This document provides an overview of blistering skin diseases that commonly affect the elderly. It begins with a case study of an 81-year-old man presenting with an extensive blistering rash. It then discusses bullous pemphigoid (BP) in detail, including epidemiology, pathogenesis, clinical features, treatment, and prognosis. It also briefly mentions related diseases like cicatricial pemphigoid, epidermolysis bullosa acquisita, porphyria cutanea tarda, linear IgA disease, and dermatitis herpetiformis. Finally, it provides an introduction to pemphigus vulgaris (PV), pemphigus foliaceus (PF),
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Blistering skin diseases in elderly
1. Basil Tumaini
Blistering skin diseases in elderly
Dr. Basil Tumaini MD, MMED (Internal Medicine)
Muhimbili University of Health and Allied Sciences
2. Basil Tumaini
Outline
• Case scenario
• Overview
• Pemphigoid: BP and related diseases
• Pemphigus: PV, PF, PNP
• Key points
• Further reading
3. Basil Tumaini
Case scenario
An 81 year old man presented with a 10 day history of an
extensive blistering rash that was profoundly itchy.
He had a history of hypothyroidism and a deep vein thrombosis.
He was receiving carbimazole and warfarin and denied any recent
changes in medication.
On examination he had more than 100 blisters over his whole
body, although his face was spared.
Some of the blisters formed a circinate pattern. Nikolsky’s sign
was negative. The mucous membranes were not affected
Noel S, Dua J, Kaushal G. An 81 year old man with a blistering rash. BMJ: British
Medical Journal (Online). 2013;346.
5. Basil Tumaini
Questions
1. What are the possible causes of this man’s blisters?
2. Which investigations would you do to confirm your
diagnosis?
3. What comorbidities are associated with this
condition?
4. What is the best treatment for this condition?
6. Basil Tumaini
Q & short answers
1. What are the possible causes of this man’s blisters?
2. Which investigations would you do to confirm your
diagnosis?
3. What comorbidities are associated with this
condition?
4. What is the best treatment for this condition?
7. Basil Tumaini
Q & A
1. What are the possible causes of this man’s blisters?
2. Which investigations would you do to confirm your
diagnosis?
3. What comorbidities are associated with this
condition?
4. What is the best treatment for this condition?
8. Basil Tumaini
Q & A
1. What are the possible causes of this man’s blisters?
2. Which investigations would you do to confirm your
diagnosis?
3. What comorbidities are associated with this
condition?
4. What is the best treatment for this condition?
9. Basil Tumaini
Q & A
1. What are the possible causes of this man’s blisters?
2. Which investigations would you do to confirm your
diagnosis?
3. What comorbidities are associated with this
condition?
4. What is the best treatment for this condition?
10. Basil Tumaini
Introduction
• A blister: a fluid filled cavity formed within or
beneath the epidermis
• Categorized as a vesicle or a bulla
– A vesicle: <0.5 cm in diameter
– A bulla: >0.5 cm in diameter
Disorders in which blistering is the primary event are traditionally termed as
blistering disorders or vesiculobullous disorders
13. Basil Tumaini
Pathophysiology
• Aging skin is a consequence of both intrinsic aging
related to internal physiologic changes as well as of
extrinsic aging to cumulative extrinsic factors, such
as UV light exposure1
• Skin of the elderly undergoes flattening of the
dermal papillae leading to increased risk of blister
formation1
1. Farage MA, et al. Clinical implications of aging skin: cutaneous disorders in the elderly.
Am J Clin Dermatol. 2009;10(2):73–86
14. Basil Tumaini
• A decreased rate of epidermal turnover with
subsequent retardation of cellular migration and
proliferation leads to delayed re-epithelization and
wound healing
• Immunosenescence2 leads to altered inflammatory
response with reduced T-cell proliferative responses,
delayed hypersensitivity reaction and production
of cytokines and number of Langerhans cells
2. Loo WJ, Burrows NP. Management of autoimmune skin disorders in the
elderly. Drugs Aging. 2004;21(12):767–77
15. Basil Tumaini
• It remains unclear how these changes contribute to
the development of autoimmune blistering disorders
such as bullous pemphigoid which often occur after
the age of 70 years
• Many well known autoimmune diseases, such as SLE
typically occur in a much younger patient population
17. Basil Tumaini
BP introduction
• Blistering disease of elderly people which often
starts with pruritus and urticated and erythematous
lesions
• Later, large, tense blisters develop both on
erythematous and on normal skin
• There may be mucosal involvement with blisters and
erosions
• Blisters are subepidermal and intact epidermis forms
the roof
• Autoantibodies, chiefly IgG, to the epidermal BMZ
are found in the skin and blood
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's
textbook of dermatology. John Wiley & Sons; 2016 Feb 29.
18. Basil Tumaini
Epidemiology
• BP is an affliction of elderly people
• Onset usually after 60 years of age, mean 80 years
(few cases have been described in children)
• Risk increases with age
• BP is the commonest immunobullous disease in
Western Europe, incidence 6-42/1 000 000 where it
was associated with HLA-DQ7
Alpsoy E, Akman-Karakas A, Uzun S. Geographic variations in epidemiology of
two autoimmune bullous diseases: pemphigus and bullous pemphigoid.
Archives of dermatological research. 2015 May 1;307(4):291-8.
19. Basil Tumaini
Epidemiology …
• Tunisia: 3.84/1 000 000 reported in Tunisia*
• In a cross-sectional study of skin diseases
among elderly patients attending skin clinic at the
Regional Dermatology Training Centre, Northern
Tanzania, blistering diseases accounted for 2.8% of
the cases**
* Zaraa I, et al. Spectrum of autoimmune blistering dermatoses in Tunisia: an 11‐year study and
a review of the literature. International journal of dermatology. 2011 Aug;50(8):939-44.
** Mponda K, Masenga J. Skin diseases among elderly patients attending skin clinic at the
Regional Dermatology Training Centre, Northern Tanzania: a cross-sectional study. BMC
research notes. 2016 Dec;9(1):119.
20. Basil Tumaini
Possible associations
• Drugs: furosemide, spironolactone, penicillins, B,
enoxaparin, antipsychotics
• Local irritation/damage: PUVA, local trauma, burns,
radiotherapy, skin grafts
• Systemic diseases: DM, RA, UC,
• Neurological diseases: MS, PD
• Malignancy: gastric ca
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's
textbook of dermatology. John Wiley & Sons; 2016 Feb 29.
21. Basil Tumaini
Pathogenesis
• The disease is characterized by IgG autoantibodies
to hemidesmosome-associated proteins (BP230 and
BP180) within the adhesion complex
• BP230 and BP180 are the products of genes located
on different chromosomes, 6p and 10q respectively
• The formation of blisters begins with autoantibody
deposition which binds C3 along the
dermoepidermal junction
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's
textbook of dermatology. John Wiley & Sons; 2016 Feb 29.
23. Basil Tumaini
Pathogenesis …
• IgG autoantibodies bind to the bullous
pemphigoid antigens and activate complement
• Complement components set off an
inflammatory cascade attracting leukocytes,
degranulating mast cells and releasing
inflammatory mediators
• The activated inflammatory cells release
lysosomal enzymes and proteases, cleaving the
target antigens and disrupting the
hemidesmosomes resulting in blister formation
24. Basil Tumaini
Pathology
• The blister in BP is subepidermal with an intact and
often viable epidermis forming the roof
• Biopsies of blisters and from erythematous areas of
skin show a dermal inflammatory infiltrate containing
many eosinophils and neutrophils with lymphocytes
and histiocytes
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's
textbook of dermatology. John Wiley & Sons; 2016 Feb 29.
25. Basil Tumaini
Clinical features
• BP commonly starts with itching and a non-specific
rash on the limbs
• The prodrome usually lasts 1–3 weeks before blisters
occur
• Severe irritation with a faint, dusky erythema in a
figurate pattern may sometimes precede blister
formation
• These changes may closely resemble erythema
multiforme or dermatitis herpetiformis
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's
textbook of dermatology. John Wiley & Sons; 2016 Feb 29.
27. Basil Tumaini
Clinical features …
• Sudden generalization of the true eruption of BP
follows and most of the body may be affected within
a week
• Blisters may arise on erythematous and on normal
skin
• The blisters are tense, dome shaped with a diameter
of many centimetres
• They appear mainly on the flexural aspects of limbs
and on the central abdomen
29. Basil Tumaini
Clinical features …
• The tough blisters may remain intact for several days
contents often becoming jelly-like with coagulated fibrin
• In some blisters, the fluid is reabsorbed and the epithelium
settles back in place like a skin graft
• Blisters that rupture leave erosions that heal rapidly, leaving
mild post-inflammatory changes (erythema, milia)
30. Basil Tumaini
Clinical features …
• Mucosal lesions (confined to the mouth) occur less
frequently and are less severe than in MMP and PV
• Leukocytosis, eosinophilia (in acute phase)
• Fever is uncommon
• patients’ general condition remains good despite
extensive blistering
• Fresh crops of small blisters may continue to occur
even after apparent control has been achieved
31. Basil Tumaini
Treatment
• The aim of treatment is to suppress disease activity
with the minimum dose of drugs necessary
• BP patients are elderly, commonly on many drugs,
and very susceptible ADR and SEs
• During prolonged treatment, it is advisable to aim for
the presence of a blister once every few weeks to be
certain that the patient is not being over treated
• Steroids are the mainstay of treatment
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's
textbook of dermatology. John Wiley & Sons; 2016 Feb 29.
32. Basil Tumaini
Treatment …
• For localized BP: very potent topical steroids are
often sufficient
– clobetasol propionate, applied all over twice daily
• Systemic steroids:
– 20 mg/day in localized or mild disease
– 40 mg/day in moderate disease
– 50-70 mg/day in severe disease
* Measures to prevent osteoporosis
33. Basil Tumaini
Treatment …
• Alternative therapies:
– tetracyclines and nicotinamide (for mild to
moderate disease)
• Immunosuppressants:
– azathioprine and MMF (if the steroid dose cannot
be reduced to an acceptable level)
Wojnarowska F, Kirtschig G, Highet AS, Venning VA, Khumalo NF. Guidelines for the
management of bullous pemphigoid. British Journal of Dermatology. 2002
Aug;147(2):214-21.
34. Basil Tumaini
Treatment …
• Tapering steroid dose
– reduced quite quickly over the course of a few weeks to a
dose of 15–20 mg/day and thereafter more slowly
– majority can be managed on doses of less than 10 mg/day
prednisolone
– reduction by 1 mg/month once the dose is below 10
mg/day
35. Basil Tumaini
Prognosis
• Untreated BP runs a chronic, self limiting course over
a number of months or years
• Disease duration is usually 3–6 years, most patients
achieving complete remission off treatment
• BP is fatal in one-third of untreated patients,
particularly in the active blistering phase with 2x risk
of death compared to the general population
• Poor prognostic indicators: advanced age,
generalized disease, low Karnofsky score, low
albumin and high doses of steroids
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's
textbook of dermatology. John Wiley & Sons; 2016 Feb 29.
36. Basil Tumaini
Related diseases
• Cicatricial pemphigoid (MMP)
• Epidermolysis bullosa acquisita
• Porphyria cutanea tarda
• Linear IgA bullous disease
• Dermatitis herpetiformis
37. Basil Tumaini
Rapid deterioration of the external surface of the eyes in a
patient with severe ocular cicatricial pemphigoid, including
erythema and suffusion of the conjunctiva and narrowing
of the fornices
41. Basil Tumaini
Pemphigus: Introduction
• Pemphigus is also an autoimmune mucocutaneous
blistering disease
• Patients with pemphigus typically have
autoantibodies which target specific proteins in the
epidermal-epithelial cell membrane resulting in the
loss of cell-cell adhesion
• These lesions typically present as blisters and ulcers
of mucosal and cutaneous tissues
Sami N, Yeh SW, Ahmed AR. Blistering diseases in the elderly:
diagnosis and treatment. Dermatologic clinics. 2004 Jan;22(1):73-86.
42. Basil Tumaini
Introduction …
• Pemphigus has been classified into three major
subtypes:
1) pemphigus vulgaris (PV)
2) pemphigus foliaceus (PF)
3) paraneoplastic pemphigus (PNP)
• PV accounts for 70% of pemphigus
Sami N, Yeh SW, Ahmed AR. Blistering diseases in the elderly:
diagnosis and treatment. Dermatologic clinics. 2004 Jan;22(1):73-86.
43. Basil Tumaini
PV epidemiology
• The mean age of onset is usually the 5th decade, but
it has been reported in the elderly
• Affects men and women equally
• Commoner in Eastern countries (India, Malaysia,
China) and the Middle East as well as among
Ashkenazi Jews
• No specific data for Tanzania
Sami N, Yeh SW, Ahmed AR. Blistering diseases in the elderly:
diagnosis and treatment. Dermatologic clinics. 2004 Jan;22(1):73-86.
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's
textbook of dermatology. John Wiley & Sons; 2016 Feb 29.
44. Basil Tumaini
PV risk factors
• Genetic factors: 1st degree relatives of patients with PV are
more susceptible than controls; certain MHC class II
genotypes - alleles of HLA-DRB1; certain haplotypes of the
desmoglein-3 gene
• Other disorders characterized by immunological
disturbances: thymoma or myasthenia gravis, SLE,
lymphoproliferative diseases
• Infections: EBV, HIV
• Drugs and toxins: OCPs, pesticide exposure
• Possibly protective effect from cigarette smoking*
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's textbook
of dermatology. John Wiley & Sons; 2016 Feb 29.
* Yoshida K, Takae Y, Saito H et al. Cutaneous type pemphigus vulgaris: a rare
clinical phenotype of pemphigus. J Am Acad Dermatol 2005; 52: 839–45.
45. Basil Tumaini
PV pathogenesis
• Autoantibodies against desmoglein-3 (sts
desmoglein-1), desmosomal cadherin involved in
mediating intercellular adhesion in the epidermis
• It expressed more strongly in buccal mucosa and
scalp skin than in skin from the trunk
• Mechanisms involved in acantholysis not fully
understood
• Hypothesis: antibody binding may cause direct
inhibition of the adhesive function of desmogleins
Tsunoda K, Ota T, Aoki M et al. Induction of pemphigus phenotype by a mouse
monoclonal antibody against the amino-terminal adhesive interface of
desmoglein 3. J Immunol 2003; 170: 2170–8.
46. Basil Tumaini
Pathology
The diagnosis of PV is confirmed by direct immunofluorescence which
shows IgG deposited on the surface of keratinocytes throughout the
epidermis in and around lesions
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's textbook of dermatology. John Wiley & Sons; 2016 Feb 29.
47. Basil Tumaini
Clinical features
• Nearly all patients have mucosal lesions
– Oral lesions in 50 to 70% of patients
– may precede cutaneous lesions by months
– May be the only manifestation of the disease
– Intact bullae are rare in the mouth
– More commonly, ill-defined, irregularly shaped
buccal or palatal erosions, slow to heal
• Other mucosal surfaces: conjunctiva, nasal, pharynx,
larynx, oesophagus, urethra, vulva and cervix
Sami N, Yeh SW, Ahmed AR. Blistering diseases in the elderly:
diagnosis and treatment. Dermatologic clinics. 2004 Jan;22(1):73-86.
48. Basil Tumaini
PV: erosions in mouth
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's textbook
of dermatology. John Wiley & Sons; 2016 Feb 29.
49. Basil Tumaini
Clinical features
• Most patients develop cutaneous lesions
• Predilection for the scalp, face, axillae, groins and
pressure points
• Flaccid blisters filled with clear fluid either arise on
normal skin or an erythematous base
• The contents soon become turbid or the blisters
rupture, producing painful erosions which extend at
the edges as more epidermis is lost
• At this stage, the Nikolsky sign is positive
52. Basil Tumaini
Clinical features
• Healing occurs without scarring but pigmentary
change and acanthomas may occur
• Lesions in skin folds readily form vegetating
granulations, flexural PV merges with its variant
pemphigus vegetans (shown below)
53. Basil Tumaini
Treatment of PV
• Oral disease
– Good oral hygiene including treatment of
periodontal disease
– Potent topical or intralesional steroids
– stop oral steroids when the disease is inactive
– topical nystatin reduces the risk of oral candida
• Systemic therapy
– Corticosteroids with an adjuvant
– Immunosuppressive agents
– Plasmapheresis or IVIg
– PUVA
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's textbook
of dermatology. John Wiley & Sons; 2016 Feb 29.
54. Basil Tumaini
Treatment of PV …
• Prednisolone 1.0 to 1.5 mg/kg/day + topical or
intralesional steroids (Optimum: Prednisolone 60–
100 mg/day or IV pulses of either 1 g
methylprednisolone or 100 mg dexamethasone are
safer alternatives)
– The dose should be titrated to the clinical
response
55. Basil Tumaini
Treatment of PV …
• Generalized disease requires more aggressive
immunosuppression to suppress blistering
– High doses of Prednisolone (120–240 mg/day)
have been recommended for severe pemphigus
– Challenge: achieving a balance between the risks
associated with high-dose steroid therapy and
those of poorly controlled disease
56. Basil Tumaini
Adjunctive treatment
• Azathioprine
– is widely used in a dose of 2.5 mg/kg/day
– The combination of prednisolone and azathioprine
is more effective than prednisolone alone both in
terms of mortality and remission
• Mycophenolate mofetil (2 g/d) has been found
helpful as a steroid sparing agent
• Other therapies: tetracycline + prednisolone, PO or
IM gold + P; plasmapheresis + steroids; high dose
IVIg (short-lived benefit)
57. Basil Tumaini
Prognosis
• Without treatment with corticosteroids most
patients with PV will die
• Treatment with systemic steroids has reduced the
mortality to between 5% and 15%
• Morbidity and mortality are related to:
– extent of disease
– maximum dose of prednisolone required to
induce remission
– presence of co-morbidities
• Prognosis is worse in older patients
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's textbook
of dermatology. John Wiley & Sons; 2016 Feb 29.
59. Basil Tumaini
Pemphigus foliaceus (PF)
• PF is considered a less severe form of the disease
and rare in the elderly*
• A small group of PF patients can progress to have
extensive involvement →exfoliative erythroderma*
* Sami N, Yeh SW, Ahmed AR. Blistering diseases in the elderly:
diagnosis and treatment. Dermatologic clinics. 2004 Jan;22(1):73-86.
Griffiths C, Barker J, Bleiker T,
Chalmers R, Creamer D, editors.
Rook's textbook of dermatology.
John Wiley & Sons; 2016 Feb 29.
60. Basil Tumaini
PF
• IgG autoantibodies in the sera of patients with PF
more specifically recognize desmoglein-1
• Patients develop superficial blisters on an
erythematous base that erode easily, no mucous
membrane involvement
Sami N, Yeh SW, Ahmed AR. Blistering diseases in the elderly:
diagnosis and treatment. Dermatologic clinics. 2004 Jan;22(1):73-86.
61. Basil Tumaini
PF
• The treatment of PF is usually less challenging as
compared with PV since it is more benign
• Local and systemic treatments are similar to those
used in PV
• Pemphigus foliaceus has a better prognosis
compared with PV except in erythrodermic and
refractory forms of PF
Sami N, Yeh SW, Ahmed AR. Blistering diseases in the elderly:
diagnosis and treatment. Dermatologic clinics. 2004 Jan;22(1):73-86.
63. Basil Tumaini
Paraneoplastic pemphigus (PNP)
• Distinctive form of pemphigus described in
association with a variety of underlying neoplasms
such as:
– Hodgkin’s lymphoma
– CLL
– Castleman’s disease
– thymomas
– Waldenstrom’s macroglobulinemia
– Bronchogenic carcinoma
Sami N, Yeh SW, Ahmed AR. Blistering diseases in the elderly:
diagnosis and treatment. Dermatologic clinics. 2004 Jan;22(1):73-86.
64. Basil Tumaini
PNP
• In 2/3, PNP occurs with an existing neoplasm
• In the remainder the neoplasm is detected after the
mucocutaneous disease has occurred
• May present many years after a malignancy is
considered to be in remission
• Some cases have been linked with the use of
fludarabine
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's textbook
of dermatology. John Wiley & Sons; 2016 Feb 29.
65. Basil Tumaini
PNP: clinical features
• Affects both mucosal and cutaneous tissues
• Cutaneous disease can mimic other dermatologic
conditions including PV, erythema multiforme, and
lichen planus
• Patients have severe mucosal erosions and
polymorphous cutaneous signs including blisters,
erosions, particularly on the upper body, and
palmoplantar target lesions
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's textbook
of dermatology. John Wiley & Sons; 2016 Feb 29.
67. Basil Tumaini
Diagnosis
• DIF reveals IgG and/or complement at the BMZ as
well as on the surfaces of keratinocytes
• Antibodies are predominantly antiplakin antibodies
• Immunoprecipitation is the gold standard test for the
diagnosis of PNP:
– can confirm the presence of specific autoAb in the sera of
patients with PNP
– These include: desmoplakin I and II, BP antigen 1 and
periplakin.
Sami N, Yeh SW, Ahmed AR. Blistering diseases in the elderly:
diagnosis and treatment. Dermatologic clinics. 2004 Jan;22(1):73-86.
68. Basil Tumaini
Treatment
• Paraneoplastic pemphigus is generally refractory to
all treatments
• Most patients deteriorate inexorably, with death
usually secondary to sepsis, GI bleeding, multiorgan
failure or respiratory failure
• Cases associated with benign or low-grade neoplasia,
such as thymoma or Castleman’s disease may remit
partially after surgical removal of the neoplasm
Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's textbook
of dermatology. John Wiley & Sons; 2016 Feb 29.
Sami N, Yeh SW, Ahmed AR. Blistering diseases in the elderly:
diagnosis and treatment. Dermatologic clinics. 2004 Jan;22(1):73-86.
69. Basil Tumaini
Other common blistering diseases in elderly
• Infections
– Herpes simplex
– Herpes zoster
• Drugs
– Erythema multiforme
– SJS/ TEN
70. Basil Tumaini
Keypoints
• Blistering diseases in elderly may have various
etiologies including drugs, infections, autoimmune,
genetic and metabolic
• Good knowledge of the different presentations of
blistering disorders facilitates prompt diagnosis and
management
• The diagnosis of both immune- and non–immune-
mediated blistering disorders can be confirmed with
the help of histologic and immunopathologic studies
71. Basil Tumaini
• The treatment of blistering disorders includes both
localized and systemic treatments
• Elderly are more prone to the SEs of therapy, routine
monitoring is essential
• Elderly patients almost invariably have multiple co-
morbidities, treatment should be evaluated carefully
and appropriate follow-up is warranted
72. Basil Tumaini
Further reading
• Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's
textbook of dermatology. John Wiley & Sons; 2016 Feb 29.
• Kim M, Borradori L, Murrell DF. Autoimmune blistering diseases in the
elderly: clinical presentations and management. Drugs & aging. 2016 Oct
1;33(10):711-23.
• Mutasim DF. Autoimmune bullous dermatoses in the elderly. Drugs &
aging. 2010 Jan 1;27(1):1-9.
• Sami N, Yeh SW, Ahmed AR. Blistering diseases in the elderly: diagnosis
and treatment. Dermatologic clinics. 2004 Jan;22(1):73-86.
• Wojnarowska F, Kirtschig G, Highet AS, Venning VA, Khumalo NF.
Guidelines for the management of bullous pemphigoid. British Journal of
Dermatology. 2002 Aug;147(2):214-21.
Editor's Notes
Not very clear which auto-Ag is most important, probably BP180
Limited to mucous membranes entropion corneal injury visual loss
Slow onset, chronic autoimmune subepidermal (blisters, scars, milia at trauma prone areas=hands and feet) blistering disease
Skin and mucous membranes
AB to type VII collagen (at DEJ)
Large flat blisters
Tx: Dapsone 25mg (initial dosage, increase as tolerated), GCS.
Deficiency of UROD
RF: induced by estrogen, alcoho, a/w hepatitis C
Blisters on sun exp, skin fragility, milia, hypertrichosis
Urine and fecal porphyrins: copro & uroporphyrins
Tx: Serial phlebotomy ± LD antimalarials
Variegate porphyria=PCT+systemic sx Tx: glucose, hematin
Extremely itchy grouped vesicles, extensor surfaces, erosions
A/w gluten sensitive enteropathy-celiac dse
Tx: Dapsone 50-100 mg PO OD (R/O: G6PD def)
IF: IgA in dermal papillae in a granular pattern
In Linear IgA bullous dermatosis (LABD) – IgA in a linear pattern
** Mponda K, Masenga J. Skin diseases among elderly patients attending skin clinic at the Regional Dermatology Training Centre, Northern Tanzania: a cross-sectional study. BMC research notes. 2016 Dec;9(1):119.
Acantholysis=Separation of individual epidermal keratinocytes from their neighbor
Pemphigus vulgaris. Erosions in mouth
Nikolsky sign=firm sliding pressure with a finger separates normal-looking epidermis from dermis, producing an erosion
Acanthoma=A tumor formed by proliferation of epithelial squamous cells
* Azathioprine may be an effective monotherapy in mild cases, although the therapeutic effect is delayed for 3 to 5 weeks
* Cyclophosphamide alone is not effective in pemphigus
* Ciclosporin alone is not helpful in the acute phase of the disease
Usually no blisters are seen
DIF=direct immunoflorescence
BMZ=basement membrane zone