Article Type : Case Report
Authors : Putra Gofur NR1, Putri Gofur AR, Soesilaningtyas, Putra Gofur RR, Kahdina M and Martadila Putri H
Keywords : Duhring’s disease; Vesibolous; Herpetiformis; Autoimmune
Introduction: Duhring
disease is an autoimmune disease. The clinical manifestation may vary. Rash is
polymorphic, mainly in the form of vesicles that are arranged in groups and
symmetrical. Symptoms vary in intensity but many sufferers complain of a very
severe itching, burning and burning sensation with predilection, especially on
the extensor surface of the upper and lower limbs, elbows, knees, scalp, back
of the neck (nape), back, shoulders, sacrum area and butt. The face and groin
can also be affected.
Discussion: 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. Symptoms vary in intensity but many sufferers complain of intense
itching, burning and intense burning. Which is often without precedence overt
mainstream lesions within hours. Lesions that appear on the head, face, thighs
and flexor area are seen in more severe cases. Pustules are very rare except
when secondary infection occurs. Vesicles that arise especially if they are on
the hands may be hemorrhagic. Lesions that appear and disappear continuously,
will cause hypopigmentation and hyperpigmentation.
Conclusion: The main
lesions of dermatitis herpetiformis are clusters of erythematous papules,
urticarial plaques or most commonly vesicles that appear in groups but may also
appear in groups. Papulovesicles 'herpetiformis' 3-6 mm in size with an
erythematous base are characteristic of the disease, large bullae are rare.
Dermatitis
herpetiformis or also known as duhring disease is a rare autoimmune
vesicobulosa disease, specific and recurrent chronic in nature and has a
relationship with celiac disease and gluten-sensitive enteropathy. The rash is
polymorphic, mainly in the form of vesicles that are arranged in groups and
symmetrical. Symptoms vary in intensity but many sufferers complain of a very
severe itching, burning and burning sensation with predilection, especially on
the extensor surface of the upper and lower limbs, elbows, knees, scalp, back
of the neck (nape), back, shoulders, sacrum area and butt. The face and groin
can also be affected. Dermatitis Herpetiformis can affect any age, but appears
more often for the first time in young adults between the ages of 30 and 40,
more often in men than in women [1,2].
Dermatitis
herpetiformis (DH) is a chronic condition that has problems with severe
itching, skin rashes, and blisters that are a manifestation of enteropathy, due
to intolerance to gluten (gluten-sensitive enteropathy) or commonly referred to
as celiac disease, DH or what is also known as Dühring's disease, affects or
occurs in approximately 10% of patients with celiac disease. The term
herpetiformis in DH tax appears on her skin blisters or lumps that appear in
groups, the address of the blisters in herpes, but these blisters are not
caused by herpes viruses [3,4].
The
initial symptom that a patient with DH may notice or feel is skin that feels
like burning or stinging in several locations. Then, after these symptoms
appear, small lumps appear that cluster and feel very itchy. These bumps can
have distinct lines, such as: blisters, a wound that has fluid in it, deep
wounds of urticaria, the wound is rising, red, thickened plaques are skin
symptoms in dermatitis, scabies and papular urticaria, erosion or crusting due
to scratching of itchy skin [5,6].
At
this stage, the clinical skin manifestations of DH are often mistaken for
eczema. Often, the clinical skin manifestations of DH appear in certain areas.
It is possible that these skin manifestations also appear on the body, neck,
around the thighs, and even appear on tooth enamel. Generally, these skin
manifestations appear on both sides of the body or have a symmetrical
distribution. The blisters on DH generally dry up and heal within 1-2 weeks.
However, new blisters appear at the same location, so that the blisters on DH
appear as chronic blisters that don't go away. In the manifestations of healed
DH skin, post-inflammatory hypopigmentation or hyperpigmentation may appear [7,8].
Clinical
manifestation of duhring’s disease
The main lesions of dermatitis herpetiformis are clusters of erythematous papules, urticarial plaques or most commonly vesicles that appear in groups but may also appear in groups. Papulovesicles 'herpetiformis' 3-6 mm in size with an erythematous base are characteristic of the disease, large bullae are rare [1,4]. 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].
Figure
1: Dermatitis herpetiformis’s clinical manifestasion.
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].
Symptoms vary in intensity but many sufferers complain of intense itching, burning and intense burning. Which is often without precedence over mainstream lesions within hours. Lesions that appear on the head, face, thighs and flexor area are seen in more severe cases. Pustules are very rare except when secondary infection occurs. Vesicles that arise especially if they are on the hands may be hemorrhagic. Lesions that appear and disappear continuously, will cause hypopigmentation and hyperpigmentation (Figure 2) [1,3,8].
Figure 2:
Wide eruption with papules, vesicles, and crusta on the back (A) Vesicles and
bullaes with clear margen, some erotion and hiperpigmentation, vesicles are in
annular pattern (B). Papules, veiscles, and crustae on the knee (C) [1,4].
A rare clinical manifestation is purpura on the palms. This clinical picture is more common in children, but several cases in adults have also been reported. Clinically purpura appears on the hands, as in Figure 3, and / or the soles of the feet. It is suspected that trauma is an etiological factor. No dorsal surface involvement of the hands and feet was reported [1,4].
Figure 3:
Purpura on the fingers of patient suffering from dermatitis herpetiformis [4].
Mucosal
involvement is very rare in dermatitis herpetiformis. Many studies reported a
lack of information on oral lesions by direct immunofluorescence examination of
the oral mucosa. Dermatitis herpetiformis is associated with various
conditions, such as celiac disease and autoimmune connective tissue disease
which may cause lesions in the mouth itself, thus there are many possible
causes of mucosal lesions in dermatitis herpetiformis patients [1,8].
Although
limited, several studies suggest that oral mucosal lesions are associated with
the diagnosis of dermatitis herpetiformis, where there are vesicles,
erythematous macules and erosions of the oral mucosa as shown in Figure 4,
including the tongue. This condition causes painful swallowing and a burning
sensation
[8].
It is important to note, however, that celiac disease itself is associated with apoptosis of the mouth and mucosal lesions. Therefore, the relationship between oral mucosal lesions in dermatitis herpetiformis remains unclear. Finally, dental abnormalities have been explained in patients with celiac disease and patients with dermatitis herpetiformis. Defects in enamel on permanent teeth, as in Figure 4, are seen in children and adults with celiac disease and dermatitis herpetiformis. Horizontal grooves, defects in tooth enamel color and large holes in the enamel are characteristic features of teeth in patients with dermatitis herpetiformis [1,8]. Although not found in all patients with dermatitis herpetiformis, they are associated with gastrointestinal abnormalities caused by gluten sensitivity. From the study, patients with dermatitis herpetiformis also found symptoms of steatorrhea (20-30% of patients) [2].
Figure 4: Multiple erosion on the lips [8].
Dermatitis
herpetiformis has several similarities with the following diseases, so that
these diseases are often used as a differential diagnosis rather than
dermatitis herpetiformis, they are pemphigoid vulgaris, pemphigoid bullosa, and
linear IgA dermatosis.In pemphigus vulgaris, the main cause of this disease is
autoimmune. The prevalence of patients who most often appear is between the 3-6
decades (30-60 years). The main complaint of patients is usually not itching.
And on physical examination found loose-walled bullae and if there is a
rupture, crusts can arise which persist for a long time [1].
The clinical picture is shown in Figure 5. In 60% pemphigus vulgaris, there are abnormalities in the oral mucosa. Nikolsky's sign for pemphigus vulgaris is positive. This disease predilection is general (can appear in all parts of the body). If a direct immunofluorescence examination is performed, IgG and its complement can be seen in the epidermis. This disorder does not involve gastrointestinal disorders, disorders related to gluten sensitivity, and no involvement of HLA. Therapy of this disease is carried out using corticosteroids and cytostatics [1,7,10].
Figure
5: Patient
with pemphigus vulgaris on sacrum area. It is shown krusta [10].
In pemphigoid bullosa, the main cause of the disease is suspected to be autoimmune. The prevalence of sufferers is in old age (over 60 years). The main complaint of patients is usually not itching. And on physical examination, a tense walled bullae was found as shown in Figure [15]. In 10-40% of pemphigoid bullosa, there are abnormalities in the oral mucosa. The Nikolsky sign on pemphigoid bullosa is negative. The predilection for this disease is the abdomen, flexor arms, groin and medial legs. If a direct immunofluorescence examination is performed, it can be seen that IgG is shaped like a band on the basal membrane. This disorder does not involve gastrointestinal disorders, disorders related to gluten sensitivity, and no involvement of HLA. Therapy of this disease is carried out using corticosteroids [7,11].
Figure
6: Patients with pemphigoid bullose [11].
For Linear IgA dermatosis as shown in Figure 6, differentiation from dermatitis herpetiformis will be difficult if it only relies on clinical and histopathological examinations. Additional examination in the form of immunological examination will be able to distinguish these two diseases [7,12].
Figure 7:
Linear IgA dermatosis in children has a clinical picture similar to dermatitis
herpetiformis so it is difficult to distinguish it. The diagnosis is confirmed
by performing an immunological examination [12].
Authors
in other literature have also found that dermatitis herpetiformis was diagnosed
as compared to scabies, and hypersensitivity was due to insect bites. For
scabies, the morphology of the scabies is papules, vesicles and a scabies
tunnel as a pathognomonic sign as shown in Figure 7. The distribution of
scabies is usually in the gaps of the fingers, wrists, ulnar region of the
forearm, genitals and lower abdomen. It stands out is that scabies definitely
responds to anti-scabies therapy [7].
Figure 8:
Clinical sign of scabies [13].
In
the field of pediatric dermatology as shown in Figure 8, it is not uncommon for
dermatitis herpetiformis to be diagnosed differently with atopic dermatitis.
This is because when there are complaints of intense itching on the skin of the
child, which is accompanied by chronic inflammatory lesions it is usually due
to atopic dermatitis, and rarely due to dermatitis herpetiformis, which is the
most classic manifestation of celiac disease. The difference between the two is
that the history of atopic dermatitis refers to a family and / or personal
history of atopy. The prevalence of atopic dermatitis was 12.8% of all childhood
diseases, while DH was 0.1% of all diseases in children. Atopic dermatitis the
prevalence can be at any age, but 50% more occurs in the first 6 months of life
[13].
Whereas in dermatitis herpetiformis, the infantile form begins to appear in the second year of life. The lesion type atopic dermatitis is eczematous, whereas in dermatitis herpetiformis the lesions are typically erythematous and infiltrative lesions with flat, circinate surfaces with easily visible vesicles. The distribution of lesions in atopic dermatitis in children in the first year of life is often on the face, and in the following, on the flexor surfaces of the elbows and knees. Whereas in children suffering from dermatitis herpetiformis, the lesions are usually centripetal, often on the chest, especially the scapular region and the base of the trunk, especially the triangular area of the extensor surface of the forearm with a base on the elbow. In atopic dermatitis, the skin prick test for atopy is often positive, and in atopic dermatitis there is no improvement in the introduction of a gluten-free diet [1,7,14] (Figure 9).
Figure 9: Comparison of atopic dermatitis and dermatitis herpetiformis (Left)
Pediatric patients suffering from atopic dermatitis, (Right) pediatric patients
suffering from dermatitis herpetiformis [14].