Psoriasis and pemphigus skin disortders.pptx

It is a type of autoimmune skin disorder

1. Krishna Priya C V M.Sc Nursing 1 st year Medical surgical Nursing

2. LESION TYPE (PRIMARY MORPHOLOGY ): Macules are flat, nonpalpable lesions usually < 10 mm in diameter. Macules represent a color change and are not raised or depressed compared to the skin surface. A patch is a large macule. Examples include freckles, flat moles, tattoos, port-wine stains, rashes of rickettsial infections, rubella, measles (can also have papules and plaques), and some allergic drug eruptions.

3. Papules are elevated lesions usually < 10 mm in diameter that can be felt or palpated . Examples include nevi, warts, lichen planus, insect bites, seborrheic keratoses, actinic keratoses, some lesions of acne, and skin cancers. A Plaque has been described as a broad papule, or confluence of papules equal to or greater than 10mm alternatively as an elevated, plateau-like lesion that is greater in its diameter than in its depth. Plaques are palpable lesions > 10 mm in diameter that are elevated or depressed compared to the skin surface. Plaques may be flat-topped or rounded. Lesions of psoriasis and granuloma annulare commonly form plaques

4. Nodules are firm papules or lesions that extend into the dermis or subcutaneous tissue . Examples include cysts, lipomas, and fibromas. Vesicles are small, fluid-filled blisters < 10 mm in diameter. Vesicles are characteristic of herpes infections, acute allergic contact dermatitis, and some autoimmune blistering disorders ( eg , dermatitis herpetiformis) Bullae are fluid-filled blisters > 10 mm in diameter . These may be caused by burns, bites, irritant contact dermatitis or allergic contact dermatitis, and drug reactions. Classic autoimmune bullous diseases include pemphigus vulgaris and bullous pemphigoid. Bullae also may occur in inherited disorders of skin fragility.

5. Pustules are vesicles that contain pus. Pustules are common in bacterial infections and folliculitis and may arise in some inflammatory disorders including pustular psoriasis. Scale is heaped-up accumulations of horny epithelium (stratum corneum) that occur in disorders such as psoriasis, seborrheic dermatitis, and fungal infections. Pityriasis rosea and chronic dermatitis of any type may be scaly.

6. SKIN The epidermis is the outer layer of skin. It is formed by stratified epithelium, which consists of 5 layers: 1. Stratum corneum 2. Stratum lucidum 3. Stratum granulosum 4. Stratum spinosum 5. Stratum germinativum

7. The important feature of the epidermis is that it does not have blood vessels.The nutrition is provided to the epidermis by the capillaries of the dermis. STRATUM GERMINATIVUM : Also called stratum basale , composed of a single row of cuboidal or columnar keratinocytes, here new cells are constantly formed by mitotic division . The newly formed cells move continuously towards the stratum corneum. The stem cells, which give rise to new cells, are known as keratinocytes.  

8. Another type of cells called melanocytes are scattered between the keratinocytes. The melanocytes produce the pigment called melanin. The color of the skin depends upon melanin. From this layer, some projections called rete ridges extend down up to dermis these projections provide anchoring and nutritional function. Two other types of cells are common to the epidermis, Merkel and Langerhans cells . Merkel cells are receptors that transmit stimuli to the axon through a chemical synapse. Langerhans cells are believed to play a significant role in cutaneous immune system reactions

9. STRATUM SPINOSUM: Numerous keratinocytes arranged in 8-10 layers contain a bundle of keratin intermediate filaments, containing an armlike process of melanocytes and Langerhans cells. It provides strength and flexibility to the skin. STRATUM GRANULOSUM : Consists of three to five flattened keratinocytes that undergo apoptosis. The nuclei and other organs begin to degenerate as they move farther from the source of nutrition. cells contain keratohyalin ( convert keratin intermediate filament into keratin )

10. STRATUM LUCIDUM: It is present only in areas like fingertips, palms, and soles, and consists of 4-6 layers of flattened clear, dead keratinocytes that contain large amounts of keratin. STRATUM CORNEUM: 25-30 Layers of flattened death cells. These cells lose their nucleus due to pressure and become dead cells. The cytoplasm is flattened with fibrous protein known as keratin

11. PSORIASIS The word psoriasis is derived from the Greek word 'psora' means 'itching'. I t is a papulosquamous disorder. Psoriasis is a chronic non-infectious, inflammatory disease of the skin in which epidermal cells are produced at a rate that is about six to nine times faster than normal. The cells in the basal layer of the skin divide too quickly, and the newly formed cells move so rapidly to the skin surface that they become evident as profuse scales or plaques of epidermal tissue .

12. Normal skin cells grow deep in the skin and rise to the surface about once a month. When you have psoriasis, this process takes place in 14 days rather than in 3 to 4 weeks . This results in dead skin cells building up on the skin's surface, forming collections of scales. Because the dead skin and white blood cells can't shed fast enough, they build up and create thick, scaly plaques on the surface of the skin.

13. INCIDENCE RATE: Prevalence is 125 million people worldwide — 2 to 3 percent of the total population — have psoriasis, according to the World Psoriasis Day consortium. With a prevalence of 0.44-2.8 percent in India , it commonly affects individuals in their third or fourth decade with males being affected two times more commonly than females. Psoriasis significantly impairs the quality of life of patients and their families resulting in great physical, emotional, and social burden The disease has a bimodal distribution of age of onset. The early peak age of onset is 22 years in males and 16 years in females and the later one at 60 years in males and 57 years in females

14. RISK FACTORS

15. KOEBNER PHENOMENON

16. PATHOPHYSIOLOGY

17. TYPES Plaque psoriasis [Psoriasis Vulgaris ] : The most common (75%) form, plaque psoriasis causes dry, red skin lesions (plaques) covered with silvery scales . The plaques itch or feel and may occur anywhere on the body Guttate psoriasis : It constitutes 12% of total psoriasis . This primarily affects people younger than 30 and is usually triggered by a bacteria such as streptococcal throat. It's marked by small, water-drop-shaped sores on the trunk, arms, legs, and scalp

18. PLAGUE PSORIASIS

19. Guttate psoriasis

20. Erythrodermic psoriasis : The less common type constitutes 7% of total psoriasis. Erythrodermic Psoriasis is the involvement of the entire body surface (≥90%) with a red, scaly rash. It may be triggered by severe sunburn, by corticosteroids and other medications, or by another type of psoriasis that's poorly controlled. Erythrodermic psoriasis is a rare type of psoriasis, but it’s much more serious than many other subtypes. The plaques can cover almost your entire body, potentially leading to life-threatening problems.

21. Abruptly stopping psoriasis treatments, like corticosteroids or immunosuppressants, can cause erythrodermic psoriasis. Overusing medications like topical steroids or retinoids (a vitamin A-related drug) can also cause symptoms. Some people develop erythrodermic psoriasis after having:  Allergic reaction to a medication. Illness or infection. Severe sunburn. Stress. Substance use disorder.

22. Pustular psoriasis : This rare (2%) form of psoriasis can occur in generalized ( generalized pustular psoriasis or Von Zumbusch's type ) or in localized to hands, feet or fingertips ( Acrodermatitis continua of Hallopeau ). It generally develops quickly with pus-filled blisters appearing just hours after skin becomes red and tender. The blisters dry within a day or two but may reappear every few days or weeks. Occasionally, it develops from unstable plaque psoriasis or acrodermatitis continua after inappropriate irritant therapy or withdrawal of extensive topical steroids

23. Inverse psoriasis is an immune-mediated condition. It causes a rash in areas of your skin that rub together, including your groin and armpits . It is found in the skin folds such as inguinal, axilla and sweating areas . Scaling is minimal or absent and lesion appears glossy, smooth and bright red. That's because having a higher-than-moderate body weight produces excess skin and deeper skin folds.

24.   TYPES BASED ON THE AREA AFFECTED: Nail psoriasis : In 25-50% of psoriasis patients can have nail changes. It affects fingernails and toenails, causing pitting, abnormal nail growth, waxy yellow or orange-brown discoloration ( oil-drop sign), and subungual hyperkeratosis. Psoriatic nails may become loose and separate from the nail bed (onycholysis). Severe cases may cause the nail to crumble

25. NAIL PITTING

26. onycholysis

27. oil-drop sign

28. subungual hyperkeratosis

29. Scalp psoriasis: Psoriasis on the scalp appears as red, itchy areas with silvery white scales firmly adherent to the scalp and associated hair has been termed Pityriasis (tinea) amiantacea . Scalp psoriasis can affect the hairline, the forehead, the back of the neck, and the skin in and around the ears.

30. Pityriasis (tinea) amiantacea

31. Genital psoriasis : It occurs when the autoimmune disease affects the skin on the buttocks or in the skin folds around the anus . It can also affect other genital tissue, including the penis, vulva, and pubic area. Psoriasis on the buttocks causes a painful and itchy rash or scaly plaques on the skin .It involves both inverse and plague type psoriasis

32. Drug-induced psoriasis: A drug may trigger the first episode in someone with no prior history of the disease ( de novo psoriasis ). A drug may trigger symptoms that will continue until the drug is stopped ( drug-induced psoriasis ) A drug may not trigger a flare but cause an acute episode to worsen and persist even after the drug is stopped ( drug-aggravated psoriasis). A drug may induce symptoms secondary to psoriatic skin lesions (such as psoriatic arthritis, nail arthritis, or a non-psoriatic autoimmune disease).

33. CHARACTERISTICS OF PSORIATIC LESIONS : The lesions are most abundant over the scalp, the extensor surface of the elbows and knees, the lower part of the back, and the genitalia Bilateral symmetry is a feature of psoriasis. In approximately one-fourth to one-half of patients, the nails are involved, with pitting, discoloration, crumbling beneath the free edges, and separation of the nail plate. When psoriasis occurs on the palms and soles, it can cause pustular lesions called palmar pustular psoriasis.

34. SIGNS AND SYMPTOMS I nitially , the first sign of psoriasis is often red spots on the body. Dry, swollen, and inflamed patches [defined margin between plaque and normal skin] Patches Covered with silver-white flakes Raised and thick skin Other symptoms of psoriasis include . Pain, itching and burning sensation Cracked and bleeding skin Hair loss Pus-filled blisters

35. Genital lesions in males. Pitting, small depression on the surface of the nail Yellow, discolored nail Distal separation of the nail plate from the nail bed(onycholysis) Yellow-brown discoloration underneath the nail plate("oil drop" sign) Subungual hyperkeratosis Thickening of the nail (onychodystrophy). Arthritis Restricted joint motion or pain

36. Swollen and painful joint (Sausage digits) Severe skin redness over a large part of the body Skin shedding that occurs in large sheets rather than smaller flakes or scales Pustules or blisters Burnt-looking skin Severe itching Intense pain Increased heart rate Fluctuations in body temperature Dehydration Hypothermia

37. DIAGNOSIS: History collection including family history, aggravating factors, age of onset , pattern of recurrence, drug history, stress Physical examination Grattage Test : When the psoriatic plaque is scraped with a glass slide, the very characteristic silvery scales come out that is known as the Candle Wax/Grease Sign. Auspitz Sign : The successive removal of psoriatic scales usually reveals an underlying smooth glossy red membrane (Berkley membrane) with small bleeding points which appear when the thin suprapapillary epithelium is torn off. It refers to pinpoint bleeding under the skin's surface . It typically occurs after scratching psoriasis plaques or other forms of skin scales due to capillaries just beneath the skin's surface get rupture.

38. Skin biopsy under local anesthesia Blood and radiography test was done to rule out psoriatic arthritis (ESR, C- Reactive protein

39. MANAGEMENT: The goals of management are: • There is no known cure. • To promote resolution of the psoriatic lesions • To control the natural cycles of the disease. First, avoid any precipitating or aggravating factors • An assessment is made of lifestyle, because psoriasis is significantly affected by stress.

40. The standard treatment modalities includes: Topical therapy Intralesional therapy Systemic therapy Photochemotherapy

41. TOPICAL THERAPY   In general, high-potency topical corticosteroids should not be used on the face and intertriginous areas, and their use on other areas should be limited to a 4-week course of twice-daily applications . A 4-week break should be taken before repeating treatment with the high-potency corticosteroids. For long-term therapy, moderate-potency corticosteroids are used. On the face and intertriginous areas, only low-potency corticosteroids are appropriate for long-term use The most important principle of psoriasis treatment is the removal of scales gentle

42. This can be accomplished with baths. Oils ( eg , olive oil, mineral oil) or coal tar preparations ( eg. Balnetar ) can be added to the bath water, and a soft brush used to scrub the psoriatic plaques gently. After bathing, the application of emollient creams containing alpha-hydroxy acids ( eg. Lac- Hydrin . Penederm ) or salicylic acid will continue to soften thick scales .[Keratolytic] Vitamin D analogues-e.g.. calcipotriene , it suppress epidermopoiesis (development of epidermal cells) causing sloughing of growing epidermal cells Coal tar - dry distillation product of organic matter heated in the absence of oxygen, combination of creams, ointments and pastes. Tazarotene – [Retinoid analog ] it reduce mainly scaling & plaque Thickness, normalize the DNA activity.[ Teratogenecity ] Topical Calcineurin Inhibitors - tracolimus , they inhibit activation of the cells which reduce inflammation and plaque build up. Emollients to avoid dryness. It reduce scaling and limit pain.

43. Occlusive dressings. Use plastic wrap or bags as the occlusive dressing. and use rubber gloves on the client's hands, plastic bag on the feet, and a shower cap on the head if affected. Anthralin preparations ( Anthra -Derm, Drit Crème, Lasan ) for thick psoriatic plaques resistant to other coal tar or steroid preparations. [ It inhibit DNA synthesis and mitotic action] Topical corticosteroids, used for short periods because of their side effects

44. . INTRALESIONAL THERAPY. Injections into highly visible or isolated patches of psoriasis that are resistant. Triamcinolone [ intermediate glucocorticoid ] acetonide is injected, and care is taken so that normal skin is not injected

45. . SYSTEMIC THERAPY Methotrexate have been used in treating extensive psoriasis that fails to respond to other forms of therapy. It inhibits DNA synthesis in epidermal cells and thus reducing the epidermopoesis . It is an effective agent with well-studied efficacy and toxicity. Its use is reasonably standardized Indications include psoriatic erythroderma, acute pustular psoriasis, localized pustular psoriasis, psoriatic arthritis, extensive psoriasis unresponsive to other, less toxic therapies: psoriasis that interferes with quality of life, and psychologically disabling psoriasis  Oral retinoids (synthetic derivatives of Vitamin A and its metabolite, Vitamin A acid ) Antinflammatory and antiproliferative

46. Hydroxyurea ( Hydrea ). [ Antimetabolites ]Monitor signs and symptoms of bone marrow depression. Cyclosporine A [ Calcineurin Inhibitors ] Reinforce women of childbearing age that retinoids and methotrexate are teratogenic; women must be using birth control.

47. DRUG DOSAGES : Cyclosporine Initially 2.5 mg/kg daily in two divided dosages, increasing gradually to a maximum 5 mg/kg daily if no improvement within 1 month. Initial dosage of 5 mg/kg daily justified if condition Hydroxyurea Requires rapid improvement Initially, 500 mg twice daily (maximum 1.5 g/day) Acitretin Initially 25-30 mg daily for 2-4 weeks, adjusted accor ding to response, usually within range 25-50 mg daily ( upto a maximum 75 mg)

48. Methotrexate 10-25 mg once weekly, adjusted according to response, consider dose reduction in elderly patients, commencing at 5 mg weekly in patients >65 years of age PHOTOCHEMO THERAPY Sunlight-activated T-cells in skin are destroy lead to reduce scaling and inflammation  A treatment for severely debilitating psoriasis is Psoralen and Ultraviolet A (PUVA) Therapy. which involves taking a photosensitizing drug (usually 8-methoxypsoralen) in a standard dose with subsequent exposure to long-wave ultraviolet light when peak drug plasma levels are obtained. UVB light is also used to treat generalized plaque.

49. OTHERS HANDOUT

50.   THERAPY OF ERYTHRODERMIC (EXFOLIATIVE) PSORIASIS   First Steps   1. If patient appears systemically ill, hospitalize the patient in a comfortably warm environment (to limit loss of body heat), and carefully monitor for sepsis and potential cardiovascular, thermoregulatory, fluid, and electrolyte problems. For otherwise healthy patients, a day treatment center may be appropriate.   2. Treat with medium strength topical corticosteroids in an ointment or cream twice a day under sauna suit occlusion if tolerated.

51. Initiate treatment with cyclosporine if there are no contraindications. Expect improvement in first week.  4. If cyclosporine not an option, consider methotrexate therapy.  5. Phototherapy, systemic steroids, and potentially irritating topical agents (tazarotene, calcipotriene, tar) should be avoided during the period of acute inflammation. Subsequent Steps When erythroderma improves, taper cyclosporine and begin the patient on the Goeckerman regimen (if available) or transition to acitretin 25-50 mg daily

52. Maintenance therapy for psoriasis is often required following resolution of the erythrodermic episode. depending on the residual clinical morphology. This may include acitretin, phototherapy, topical agents,

53. NURSING MANAGEMENT : FOR PHOTOCHEMO THERAPY : The patient is usually treated two or three times each week until the psoriasis clears. An intermediate period of 48 hours between treatments is necessary to allow any burns resulting from PUVA therapy to become evident The skin and eyes must be protected from ambient UVL irradiation from the time psoralens are ingested until 8 hours after taking the photosensitizing medication. The client should asked to wear protective clothing, such as long sleeves

54. Apply sunscreen to exposed skin Minimize natural skin exposure with topical psoralen Wear both uva and uvb protective eyewear for 24 hours after taking the medication MONITORING OF METHOTREXATE THERAPY:   CBC with differential and platelets should be monitored weekly during dose escalation and at monthly intervals when the dose is stabilized. Always obtain blood counts 1 week after drug administration.

55. Perform liver function studies every 1-2 months. Perform renal function studies every 3-4 months.  Because liver damage may occur in the absence of abnormal LFTs, liver biopsy should be obtained after the first 1.5 g of methotrexate administered in patients without risk factors. Repeat biopsies should be obtained at 1.0-1.5 g intervals.  Patients with risk factors for hepatic fibrosis should have a pretreatment or early treatment liver biopsy. with repeat biopsy after each 1.0 g of methotrexate. A chest X-ray should be performed yearly in patients with acute or chronic pulmonary disease to rule out methotrexate pneumonitis.

56. OTHERS Provide psychological support Educate the family members and patients about the prognosis ot the disease   DIETARY MANAGEMENT: Avoid dairy products, glutens, and alcohol as it promote inflammation Take vitamin D-rich food  Drink plenty of water

57. NURSING DIAGNOSIS : Impaired skin integrity related to lesion and inflammatory response as evidence by itching all over body. Disturbed body image related to embarrassment over appearance and self-perception of uncleanliness Deficient knowledge about the disease process and treatment Risk for infection related to break in the integrity of the skin. Acute pain related to inflammation.  

58. COMPLICATIONS   Infection Fluid and electrolyte imbalance Low self esteem. Depression Stress Metabolic syndrome (increased blood pressure, high blood sugar, excess body fat around the waist, and abnormal cholesterol levels) Hypertension Joint damage Psoriatic arthritis  

59. RESEARCH ARTICLE: Isomorphic (Koebner) Phenomenon Induced by Insulin Analogue Injections in Psoriasis Saartje Thijs 1, Eric Balti 1, Corinne Degraeve 2, Peter Coremans 1 Abstract Koebner phenomenon is an uncommon skin-related complication of subcutaneous insulin injection in patients with diabetes mellitus. This reaction, also referred as isomorphic phenomenon, has previously been described in various conditions including vitiligo, lichen planus, and psoriasis. We report a 56-year-old woman insulin-treated patient with type 2 diabetes mellitus who developed new-onset, sharply well-demarcated erythematous scaly plaques at the insulin injection sites consistent with Koebner phenomenon. These lesions occurred after withdrawal of methotrexate initiated for the treatment of psoriasis. The lesions responded well to guselkumab , an interleukin-23 targeting agent but not ciclosporin. Of note, unlike previously reported cases, our patient developed isomorphic response under treatment with insulin analogues and during psoriasis flare-up. This case highlights the paramount role of thorough and cautious examination of injection and insertion sites in patients at risk undergoing subcutaneous continuous glucose monitoring or treated with continuous transdermal/subcutaneous insulin injections.

60. PEMPHIGUS The vesiculobullous diseases although uncommon have an economic impact on the patient and the family.

61. Pemphigus is an autoimmune disease IgG antibodies are directed against specific cell surface antigens on epidermis Pemphigus, an autoimmune disease is characterized by intraepidermal blistering at various levels in the epidermis . Because of disruption of the intercellular cementing substance. It is due to an autoantibody attack (IgG) on the cellular adhesion proteins ( desmogleins ) leading to acantholysis seen on tzanck smear

62. Epidemiology Rare, equal in men and women, onset at 40 to 60 years of age, more common in Jewish or Mediterranean descendants. Pemphigus vulgaris has been reported to occur worldwide. Pemphigus vulgaris incidence varies from 0.5-3.2 cases per 100,000 population The incidence of pemphigus among the dermatology outpatient attendees has varied widely, 0.09-1.8%

63. TYPES Pemphigus vulgaris Pemphigus foliaceus Pemphigus erythematosus Pemphigus vegetans Drug-induced IgA (SCPD, Intra-epidermal type) pemphigus Paraneoplastic pemphigus

64. PEMPHIGUS VULGARIS   It is the most common form of the disorder and occurs when  antibodies  attack  Desmoglein-3 . Sores often originate in the mouth , making eating difficult and uncomfortable. Although PV may occur at any age, it is most common among people between 40 and 60. It is more frequent among  Ashkenazi Jews . Rarely, it is associated with  myasthenia gravis .  Nail disease  may be the only finding and has prognostic value in management .

65. PEMPHIGUS FOLIACEUS (PF) It is the least severe variety. Desmoglein 1, the protein that is targeted by the autoantibodies, is enriched in the upper skin layers. PF is characterized by crusty sores that often begin on the scalp, and may move to the chest, back, and face. Mouth sores do not occur. This form is also frequent among Ashkenazi Jews. It is not as painful as PV, and is often misdiagnosed as dermatitis or eczema

66. PEMPHIGUS ERYTHEMATOSUS It is a variant of pemphigus foliaceous characterized by immunological features of both pemphigus and lupus erythematosus (LE), that is, intercellular IgG & C3 in the epidermis (as in pemphigus) and in the basement membrane zone and antinuclear antibodies (as in LE). Clinically, erythematous, scaly rash over the nose and cheeks simulate LE while lesions on the trunk are similar to those in pemphigus foliaceous  

67. PEMPHIGUS VEGETANS It is a clinical variant of pemphigus vulgaris characterized by vegetating lesions primarily in the flexures, Initial lesions are bullae or pustules, which rupture and progress to form vegetating plaques in the axillae and groins.

68. PATHOPHYSIOLOGY

69. CLINICAL MANIFESTATION The blisters will appear in the mouth and the scalp first and then spreads to the face, back, chest, umbilicus, and groins. The blister will rupture resulting in crusting and oozing of fluid with foul smell. Pain Nikolsky's sign (the pressure within the blister leads to the spread of blister to adjacent tissues Asboe Hansen sign or indirect nikolsy’s sign

70. INVESTIGATION 1. Tzanck smear from the floor of the blister shows acantholytic cells, which is a large, rounded epidermal cell with a large vesicular nucleus with multiple nucleoli with perinuclear halo and peripheral condensation of cytoplasm 2 . Histopathological examination A supra- basal cleft in the epidermis containing eosinophils and acantholytic cells. The basal keratinocytes remain attached to the basement membrane but are separated from each other and stand like a 'row of tombstones". 3. Direct immunofluorescence from the lesional skin will have deposition of intercellular IgG throughout the epidermis in a 'fish-net pattern (IgG, IgA.C3.Ciq)   4. Indirect immunofluorescence detects circulating IgG antibodies in 80-90% of the cases whose levels correlate with disease activity

71. MANAGEMENT The goals of therapy are • To bring the disease under control as rapidly as possible • To prevent loss of serum and the development of secondary infection • To promote re-epithelization Corticosteroids are administered in high doses to control the disease and keep the skin free of blisters. In some cases, corticosteroid therapy must be maintained for life. Immunosuppressive agents ( eg. Azathioprine, Cyclophosphamide) may be prescribed to help control the disease and reduce the corticosteroid dose. Plasmapheresis ( ie , plasma exchange) temporarily decreases the serum antibody level and has been used for life-threatening cases.

72. TREATMENT OF PEMPHIGUS VULGARIS Systemic steroids in a dose of 2-3 mg per kg body weight is the drug of choice. First Step Treat with prednisone 60-80 mg/day. In the case of persistent oral lesions, treat for candidiasis with oral fluconazole 150 mg once weekly. Dental trays filled with superpotent topical steroids worn for 4-8 hours daily may be effective for refractory gingival disease.

73. Subsequent Steps   1. In patients responding to the above treatment, gradual taper the systemic steroids and immunosuppressives over 3-6 months (described on page 199, 462-464) 2. The titer of the circulating autoantibody may be useful in determining the response to treatment and the rate at which immunosuppressive therapy can be tapered. It should be monitored every 6 months and should increase with each measurement.  In refractory pemphigus vulgaris, consider the following options:  a. Increasing the dose of mycophenolate Mofetil to 3g / day. b. Monthly pulse steroids of 1 g of methylprednisolone daily for 3 days intravenously; or dexamethasone 100 mg daily for 3 days with cyclophosphamide 500 mg/2 nd day. c. High-dose intravenous immunoglobin 2 g/Kg/month over several days.  

74. COMPLICATIONS • Secondary bacterial infection • Fluid and electrolyte imbalance • Hypoalbuminemia

75. NURSING DIAGNOSES • Acute pain of skin and oral cavity related to blistering and erosions • Impaired skin integrity related to ruptured bullae and denuded areas of the skin • Anxiety and ineffective coping related to the appearance of the skin and no hope of a cure • Deficient knowledge about medications and side effects

76. NURSING INTERVENTIONS • Meticulous oral hygiene is important to keep the oral mucosa clean and allow the epithelium to regenerate. • Frequent rinsing of the mouth is prescribed to rid the mouth of debris and to soothe ulcerated areas. • The lips are kept moist with lip balm • Cool wet dressings are protective and sooth • The patient with painful and extensive lesions should be pre-medicated with analgesics before skin care is initiated. • Hypothermia is common, and measures to keep the patient warm an nursing activities

77. • After the patient's skin is bathed, it is dried carefully and dusted liberally with non-irritating powder, which enables the patient to move freely in bed. • Reducing anxiety of the patient. • The patient is encouraged to express freely anxieties, discomfort, and feelings of hopelessness. • Arranging for a family member or a close friend to spend more time with the patient can be supportive. • Referral for psychological counseling may assist the patient in dealing with fears, anxiety, and depression

78. Pulse Therapy in Pemphigus: Ready Reckoner Anil Abraham ,  Gillian Roga , and  Anupa Mary Job Pulse therapy for the treatment of pemphigus has been in vogue for several years and is administered by many dermatologists across the world. However, even though there is enough evidence about its efficacy and methodology, there continue to be doubts and questions regarding the rationale of use of high dose intravenous steroids and steroid-sparing immunosuppressants. This article has aimed to provide clarity to young dermatology residents on the administration of pulse therapy, and the various controversies and modifications that have been mentioned in literature over the past couple of years.

79. Dexamethasone-azathioprine pulse (DAP): Cyclophosphamide is replaced by daily oral azathioprine. No bolus dose of azathioprine is given during the pulse Dexamethasone-methotrexate pulse (DMP ): Cyclophosphamide is replaced by 7.5 mg of oral weekly methotrexate (three doses of 2.5 mg at 12 h apart), during the three phases of pulse therapy Rituximab is also given for pemphigus according to the rheumatoid arthritis protocol as 2 doses of 1 g, 2 weeks apart and according to the lymphoma protocol as 375 mg/m2 weekly for 4 weeks.

80. BIBLIOGRAPHY BOOK REFERENCE 1 ) Garg K G, Sardana K .Comprehensive textbook of dermatology,1 st edition :Pee publication;2010.pgno180-87, 200-10 2) Suddharth &Brunner. Textbook of Medical Surgical Nursing, 13 th edition: Wolter Kluwer publication; 2014.pgno 1450-1460. 3)Black JM, Hawks JH. Medical Surgical Nursing, 1 st edition : Elseiver publication;2019. pgno 1458-1489. 4)Kaur L, Kaur p. Adult Medical Surgical Nursing ,3 rd edition :Lotus publication;2008 . pgno [1080-98] 5) Workman, Ignatavicus . Medical Surgical Nursing,7 th edition :Evolve publication;2009 . pgno [1080-98]

81. JOURNAL REFERENE   Thijs S, Balti E, Degraeve C, Coremans P. Isomorphic (Koebner) Phenomenon Induced by Insulin Analogue Injections in Psoriasis. JCEM Case Rep. 2022 Nov 30;1(1):luac016. doi: 10.1210/ jcemcr /luac016. PMID: 37908249; PMCID: PMC10578368. Matthews R, Ali Z. Comorbid mental health issues in patients with pemphigus vulgaris and pemphigus foliaceus. Clin Exp Dermatol. 2022 Jan;47(1):24-29. doi: 10.1111/ced.14916. Epub 2021 Oct 19. PMID: 34459019.