Blisters on the skin and mucous membranes are caused by an immune defect doctors today

Is there an immune defect behind it??blisters on skin and mucous membranes are caused by an immune defect doctors today

Blisters on the skin or mucous membranes can have various causes, one of which is the so-called bullous autoimmune dermatoses (BAID). When the body creates antibodies against the structures of the skin, it peels and blisters. The group of blistering autoimmune diseases includes several skin conditions that can occur at any age. Bullous pemphigoid (BP) is most common, affecting mostly elderly patients.

Pemphigus vulgaris

Pemphigus vulgaris occurs in Germany with an incidence between 0.5 and one case per 1 million [1]. The disease manifests itself most frequently in the fourth to sixth decade of life. Adhesion loss triggered by autoantibodies is in-traepidermal (suprabasal). The blisters are therefore usually flaccid. They burst quickly. Leading to painful erosions (Fig. 1). Blistering does not only occur on the skin, mucous membranes are also affected [5]. As a rule, pemphigus vulgaris first appears on the oral mucosa (Fig. 2).

Other predilection sites are the scalp and trunk. The intact epidermis can be pushed off with the finger (Nikolski I sign). Some drugs can trigger pemphigus in rare cases, even after years of good tolerance (Table 2) [11]. A paraneoplastic form is also known.

Bullous pemphigoid (BP)

BP is the most common representative of BAID (Table 1). Disease incidence ranges from 2.5 to 42.8 cases per 1 million [1]. The incidence peak is between 70. and 80. Age reached [1].

Blister formation proceeds subepidermally. The manifestations on the skin can be very polymorphic. On physical examination, symmetrically distributed, pruritic, bulging blisters are usually seen on reddened or healthy-appearing skin (Fig. 3) [7, 14]. Predilection sites are the flexor sides of the extremities. Secondarily, encrusted erosions develop. Sometimes prurigo or eczema-like skin lesions appear without blistering (Fig. 4) [7, 14]. The mucous membranes are affected in 10-30% of cases. The course of BP is usually self-limiting. Rarely, this blistering condition is triggered by tumors or drugs (Table 2).

Associated diseases

A connection between BP and some neurological and psychiatric diseases such as Parkinson's disease, multiple sclerosis, apoplexy and dementia has been established [2, 3, 16], but there is no therapeutic consequence.

Other BAID

Mucosal pemphigoid (MMP) primarily affects the mucous membranes. Scarring often occurs, which can cause irreversible and distressing complications (z. B. Laryngeal stenosis) [7]. The course of MMP is highly chronic and often unfavorable [6].

Dermatitis herpetiformis Duhring (DHD) is the cutaneous manifestation of celiac disease. This chronic disease of the small intestine is usually mild or asymptomatic. It occurs in one to five cases per 1 million [7]. The incidence peak in Europe is between the 25. and 55. Year of life. The plump, herpetiform vesicles appear over areas of skin that are subject to prere or tension, such as the elbows, knees, or buttocks (sacrogluteal). Vesicles are often absent; instead, crusted erosions, papules, or excoriations triggered by intense itching are found (Fig. 5) [12]. DHD is often associated with other autoimmune diseases (z. B. Autoimmune thyroiditis, diabetes mellitus, pernicious anemia) [7].

Diagnostics

Even if BAID is suspected, a routine laboratory is helpful. Blood count changes such as eosinophilia may indicate BP. Signs of digestive weakness (malabsorption) occurring in the process, z. B. macrocytic or iron deficiency anemia, as well as vitamin D deficiency, are typical of DHD. The physician should ask about the intake of possibly triggering drugs (Table 2) [6, 11]. The final diagnosis is made by the dermatologist. In addition to history and physical examination, it is based on the evaluation of skin samples, i.e., dermatohistology (direct immunofluorescence, or IF) and autoimmune serology (indirect IF, BP180 and BP230 ELISA) [13, 9, 14, 5].

Differential diagnosis

For the differential diagnosis, allergic, blistering skin reactions (bullous drug exanthema such as erythema multiforme, Stevens-Johnson syndrome, toxic epidermal necrolysis) must be excluded above all. Recently prescribed medications (period: one to eight weeks) are particularly suggestive of an acute drug reaction.

Infections caused by staphylococci may also be associated with blistering [15]. Acutely appearing blisters, usually visible on the opposite skin surfaces, and – in younger patients – yellowish crusted erosions indicate bullous impetigo contagiosa. Smaller vesicles instead of blisters are typical for viral infections. In herpes zoster, for example, grouped vesicles confined to dermatomes are typical.

If mucosal erosions occur without other skin involvement, aphthae, candidiasis, erosive lichen, or ill-fitting dentures should be ruled out. Frostbite or burns to the skin can also lead to blisters. Self-inflicted skin lesions (artifacts), mechanical blisters, or reactions to insect bites should also be considered [12]. Isolated blisters on the shins may appear in association with diabetes mellitus (bullosis diabeticorum) or indicate inadequate diuretic therapy (tension blisters). In elderly patients in whom eczema, prurigo, or scabies, for example, are suspected, the physician must rule out nonbullous BP [7]. He must also note that infantile skin reacts particularly with blistering in different dermatoses [12].

Therapy

With the exception of DHD, the treatment of choice is systemic immunosuppression. In mild BP, topical monotherapy with clobetasol propionate is exceptionally recommended [13]. How to avoid systemic side effects. A disadvantage, however, is the additional nursing effort. Long-term use can also lead to severe skin atrophy [6].

1. Pruritic, eczematiform, or bullous erosive dermatosis from 70. year of age => V. a. BP. 2. Similar dermatosis in younger patients and over the large joints => V. a. DHD. 3. Painful, oral erosions with or without blisters on the skin between the 40. and 60. Year of life => V. a. Pemphigus vulgaris or MMP.

Initiation and tapering of systemic immunosuppression and a change in therapy should be performed by an experienced dermatologist. In BP or pemphigus, systemic therapy consists of prednisolone or methylprednisolone [4, 8, 9]. At high doses, the glucocorticoid should be combined with another steroid-sparing immunosuppressant to limit side effects [4, 8, 9]. Dose reduction of glucocorticoids should be gradual (by "logarithmic reduction") [4, 8, 9]. For DHD, dapsone is the therapy of choice, supplemented by a gluten-free diet [8].

Steroid-sparing immunosuppressants

Methotrexate: Initially, one laboratory troll per month is necessary; later, bimonthly to trimonthly testing is sufficient. In more severe concomitant diseases, these should also be done more frequently if necessary. CAVE: Daily (instead of weekly) use of methotrexate may have lethal consequences. Caution in dementia!
Azathioprine: Because of very common liver toxicity in the elderly, biweekly checks are recommended for the first two months (then every two to three months).
Mycophenolate: The dose must be adjusted for Asians resp. Africans are significantly reduced or increased, respectively. Rule of thumb: one gram for Asians, two grams for Caucasians, three grams for Africans (daily).
Dapsone: Obligatory consequences of therapy are hemolysis and methemoglobin formation, which – due to other factors – can lead to ischemic complications. Laboratory checks should be weekly for the first three months, and later every two to three months.

Systemic drugs and drug interactions

Serious complications can be caused by immunosuppressant elimination inhibiting drugs. The most common error may be concomitant administration of methotrexate with proton pump inhibitors. Myelotoxicity, hepatotoxicity, and nephrotoxicity of methotrexate are thereby potentiated. This can be avoided by H2 antihistamines (z. B. Avoid ranitidine 300 mg/d) instead of proton pump inhibitors. Another potentially life-threatening combination is azathioprine with allopurinol or aminosalicylic acid derivatives such as mesalazine and sulfasalazine, respectively. The physician should also avoid the concomitant administration of azathioprine. Avoid ACE inhibitors – because of their increased myelotoxicity. The same applies to the combinations of mycophenolate and aciclovir and methotrexate and NSAIDs over a prolonged period of time-otherwise there is a risk of severe bone marrow suppression, aplastic anemia, and gastrointestinal toxicity. [6].

Geriatric aspects

In elderly patients, concomitant diseases such as impaired liver and kidney function are the biggest problem. In dementia, medication adherence and compliance is difficult. Limited self-care promotes superinfections. Elderly people are also often immobile. Have a reduced sense of thirst. In erosions, transepidermal water loss is greater, which can lead to significant dehydration [6]. Here, adequate pain relief, water and electrolyte administration, and infection prophylaxis are important. Certain triggers, such as suspicious medications [13], UV radiation, and neoplasms, should be eliminated if possible. As part of infection prophylaxis, physicians should check vaccination status and make up any missing vaccinations [10]. Screening examinations (X-ray thorax, upper abdominal sonography, hemoccult, serum PSA) in the direction of neoplasia or. of chronic infections such as tuberculosis are recommended. During systemic treatment, laboratory parameters must include. Blood prere levels are monitored regularly. Because of the risk of osteoporosis with steroid therapy, prophylactic substitution of vitamin D and calcium is recommended [17].

– Prompt dermatologic consultation is required for blistering or erosion of unclear etiology. – In all chronic pruritic inflammatory dermatoses in the elderly, BP should be excluded. – With most, well treated BAID the prognosis is favorable.

1. Alpsoy E, Akman-Karakas A, Uzun S (2015) Geographic variations in epidemiology of two autoimmune bullous diseases: pemphigus and bullous pemphigoid. Arch Dermatol Res 307:291-298

2. Bastuji-Garin S, Joly P, Lemordant P et al (2011) Risk factors for bullous pemphigoid in the elderly: a prospective case-control study. J Invest Dermatol 131:637-643

3. Brick KE, Weaver CH, Savica R et al (2014) A population-based study of the association between bullous pemphigoid and neurologic disorders. J Am Acad Dermatol 71:1191-1197

4. Eming R, Sticherling M, Hofmann S et al (2015) S2k guideline on the therapy of pemphigus vulgaris / foliaceus and bullous pemphigoid. J Dtsch Dermatol Ges 13:833-844

6. Horvath ON, Jankaskova J, Walker A, Sardy M (2015) Pemphigoid diseases. Autoimmune diseases of old age. Dermatologist 66:583-588

7. Kneisel A, Hertl M (2011) Autoimmune bullous skin diseases. Part 1: Clinical manifestations. J Dtsch Dermatol Ges 9:844-856

8. Kneisel A, Hertl M (2011) Autoimmune bullous skin diseases. Part 2: diagnosis and therapy.

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