, plaques, and papules on the forehead and sclap. ...
... C Excoriation and hemorrhagic crust on the knee. D. Cesicles on reddish back [9]. ...
... Barley, wheat and rye and all kinds of products must be removed, but pure oats, rice and corn can still be consumed. This is evident in patients who have better progression lesions after a GFD, although not in the recent time [10]. ...
... Cetirizine is provided to reduce the symptoms of itching at a dose of 10 mg/day. Combined treatment with nutritionist for a GFD is needed [10]. In this patient, symptoms are reduced in 5 days after initial treatment and complete resolution of lesions with residual hyper-and hypopigmented macules and patches in 8 weeks. ...
Full-text available
Dermatitis herpetiformis (DH) is a rare autoimmune bullous disease characterized by intensely pruritic, chronic, and recurrent vesicles on extensor surfaces such as the elbows, knees, and buttocks. There is a genotype relationship with HLA-DR3, HLA DQw2, discovered about 80-90% of cases. Immunoflorescence is the gold standard for diagnosis, but serologic testing can help if immunofluorescence result is negative. On histopathological examination, at the tips of papillary dermis, a collection of neutrophils are found and granular immunoglobulins A. Dermatitis herpetiformis associated with gluten intolerance (celiac disease), although the mechanism is not fully understood. Patients with gluten free diet will reduce of this disease both in the skin and intestinal tract, thereby reducing risk of lymphoma progression. Dapsone is the main therapy, but it require monitoring side effects.
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Transglutaminase 2 (TG2) is well characterized as the main autoantigen of celiac disease. The ability of TG2 to deamidate and crosslink gluten peptides is essential for the gluten-dependent production of TG2 specific autoantibodies. In patients with primarily extraintestinal manifestation of gluten sensitivity the repertoire of autoantibodies may be different. In dermatitis herpetiformis (DH), TG3 appears to be the target autoantigen whereas in gluten ataxia (GA) autoantibodies reactive with TG6 are present. A functional role for TG3 and TG6 in these diseases has yet to be described. It is also not known whether these enzymes can use gluten peptides implicated in the pathology as substrates. We here report that similar to TG2, TG3 and TG6 can specifically deamidate gluten T cell epitopes. However, the fine specificities of the enzymes were found to differ. TG2 can form covalent complexes with gluten by iso-peptide and thioester bonds. We found that both TG3 and TG6 were able to complex with gluten peptides through thioester linkage although less efficiently than TG2, whereas TG6 but not TG3 was able to form iso-peptide linked complexes. Our findings lend credence to the notion that TG3 and TG6 are involved in the gluten-induced autoimmune responses of DH and GA.
The teeth of 30 adult patients with dermatitis herpetiformis and 66 sex- and age-matched healthy controls were examined for dental enamel defects. Sixteen of the patients (53%) with dermatitis herpetiformis, opposed to only one (2%) of the healthy controls (p less than 0.001), were found to have coeliactype permanent-tooth enamel defects. The grades of these defects were milder than those described for severe coeliac disease. There was no correlation between the degree of enamel defects and jejunal villous atrophy. The present finding of frequent coeliactype dental enamel defects in adults with dermatitis herpetiformis suggests that these patients were already suffering from subclinical gluteninduced enteropathy in early childhood, at the time when the crowns of permanent teeth develop.
Because dermatitis herpetiformis (DH) is seen as an associated disorder of celiac disease or a complication that is related, the same clinical diet is recommended. There is strong evidence that the changes in the intestinal mucosa and the immunologic findings in the majority of patients are identical to those found in celiac disease. Second, gluten has been found to be closely related to the skin rash that occurs with dermatitis herpetiformis (1,2).
24 patients with dermatitis herpeti-formis (D.H.) have been treated with a gluten-free diet (G.F.D.) for periods varying from four months to five years. Of 20 patients who have taken a G.F.D. for a year or longer, 16 (80%) have been able to stop taking dapsone or substantially reduce the dose of the drug. 10 patients have been able to stop taking dapsone completely and are now free of all skin lesions. No patient was able to reduce his dapsone requirements before five months, and 1 patient took twelve months to do this. The length of time taken to be able to stop dapsone completely varied from eight to forty-eight months. In another group of 20 patients with D.H. who did not take a G.F.D. but have been followed up for a similar length of time, there was no significant alteration in dapsone requirements. Reintroduction of gluten in 4 patients who were completely free of skin lesions on a G.F.D. produced irritation and blisters within a week in 3 and within three weeks in the fourth. Dapsone was required for the immediate control of these lesions, which were subsequently controlled again by a G.F.D. alone. Electron-microscopic studies have shown subepidermal membrane-bound vacuoles in clinically normal skin in all 7 patients studied whose eruption was being controlled by dapsone, but these vacuoles were not present in any of the 6 patients whose eruption was controlled by a G.F.D. alone. These results show that the skin lesion in D.H., like the gut lesion, is gluten dependent and that both lesions are part of the same disease process. The length of time for patients to become free of skin lesions after beginning a G.F.D. is stressed, and probably accounts for the previous reports in which it has been stated that the skin lesions are not influenced by a G.F.D.
One hundred and fifty-two patients in whom a diagnosis of dermatitis herpetiformis was made at St John's Hospital for Diseases of the Skin, London, during 1950-85, were followed 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. Thirty-eight deaths occurred under the age of 85, slightly fewer than expected on the basis of national general population rates [standardized mortality ratio (SMR) = 87; 95% confidence interval (CI) 61-119]. All-cause mortality was somewhat lower in patients who had followed a gluten-free diet (SMR = 51; 17-120) than in those who had not (SMR = 97; 66-136), but the difference in SMRs was not significant (P = 0.3). Cancer mortality was non-significantly below expectations from national rates (SMR = 72; 31-142), but cancer incidence was significantly increased [standardized registration ratio (SRR) = 394; 180-749]. No particular cancer site accounted for the cancer incidence excess. One death occurred from cancer of the small intestine (SMR = 4953, P = 0.04), and one lymphoma was incident (SRR = 1555, P = 0.12). Increased risks of these malignancies have previously been found to be associated with coeliac disease (which is present in many patients with dermatitis herpetiformis), and with dermatitis herpetiformis, respectively. Mortality from ischaemic heart disease (IHD) was significantly below national rates (SMR = 37; 95% CI 12-86), and was similar in patients who had followed a gluten-free diet and those who had not.(ABSTRACT TRUNCATED AT 250 WORDS)
Dermatitis herpetiformis (DH) is seen most commonly as a pruritic, papulovesicular eruption in young children or adolescents. Differentiation from other bullous diseases of childhood may be difficult. We report the first case of an adolescent in whom pruritic, palmar, purpuric macules and papules were the only manifestations of DH. The patient later developed typical vesiculobullous extensor lesions and symptomatic gluten-sensitive enteropathy (GSE). All lesions and GSE symptoms resolved with dapsone and a gluten-free diet. Our purpose is to illustrate an unusual presentation of pediatric DH.
In celiac disease, the ingestion of gluten-containing cereals, such as wheat, rye, and barley, results in small-bowel mucosal inflammation and villous atrophy with crypt hyperplasia. The prevalence of the condition may be as high as 1% in the adult population. The disease can also embrace various extraintestinal manifestations, of which dermatitis herpetiformis is the best known. Earlier, dermatitis herpetiformis was considered a skin disease occurring often concomitantly with celiac disease. At present, a body of evidence shows that dermatitis herpetiformis is a cutaneous manifestation of celiac disease, and affects approximately 25% of patients with celiac disease. Both conditions can appear in the same family and are closely linked to HLA class II locus in chromosome 6; 90% of patients have HLA DQ2 and, almost all the remainder, HLA DQ8. All patients with dermatitis herpetiformis have at least some-degree of mucosal inflammation or lesion consistent with celiac disease. The etiology of celiac disease in not fully understood, but tissue transglutaminase seems to be the predominant autoantigen both in the intestine and the skin. Serum antibodies against tissue transglutaminase can be used in the serologic screening and follow-up of dietary compliance of patients with celiac disease. Gluten-free diet is essential in the treatment of both conditions, and oral dapsone is usually needed in newly detected dermatitis herpetiformis in order to alleviate symptoms. Oral mucosal lesions, alopecia areata, and vitiligo probably occur more frequently in patients with dermatitis herpetiformis than in the general population. By contrast, the reported association of celiac disease with psoriasis seems to be coincidental.