Dr Selva Panda
Pemphigus is an autoimmune blistering disease, which affects the skin and the mucous membranes. The disease is chronic in nature, and runs a relapsing course. It has got a potentially devastating impact on the life of the patient not only physically but psychologically too. A thorough understanding of its etiology, classification, clinical manifestations, diagnosis, management is required for the treatment.
Keywords: Homeoeopathy, Pemphigus, Acantholysis , Nikolsky’s sign, Boericke’s Meteria Medica, Phatak’s repertory.
The word pemphigus is derived from the Greek word “pemphix” which means bubbles, pustule or blister. The word was first used in 1791, by Wichman for describing a chronic blistering dieases. It is characterized by formations of blisters and erosions on the stratified squamous epithelia such as the skin and mucous membrane, in which the production of autoantibodies against the extracellular domains of cell membrane proteins of keratinocytes which results in the loss of cell–cell adhesion between the keratinocytes (acantholysis). IgG autoantibodies targets the various adhesion molecules in the stratified squamous epithelia , including desmogleins 1 and desmogleins 3, which are the major components of desmosomes responsible for maintaining intracellular adhesion. The IgG autoantibody binds with desmogleins resulting in loss of keratinocytes adhesion, this phenomenon is referred as acantholysis. This causes intra epidermal blistering and there is clinical appearance of flaccid blisters and erosions at the site.
The prevalence varies worldwide. It is the commonest cause of autoimmune blistering in India. Generally seen in middle age or older age from 40-60 years of age., but may occur in children also. There is no gender discrimination, equally affects both male and females.
- Idiopathic autoimmune phenomenon
- Neosplasia such as thymoma and lymphoma can also induced autoimmune phenomenons.
- Hereditary cause
- Certain drugs might interfere with biochemistry of the keratinocyte membrane or the immune balance and can promote acantholysis in pemohigus. Ex- Penicillamine, and rifampicin.
- Some environmental factors can also trigger its onset such as viruses( herpes simplex virus), dietary factors and physiological and psychological stress.
Classifications & Clinical Feature:
Pemphigus can be classified into four types:
It is the most common clinical variant out of the four. The spilt is suprabasal. It can be characterized by cutaneous and mucosal blisters. Lesions are predominantly present on face, scalp, axillae, groins, and trunk. Periungual lesions frequent. The flaccid bullae develop on normal skin and they rupture to form painful erosions, which have tendency to spread and it takes very long to re-epithelialize. On application of tangential pressure usually in pretibial areas of the normal skin, it results in formation of new bulla (Nikolsky sign). And if the pressure is applied to pre-existing bulla, it results in spread of bulla (bulla spread sign). The disease in about 50% of patient begins in oral mucosa.it is most frequently involved. There is manifestations of painful erosions which extend peripherally with shedding of mucosa, giving a ragged appearance.
This is a variant of pemphigus vulgaris. The bulla is suprablasal. It is characterized by presence of heaped up, vegetating lesions which are extend centrifugally. Commonly found in the groins, axillae, angles of mouth. There may be presence of mucosal lesions.
The onset is often subtle, with a few scattered crusted lesions It is characterized by the absence of mucosal involvement i.e., there is no mucosal lesion. The split is either in granular layer or just below the horny layer. It is presented with leafy, scaly and crusted circumscribed erosions on erythematous skin. Initial seborrheic areas, including the face and the upper trunk, are mainly involved, later becomes generalized to resemble exfoliative erythroderma. The removal of the scale-crust does not reveal an erosion but only a minimally moist skin.
This is a less severe variant of pemphigus foliaceus. The split is either in granular layer or just below the stratum corneum. It is characterized by dry, hyperkeratotic, scaly lesions. Commonly found on face, upper part of chest and back.
Diagnosis of pemphigus is generally based on clinical manifestation and is can be done by:
- Being a chronic vesiculobullous disease, the presence of flaccid bullae which rupture to form nonhealing erosions can be marked. Positive Nikolsky’s and bulla spread sign are seen. Painful oral erosions can be seen.
- Confirmation can be done by – Tzanck smear, histological and immunopathological tests.
Supportive treatment is very much necessary in these cases.
- Local hygiene of mucosal and skin lesions should be maintained.
- There should be proper maintenance of water and electrolyte balance.
- Maintenance of body temperature.
- Barrier nursing, if required.
Antipyrinum- pemphigus with intense puritus,
Arsenic album- eruption, papular, dry, rough, scaly, itching, burning, worse cold and scratching. Offensive discharge. Epithelioma of skin.
Bufo rana- patches of skin has lose sensation. Pustules, suppuration from every slight injury. Pemphigus.Bullae which open and leave a raw surface, exuding and ichorous fluid. Blisters on palms and soles. Itching and burning.
Caltha palustris- pemphigus. Bullae are surrounded by a ring. Much itching. Face much swollen, especially around the eyes. Itching eruption on thighs. Pustules.
Cantharis- eruption with mealy scales. Versicular eruption, with burning and itching, rawness, with great restlessness.
Carboneum oxygenisatum- pemphigus is produced by this drug during drug proving. Pemphigus with large and small vesicles.
Cinaberis – buboes, very fiery red looking ulcers, which bleeds easily.
Dulcamara- pemphigus. Vesicular eruptions, Thick, brown-yellow crusts, bleeding when scratched
Juglans cinerea- pustules especially on hands, redness, itching and pricking when heated.
Mancinella- large blisters, as from scalds. Heavy, brown crusts and scabs. Pemphigus.
Mercurius praecipitatus ruber- pemphigus with rhagades and fissures, with gomorrhoea, urethra felt as a hard strings, suffocative attacks at night on lying down while on the point of falling asleep.
Ranunculus bulb- it has been used for vesicular eruptions; for pemphigus. Burning and intense itching, worse contact. Hard excrescences. Blister like eruption in palms. Vesicular and pustular eruption. Horny skin. Finger-tips and palm chapped.
Ranunculus sceleratus- pemphigus. Vesicular eruption, with tendency to form large blisters. Acrid exudation, which makes surrounding parts sore. Boring, gnawing pain very marked
Rhus toxicodendron- red, swollen; itching intense.Vesicles, herpes; urticaria; pemphigus; erysipelas; vesicular suppurative forms. Burning eczematous eruptions with tendency to scale formation.
Lachesis : A very important 3 mark remedy for pemphigus
PHATAK REPERTORY: ERUPTIONS, Pemphigus:
Ars; Arum-t; Canth; Dul; Hep, Lach, Manc, Ran-b, Ran-sc+, Rhus-t.
BOERICKE REPERTORY: SKIN, PEMPHIGUS:
Anac., Antipyr., Ars., Arum tr., Bufo, Caltha., Canth., Carbon. ox., Caust., Dulc., Jugl. c., Lach., Mancin., Merc. c., Merc. pr. rub., Merc. s., Nat. sal., Phos. ac., Phos., Ran. b., Ran. sc., Raph.,Rhus t., Sep., Thuja.
KENT REPERTORY: SKIN, ERUPTION, Pemphigus:
Acon., anac., ars., bell., bufo., calc., canth., caust., chin., crot-h., dulc., hep., hydrc., jug-c., Lach., lyc., merc., nat-m., nat-s., nit-ac., ph-ac., phos., psor., ran-b., ran-s., rhus-t., sars., sep., sil., sul-ac., sulph., thuj.
Pemphigus being an autoimmune disease, there is nothing much to do in allopathic treatment, the first line of treatment they have got are corticosteroids, immunosuppressive agents, and other adjuvant therapies. They have got lots of adverse effects on long term. There is no cure of it i conventional system. In these cases, homoeopathy is very effective. It is treatable in early stages and preventive in later stages. The relapsing can be checked with proper selection of similimum.
- Khanna Neena, Illustrated Synopsis Of Dermatology and Sexually Transmitted Diseases, 4th Edition, 2011, Elservier, UK.
- Phatak S.R, A Concise Repertory Of Homoeopathic Medicine, 4th edition, B. Jain Publisher (P) Ltd; New Delhi.
- Boericke William, Boerickes New Manual Of Homoeopathic Material Medica and Repertory , 9th edition;2011;B Jain Publishers, New Delhi.
- Davidson Stanley,Davidson’s Principles and Practice of Medicine, 21st edition, 2010; Churchill Livingstone Elvister, UK.
Dr. Selva Panda
PGR, Part-I , Department Of Practice of Medicine,
Jawaharlal Nehru Homoeopathic Medical College, Parul University,Waghodia, Vadodara.