Abstract
Pemphigus is the most common and also potentially the most severe immunobullous disorder involving the skin and mucous membranes. The disease has a worldwide distribution with an incidence of 0.1 to 0.5 per 100,000 population.
Keywords
Pemphigus vulgaris (PV), Pemphigus foliaceus (PF), Pemphigus vegetans, Pemphigus erythematosus, Nikolsky’s sign, desmoglein 1 (DSG1) and desmoglein 3 (DSG3).
Introduction
Autoimmune disease, characterised by acantholysis, induced by deposition of intracellular autoantibodies.
Pathogenesis of Pemphigus
Circulating IgG autoantibodies targeting antigens in the intercellular attachment plaques (desmosomal complexes) of the epidermal keratinocytes, identified as desmogleins, lead to acantholysis and cell separation, and intraepidermal blister formation. The reason for this anomalous antibody activity is not known. HLA linkage analysis suggests that there may be some genetic predisposition.
Classification of Pemphigus
- Pemphigus vulgaris – Splitting just above the basal layer
- Pemphigus foliaceus – Superficial epidermal split with short-lived blisters.
- Pemphigus vegetans – Split is suprabasal
- Pemphigus erythematosus – Split in granular layer or just below stratum corneum (2)
Clinical Features
(a) Skin lesions – Development of flaccid, fragile blisters which rupture to form painful erosions. Application of tangential pressure on normal skin in the pretibial region gives rise to new bulla (Nikolsky’s sign) or to existing bulla causes spread of the bulla (bulla spread sign). Site – Scalp, face, flexures and trunk.
(b) Mucosal lesions mostly in oral mucosa – Painful erosions with shedding of mucosa which looks ragged. Skin and mucosal infections get secondarily infected. (2)
- Pemphigus vulgaris (PV) is a mucocutaneous blistering disease that predominantly occurs in patients >40 years of age. PV typically begins on mucosal surfaces and often progresses to involve the skin. This disease is characterized by fragile, flaccid blisters that rupture to produce extensive denudation of mucous membranes and skin. The mouth, scalp, face, neck, axilla, groin, and trunk are typically involved. PV may be associated with severe skin pain; some patients experience pruritus as well. Lesions usually heal without scarring except at sites complicated by secondary infection or mechanically induced dermal wounds. (3)
- Pemphigus foliaceus (PF) is distinguished from PV by several features. In PF, acantholytic blisters are located high within the epidermis, usually just beneath the stratum corneum. Hence, PF is a more superficial blistering disease than PV. The distribution of lesions in the two disorders is much the same, except that in PF mucous membranes are almost always spared. Patients with PF rarely have intact blisters but rather exhibit shallow erosions associated with erythema, scale, and crust formation. Mild cases of PF can resemble severe seborrheic dermatitis; severe PF may cause extensive exfoliation. Sun exposure (ultraviolet irradiation) may be an aggravating factor. PF has immunopathologic features in common with PV. (3)
- Pemphigus vegetans – Heaped up vegetating peripherally spreading lesions. Sites – Groins, axillae, angles of mouth. Mucosa may be involved. (2)
- Pemphigus erythematosus – Variant of pemphigus foliaceus but less severe. Dry, hyperkeratotic, scaly lesions on face (malar regions) (2)
- Pemphigus variants – (a) Paraneoplastic – Polymorphic skin lesions, painful oral mucosal lesions, associated with lymphoma, thymoma. (b) Drug-induced – Rifampicin, penicillamine, captopril Cl. Fs. Like PF or PV. (2)
Investigation
- Tzanck smear from erosion or floor of bulla reveals acantholytic cells.
- Direct immunofluorescence shows intracellular deposits of IG, giving a fishnet
- Serology – Presence of IG antibodies with titres correlating with disease activity. (2)
- The diagnosis of PV is based on clinical, histopathologic, and laboratory studies. An H&E biopsy should be performed on lesional skin, which can be analysed to show the characteristic histopathologic findings.
- Enzyme-linked immunosorbent assay (ELISA) testing, which is commercially available, will show serum IgG against Dsg 1, Dsg 3, or both in most samples.
- desmoglein 1 (DSG1) in pemphigus foliaceus and desmoglein 3 (DSG3) and/or DSG1 in pemphigus vulgaris.
Homoeopathic management
As we know pemphigus like autoimmune disorders having final line of treatment is only based upon oral steroidal medications and immuno-suppressant therapy so from side effect of such therapy homoeopathy having a role to play in such autoimmune cutaneous disorder like pemphigus. Here are some repertorial rubrics which covers the medicine regarding pemphigus from different famous repertory are as under:
- Complete Repertory – Skin
ERUPTIONS pemphigus (55)
1 acon, 1 am-m, 2 anac, 1 antipyrin, 1 apis, 2 ars, 1 arum-t, 1 bell, 1 bufo, 1 calc, 1 calth, 1 canth, 1 carbn-o, 1 caust, 1 chin, 2 crot-h, 2 dulc, 1 gamb, 1 hep, 1 hippoz, 1 hydrc, 1 iodof, 1 jug-c, 1 kali-p, 1 lac-c, 3 LACH, 1 lipp, 2 lyc, 2 manc, 2 merc, 2 merc-c, 1 merc-pr-r, 2 nat-m, 2 nat-s, 1 nat-sal, 2 nit-ac, 1 ph-ac, 1 phos, 2 psor, 1 ran-b, 1 ran-s, 1 raph, 1 rheum, 2 rhus-t, 1 rhus-v, 2 sars, 1 scroph-n, 2 sep, 2 sil, 1 sul-ac, 1 sulfonam, 2 sulph, 1 syph, 1 ter, 1 thuj
- Phatak Repertory – Skin
ERUPTIONS, PEMPHIGUS (9)
1 ars, 2 arum-t, 1 canth, 2 dulc, 2 hep, 1 lach, 1 manc, 1 ran-b, 2 rhus-t
- Combined Repertory – Skin
ERUPTIONS, pemphigus (32)
1 acon, 2 anac, 2 ars, 1 bell, 1 bufo, 1 calc, 1 canth, 1 caust, 1 chin, 2 crot-h, 2 dulc, 1 hep, 1 hydrc, 1 jug-c, 3 LACH, 2 lyc, 2 merc, 2 nat-m, 2 nat-s, 2 nit-ac, 1 ph-ac, 1 phos, 2 psor, 1 ran-b, 1 ran-s, 2 rhus-t, 2 sars, 2 sep, 2 sil, 1 sul-ac, 2 sulph, 1 thuj
- Boericke Repertory – Skin
PEMPHIGUS (23)
2 anac, 3 ARS, 2 arum-t, 2 bufo, 3 CANTH, 2 caust, 2 dulc, 3 JUG-C, 3 LACH, 3 MANC, 2 merc, 3 MERC-C, 2 merc-pr-a, 2 ph-ac, 2 phos, 3 RAN-B, 3 RAN-S, 2 raph, 3 RHUS-T, 2 sep, 2 thuj, 2 calth, 2 antipyrin
- Murphy Repertory – Diseases
PEMPHIGUS (41)
1 acon, 2 anac, 1 antipyrin, 2 ars, 1 arum-t, 1 bell, 1 bufo, 1 calc, 1 calth, 1 canth, 1 carbn-o, 1 caust, 1 chin, 2 crot-h, 2 dulc, 1 hep, 1 hydrc, 1 jug-c, 3 LACH, 2 lyc, 2 manc, 2 merc, 2 merc-c, 2 nat-m, 2 nat-s, 1 nat-sal, 2 nit-ac, 1 ph-ac, 1 phos, 2 psor, 1 ran-b, 1 ran-s, 1 raph, 2 rhus-t, 2 sars, 2 sep, 2 sil, 2 sulph, 1 sul-ac, 1 syph, 1 thuj
Some Homoeopathic Remedies which are frequently prescribed in case of pemphigus which are as under:
- Caltha palustris – Bullae surrounded by a ring and itch greatly, on the third day they are transformed into crusts
- Cantharides – Erysipelatous inflammation of skin, forming blisters, burning, itching pains, ulcerative pain when touched; over sensitiveness accompanied by excessive weakness, urinary troubles.
- Carbonium oxygen – Excites vesication along the course of nerves and causes large and small vesicles of pemphigus
- Causticum – Large vesicle on chest and back, with anguish in chest and fever.
- Chininum sulph – Erythema forming confluent vesicles and bullae; which ulcerate and dry into crust.
- Copaiva – Pemphigus with excessive foetid discharge, attacking first mucous membranes and then the skin.
- Crotalus horridus – Pemphigus with low typhoid conditions, the contained fluid assumes a dark, body character, threatening gangrene.
- Phosphorus – painful, tense blisters full to bursting, not painful.
- Ranunculus bulb – constantly repeating eruption of blisters, secreting an offensive gluey matter, forming crusts and healing from the centre; acrid discharge makes the surrounding parts sore, pemphigus of newborn babies.
- Rhus tox – confluent blisters, containing a milky or watery fluid, with peeling of the skin.
- Thuja – pemphigus foliaceus; with offensive odor and formation of scales. (4)
Conclusion
It is essential to approach a homoeopathic practitioner for treatment of pemphigus because it is a safe and curative way of treatment for those who are getting bored from taking steroids and having some physical as well as financial problems.
Reference
1. | MUNJAL YP. API Textbook of Medicine. Ninth Edition ed. AGARWAL AK, GUPTA P, KAMATH SA, NADKAR MY, SINGAL RK, SUNDAR S, et al., editors.: The Association of Physicians of India; 2012. |
2. | GOLWALLA AF, GOLWALLA SA. Golwalla’s MEDICINE for Students: A Reference Book for the Family Physician. Twenty-Fifth Edition ed. NADKAR MY, editor. New Delhi | London | Panama: Jaypee Brothers Medical Publishers (P) Ltd ;The Health Sciences Publisher; 2017. |
3. | LOSCALZO J, JAMESON JL, LONGO DL, HAUSER SL, KASPER DL, FAUCI AS. Harrison’s principles of internal medicine. 21st ed.: McGraw Hill LLc.; 2022. |
4. | LILIENTHAL S. HOMOEOPATHIC THERAPEUTICS. SECOND REVISED AND ENLARGED EDITION ed. PHILADELPHIA: SHERMANS & CO., PRINTERS AND STEREOTYPERS; 1879. |