Pemphigus is a severe skin disease. Its characteristics include superficial vesicles and bullae that rupture easily; it is a blistering disorder resulting from loss of integrity of normal intercellular attachments (acantholysis) within the epidermis and mucosal epithelium. Pemphigus is an autoimmune disease.
Etiology and pathogenesis
The cause of this disease is still not clearly known; at present time, it is classified as an autoimmune disorder because patient’s serum yields antibodies to intercellular cement substance in stratum spinosum of epidermis of skin and mucose membrane (also known as pemphigus autoantibody) in indirect immunofluorescence examination. The main thing here is IgG, serum s pemphigus autoantibody titer is proportional to the severity of disease. The area of autopemphigus antibody reacts is the affected area where loss of intercellular attachments (acantholysed area). This autoantibdy interferes with the normal function of desmoglein 3 which responsibles for desmosome adhesion.
The majority of individuals who develop pemphigus are in the fourth to sixth decades of life, and men and women are affected equally. There are four clinical and pathologic variants: pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceus and pemphigus erythematosus.
(1) Pemphigus vulgaris: primary lesions are superficial vesicles and bullae that rupture easily, leaving shallow erosions covered with dried serum and crust. The fluid from the ruptured bullae is clear in the beginning, later with blood and it gives unpleasant smell. The superficial layer is easily slough-off under external force or slightly pressure on a large bulla will cause fluid in the bulla to spread radially – this is known as positive Nikolsky sign. The pathognesis of this situation is the loosening and separation of stratum spinosum of epidermis and leads to lacking of intercellular attachment.
It causes slight itching, leaves behind pigmentation after treatment, no scar.
About 80% patients may involve the oral cavity, external genitalia and eye conjunctiva, oral membrane erosions are easily bleeding regions with significant burning pain which normally is an early symptom of this disease before skin involvement appears.
It can affect any region of the body, especially back, axilla and groin.
Bullae appear at whole body, the affected region has erosions and more prone to secondary infections, fever, whole body weakness and etc.
(2) Pemphigus vegetans: usually affects scalp, axilla, chest, back and others. It starts with loose bullae, positive Nikolsky sign, then rupture and become erosions and presents with large, moist, verrucous (wartlike), vegetating plaques studded with pustules which superficially dirty, scarring and has bad smell.
It often involves oral cavity, nasal cavity, labia minora, anal area and other membranes area. Bullae are easily rupture and become erosions.
ymptoms for own-revealing are not significant, sometimes along with high fever and other general symptoms. The course of disease always changes, sometimes light and sometimes severe, it often continuous for many years.
Pemphigus vegetans can be divided into two forms: 1. Severe (Neumann) form which quite similar to pemphigus vulgaris. 2.Light (Hallopeau) form: no significant blisters, only small pustules and vegetative plaques at flexural surfaces. Clinically, it is difficult to distinguish from pyoderma vegetans; if uses direct immunofluorescence examination, one can see IgG deposition between cells of epidermis.
(3) Pemphigus foliaceus: bullae are so superficial that only zones of erythema and crusting, site of previous blister rupture with fatty scarring, are usually present on physical examination. It also has regional skin slough-off and fishy bad smell. Sometimes, the affected area presents erythema swelling and leaf-shaped scar which are similar to ----------. Positive Nikolsky sign.
Skin damage usually presents at scalp, facial, chest and back region. The membrane of oral cavity is less affected. The degree of itching and general symptoms vary all the time.
(4) Pemphigus erythematosus: Is also known as Sener-usher syndrome and pemphigus seborrhoicus, is a less severe form of pemphigus foliaceus. Symptoms: erythema, small bullae, Nikolsky positive with repeated scalling, scarring process; usually affects scalp, cheeks, chest and back region. It selectively involves the malar area of the face in a lupus erythematosus-like fashion. The scalp, chest and back region often have fatty scarring and picture of dermatitis seborrheica.
It usually less often spreads to the whole body, good prognosis, does not involve membranes, whole body symptoms are light. This disease can progress into pemphigus foliaceus.
1. Loose skin with big bullae, appear scarring or erosions.
2. Membranes especially of oral cavity is an early symptom of pemphigus.
3. Postitve Nikolsky sign.
4. Histological analysis of bulla reveals Tzanck cells.
5. Immunofluorescence examination.
a. Direct method: epidermal cells of affected area have deposition of IgG and C3. Besides, about 25-30% of patients showed deposition of IgA and IgM. At the unaffected area, about 60% has IgG and C3 deposition.
b. Indirect method: in patients serum, about 100% cases has pemphigus autoantibody. The autoantibody and level of disease is proportionate.
Pemphigus erythematosus has the above mentioned features with additional positive antinuclear antibody, bridges between epidermis and dermis have deposition of IgG and C3 which is quite similar as lupus erythematosus.
6. Pathogenesis: stratum spinosum of epidermis has acantholysis type of bullae, one can see disconnection of bridges under micoscpe and leads to lost of intercellular attachment. This is also the basic pathogenesis of Nikolsky sign.
Tzanck cells present in bullae.
Formation of chorionic villi at the base of bullae, slight infiltration at the dermis layer.
Pemphigus vegetans has the above mentioned features, and additional with hypertrophic of epidermis which gives a false picture of epitheloid tumor; in epidermis, there is presence of small pustules that contain lots of eosinophils.
Acantholysis of pemphigus folioceus and pemphigus erythematosus happens at superficial layer of epidermis (stratum corneum or stratum granulosum).
Western treatment: 1.Corticosteroids 2.Immunosuppression drugs.
TCM treatment: a. Poison heat form: clearing of heat, neutralizes poison, cooling the blood. b. Heart fire, spleen moist form: clearing of heart and eliminating fire. c.Injured of Qi & Yin form: benefits Qi and support Yin, clearing of poison remnants.
[Gufang treatment results] There were 560 cases adopted TCM therapy with 542 cases of completely recovery and 18 cases of shown effectiveness.