2.2. COMPOSICIÓN DE LA SALA DE ALCALDES
2.2.1. LOS ALCALDES DE CASA Y CORTE
or groups of tiny vesicles. However, they may be excoriated and it may be difficult to find the intact lesions. The vesicles arise on erythe-matous base and are grouped. Bullae are infrequent. The extensor aspects of the limbs, knees, elbows, buttocks, and the natal cleft are the usual sites of affliction (Table 27.1) (Figs 27.1A and B).
The axillary folds, shoulders, trunk, face, and scalp may also be involved. Mucosae are usually spared. The small bowel disease asso-ciated with dermatitis herpetiformis is similar to that seen in adult celiac disease. However, it may be asymptomatic or less severe. Sympto-matic steatorrhea occurs in less than 5 percent of DH patients. Chemical evidence of steator-rhea and abnormal D-xylose absorption occurs in less than one-third of the cases. However, 90 percent of dermatitis herpetiformis patients demonstrate histologic evidence of glutensen-sitive entero pathy. DH has an association with thyroid diseases. Antithyroglobulin and anti microsomal thyroid antibodies have also been demonstrated. Sporadic association with other autoimmune disorders and malignancies has been recorded. Furthermore, dermatitis herpeti formis may be precipitated by gluten containing diets, namely wheat, barley, rye, oats, iodine; thyroid replace ment therapy; and viral illnesses.
123
Dermatitis Herpetiformis
table 27.1: Diagnosis of dermatitis herpetiformis
Histopathology Direct immunofluorescence Indirect immunofluorescence
1. Accumulation of neutrophils at the Granular deposits of IgA at the dermo- Circulating IgA antibasement tips of dermal papillae forming epidermal junction of the uninvolved membrane zone antibodies are
microabscesses (Figs 27.2A and B). skin. demonstrable in only 2 percent
of the cases.
2. Dermal papilla appears necrotic Pivotal complement component C3 due to the presence of fibrin.
epidermal junction.
3. Microabscesses result in the Immunoelectron microscopy localizes separation of the tips of dermal the granular deposits of IgA at the tips papilla from the epidermis. of dermal papilla, beneath the lamina Early blisters are multilocular. densa, in the area of the anchoring
fibrils.
4. Rete ridges lose their attachment They are closely associated with the to the dermis. Multilocular bundles of microfibrils of the elastic blisters become unilocular. fibers.
Figures 27.2a and B: (A) Accumulation of neutrophils at the tips of dermal papillae forming microabscesses (H and E × 40) (B) Higher magnification (H and E × 100)
Figures 27.1a and B: (A) Dermatitis herpetiformis (B) Dermatitis herpetiformis grouped paulo-vesicular eruption over brick red base
124
Textbook of Clinical Dermatologytable 27.2: Differential diagnosis of bullous disorders
Pemphigus Pemphigoid Linear Ig/A Herpes Chronic bullous Dermatitis
dermatosis gestationis disease of herpetiformis
childhood
Age of onset 4th and 5th 6th to 8th Middle age Pregnant women Ist decade of life 3rd decade
decade decade
Morphology Discrete, flaccid Tense bullae Bilsters Erythematous, Grouped, tense Vesicles and/or bullae arising arising over intermediate in edematous vesicles and/or papulo vesicles of 3
over apparently erythematous size between papulovesicles bullae. Cluster of to 6 mm in size.
normal skin. and/or urticarial those of bullous and plaques jewels Distribution is
Nikolsky/bulla plaques. Localize pemphigoid and and/or large arrangement. symmetrical over spread sign are in the flexures. dermatitis tense bullae. Localize in the extensor surfaces.
positive. Involves Nikolsky/bulla herpetiformis. Lesions may be flexures and Elbows, proximal scalp, chest and spread sign are Localize in grouped or pelvic region. forearms, knees,
intertriginous negative. flexural area. coalesce. buttocks, and
areas. Abdomen, sacrum involved.
palms, soles, chest, and face usually involved.
Mucosal Involved in Oral lesions in Infrequent Mucosae spared. Mucosae spared Mucosa spared involvement almost all cases. 20 percent cases.
May precede Involvement of
and/or other mucosae
Variants Pemphigus Classical bullous Nil Nil Nil Nil
vulgaris and its pemphigoid, variant localize bullous
pemphigus pemphigoid,
vegetans. Nodular
Pemphigus (hyperkeratotic) foliaceus and its bullous
variant pemphigoid,
Associated Nil Nil Nil Nil Nil Gluten-sensitive
features enteropathy
HLA association HLA-A10 Nil Nil Nil HLA-B8 HLA-B8
HLA-A26 HLA-DRW3
HLA-DRW4
especially in Jews
Precipitating Drugs like Nil Nil Pregnancy, Nil Ingestion of gluten
factors D-penicillamine, usually the 2nd containing diet.
rifampicin, and 3rd Also iodine, thyroid
piroxicam, trimester. At dysfunction and
(Contd...)
125
Dermatitis Herpetiformis
phenytoin, times flares up
thyroid replace-phenobarbitone, at postpartum ment therapy.
thiopronine, period
a-mercapto-proprionyl glycine, captopril.
Location of Intraepidermal, Subepidermal, Subepidermal, Subepidermal, Subepidermal, Subepidermal, at the blister suprabasal in in the lamina in the lamina in the lamina in the lamina the tip of dermal
pemphigus lucida of lucida of BMZ lucida of BMZ lucida of BMZ papillae
vulgaris. the BMZ.
Subcorneal in pemphigus foliaceus.
Direct immuno- Deposition of Linear Linear deposits Linear deposits Linear deposits Granular deposits fluorescence IgG and pivotal deposition of of IgA at the of C3 at the of IgA at the of IgA in the tip of complement IgG and C3 at (1) Lamina lamina lucida of lamina lucida of dermal papillae of component C3 in the lamina lucida of BMZ the BMZ. 30 the BMZ uninvolved skin the intercellular lucida of or (2) below percent cases
space. the BMZ BMZ also show
deposits of IgG at the same site.
Indirect IgG pemphigus Serum IgG Negative Serum IgG Circulating IgA Negative.
immuno- antibody in the antibasement antibasement antibasement
fluorescence serum. Titers membrane zone membrane zone membrane zone
proportional to antibodies in 90 antibodies in 10 antibodies.
the severity of percent cases. to 20 percent of
the disease. the cases.
Treatment Corticosteroids Corticosteroids Dapsone, either Corticosteroids Dapsone, either Dapsone in high doses. At in moderately alone or in com- alone or in
com-remission taper high doses.At bination with bination with
steroids and add remission taper corticosteroids. corticosteroids.
immunosuppres- steroids and add sive adjuvants. immunosuppres-Maintenance sive adjuvants.
dose of steroids Maintenance
required. therapy not
required.
(Contd...)
Pemphigus Pemphigoid Linear Ig/A Herpes Chronic bullous Dermatitis
dermatosis gestationis disease of herpetiformis
childhood
Diagnosis
A high index of suspicion is required to form the clinical diagnosis of dermatitis herpetifor-mis. Its pleomorphic manifestation may make it difficult to differentiate this entity from ery
thema multiforme, pemphigus herpetiformis, neurotic excoriations, scabies, papular urticaria, transient acantholytic dermatoses, pemphigoid, and herpes gestationis. Histopathology, and immunofluorescence are required to confirm the diagnosis (Table 27.1).
treatment
Diaminodiphenyl sulfone (DDS) is the treat-ment of choice. Treattreat-ment may be initiated with 100 to 200 mg of dapsone. It dramatically improves pruritis and prevents new lesion formation within 24 to 48 hours. An occasional patient may require 300 to 400 mg of dapsone for initial improvement. Strict adherence to a gluten free diet may produce improvement of clinical symp toms, and a decrease in dapsone requirement.
126
Textbook of Clinical Dermatology reCommenDeD reaDing1. Hall RP. The pathogenesis of dermatitis herpeti
formis: Recent advances. I Am Acad Dermatol 1987;16:11291144.
2. Katz SI, Hall RP 3rd, Lawley TJ et al. Dermatitis herpeti formis: the skin and the gut. Ann Intern Med 1980;93:857874.
3. Lawley TJ, Yancey KB. Dermatitis herpetiformis.
Dermatol Clin 1983;1:187194.
4. Uander Meer JB. Glutenfree diet and elemental diet in dermatitis herpetiformis. Int J Dermatol 1990;29:679692.
Lichen planus is a papulosquamous disorder of debatable etiology, characterized by the for
mationofflattopped,polygonal,greyishwhite,
purple/liliac eruptions. Middle age people of both the sexes are its victims.
Its precise etiology is unknown. However,
it may either be bacterial or viral in origin.
Immunologic factors are also incriminated due to the presence of consistent immunofluores
cencepattern.Itmayalsofollowbonemarrow
transplantation or graft versus host reaction.
Furthermore, certain individuals are genetically predisposed to it. Also several drugs such as chloroquine, quinacrine, streptomycin, para
aminio salicylic acid (PAS), methyldopa, quini
dine, phenothiazine, chlorpropamide, gold, bismuth, levamisole, and penicillamine are incri minated. Exposure to paraphenylenedia
mine salts encountered in colorphotographic deve loper may also produce these lesions.
CliniCal Features
It is characterized by the formation of flat
topped,polygonal,greyishwhite,purple/liliac
eruptions (Figs 28.1A and B). Its surface is scaly, andistraversedbyfinewhitelines,‘Wickham’s
striae’whichbecomeprominentaftertheappli
cation of an emolient. The papules may coalesce to form plaques. The papules may be scattered or grouped. They may be linear over the marks
28 Lichen Planus
Figures 28.1a and B: Flat-topped, polygonal greyish white, purple/iliac scaly eruptions traversed by fine white lines (Wickhams’ striate). Papules coalesce to form plaque
a
B
128
Textbook of Clinical Dermatology of excoriation or trauma (Koebner’s phenomenon).Theytendtoinvolvetheflexorsurfaces
ofthewristandforearms,lumbararea,ankles,
glanspenis,anterioraspectoflowerlegs,and
the dorsal surfaces of the hands.
Mucosal surfaces are involved in nearly half the patients. The buccal mucosa and the tongue are most frequently affected but the lips, gums, palate, conjunctivae, larynx, genitalia, and gas
trointestinal tract may also be involved. The mucosal lesions consist of lacy, reticulated, white streaks, papules, plaques, and erosions
(Fig. 28.2). Chronic erosive oral lichen planus may predispose to squamous cell carci noma.
Nearly 25 percent of the male patients have involvement of genitalia. Lesions are often present over the glans and these may be typical papules in an annular configuration. Occasio
nally, the glans penis may have erosive lesions.
Nails are affected in 10 percent of the cases and the changes include:
• Pterygium, a hallmark in lichen planus. It
results from the fusion of the proximal nail foldwiththenailbedresultinginthelossof
proximal nail plate.
Acute lichen planus usually resolves in 6 to 18 months. The lesions heal leaving behind hyperpigmentation which may take years to
resolve. Chronic lichen planus persists for a long period. Oral lichen planus, hypertrophic
lichen planus, and lichen planopilaris tend to be chronic.
Lichen planus may manifest as any of the followingvariants.
Annular lichen planus: It results in a ring of typical lichen planus papules that spread peri
pherally and produce central clearing (Figs 28.3A and B).
Linear lichen planus: It consist of typical lichen planuslesionsinalinearfashion.Occasionally,
it may be in a zosteriform distribution.
Hypertrophic lichen planus (lichen verruco
sus): It arises on the shins, ankles, and soles.
It consists of intensely pruritic, lichenified,
scaly, violaceous, verrucous hyperpigmented plaques. The lesions are often symmetric and chronic (Fig. 28.4).
Atrophic lichen planus: It may occur over the mucous membranes. Atrophic lichen planus mayproduceatrophicwhitespotswhichneed
to be differentiated from lichen sclerosus et atrophicus and guttate morphea.
Vesiculobullous lichen planus: It may arise on preexisting lichen planus lesions or de novo. It is the result of separation at the dermoepidermal
Figure 28.2: Mucous membrane: Display lina, reticulate white streaks, papules and erosions
129
Lichen Planus
junction secondary to basal cell dege neration.
It requires to be differentiated, from lichen pla
nus pemphigoides, which is a coexistence of
two distinct disease entities and pemphigoid
anti bodies (IgG) can be demons trated at the lamina lucida of basement mem brane zone by immunofluorescence.
Lichen planus actinicus: This variant is usually encountered in tropics, on sunexposed areas.
Lesions are pigmented, dyschromic or granu
lomaannulare like and only mildly pruritic (Fig. 28.5).
Lichen planus erythematosus: It is observed in older patients and consists of nonpruritic,
Figures 28.3a and B: Annular lichen planus
a B
Figure 28.4: Hypertrophic lichen planus Figure 28.5: Lichen planus actinicus
130
Textbook of Clinical Dermatology soft, red papules, usually located on the forearms.
Lichen planopilaris: It presents as acuminate, hyperkeratotic, follicular papules, primarily on the scalp. The affected area may also depict scaling. Alopecia with atrophy may super
vene. A variant of lichen planopilaris is Grahm LittlePiccardiLassueur syndrome. It consists of cica t ri cial scalp alopecia (Fig. 28.6), follicular keratotic lesions of the glabrous skin, and non
cicatricial alopecia of the axillae and groins.
Ulcerative lichen planus: It consists of ulce ra
tions over the soles of the feet and of the buccal mucosaassociatedwithcicatricialalopecia,and
loss of toenails. Typical lichen planus lesions
may be present over other parts of the body.
The lesions over the feet are painful. Squamous cell carcinoma may supervene.
Twentynail dystrophy (TND): Trachyonychia, a fascinating clinical condition, was brought
to focus 25 years ago. Ever since, it has been sparingly reported. Nonetheless, the condition iswellrecognized,anditsdiagnosisismadeon
the basis of clinical features characterized by onset in infancy/childhood, and occasionally in adults. The lesions are fairly representative, and are characterized by the alternating elevation and depression (ridging) and/or pitting, lack of luster, roughening likened to sandpaper, splitting,andchangetoamuddygrayishwhite
color. Dystrophy is prominent. Several modes of occurrence have been described including a hereditary component. The confirmation of
diagnosis is through microscopic pathology corresponding either to endogenous eczema/
dermatitis, lichenplanus like or psoriasic
form. It is a selflimiting condition and may occasionally require intervention.
Diagnosis
Thediagnosisoflichenplanusisclinical;how
ever, it may be supplemented by histopatho
logy.Hematoxylineosinstainedsectionreveals
the presence of: (1) Hyperkeratosis, (2) Focal
hypergranulosis, (3) Irregular acanthosis resul
ting in saw tooth appearance of rete ridges,
(4) Liquefaction degeneration of basal cell layer, (5)Abandlikeupperdermallymphocyticinfilt
rate, (6) Incontinence of the melanin, (7) Colloid bodies may be present in the deep dermis, (8) Small separation between dermis and
epider mis may be present (MaxJoseph spaces) (Figs 28.7 to 28.9).
Figure 28.6: Lichen planopilaris affecting the scalp
131
Lichen Planus
Figure 28.7: Lichen planus (H and E × 100) Figure 28.8: Lichen planus (H and E × 400)
Figure 28.9: Lichen planus hypertrophicus (H and E × 50)
treatment
Lichen planus tends to resolve spontaneously after varying period of time. In mild cases, treat
ment is symptomatic. Antihistamines may be administered to relieve pruritus. Topical cortico
ste roids may be applied for their anti pruritic
and antiinflammatory effects. Severe, acute,
widespread lichen planus may benefit from a
tapered course of corticosteroid. Griseofulvin may also be beneficial. Oral lichen planus
respond to topical application of cortico
steroid in oral protective base. Hypertrophic
132
Textbook of Clinical Dermatologytable 28.1: treatment of lichen planus
Types of lichen planus Treatment of choice Other modalities of treatment 1. Acute widespread a. 40 to 60 mg of prednisolone every day, 1 mg/kg of etretinate every day
lichen planus tapered off over 2 to 3 months. tapered to a maintenance dose b. 250 mg of griseofulvin (Idifulvin) twice of 25 to 50 mg on alternate
a day with milk or fatty food. days after control 2. Chronic localized a. Antihistamines to relieve itching
lichen planus 25 mg of promethazine hydrochloride (Phenergan) thrice a day
25 mg of pheniramine maleate (Avil) thrice a day.
b. 5 mg of diazepam (Calmpose) at bed time.
c. Topical corticosteroids for local application Clobetasol propionate 0.05 percent (Tenovate) Fluocinotone acetonide 0.025 percent (Flucort) Betamethasone valerate 0.12 percent (Betnovate).
3. Hypertrophic a. Intralesional corticosteroid
lichen planus 0.1 to 0.3 mL of triamcinolone acetonide 10 mg/mL (kenacort), intralesional once a week b. Topical corticosteroidOr
Clobetasol Propionate (0.05 percent) under occlusion.
4. Oral lichen planus Potent steroids in Orabase for local application. 200 mg of metronidazole (Flagyl) thrice daily
Or Or
40 to 60 mg of prednisolone, in divided doses, 100 mg of isotretinoin, once a
every day day
5. Lichen planopilaris Fluocinolone acetonide 0.025 percent lotion (Flucort) for local application
OrBetamethasone valerate 0.1 percent lotion Or0.1 to 0.3 mL of triamcinolone acetonide 10 mg/mL (Kenacort), intralesional, once in two weeks.
lichen planus may benefit from intralesion
al corticosteroids or potent corticosteroids applied under occlusion (Table 28.1).
reCommenDeD reaDing
1. BoydAS,NeldnerKH.Lichenplanus.J Am Acad Dermatol 1991;25:593619.
2. Fox BJ, Odom KB. Papulosquamous diseases: A
review,J Am Acad Dermatol 1985;12:597624.
3. Sehgal VN. Abraham GJ, Malik GB. Griseofulvin therapy in lichen planusa double blind controlled trial. Br J Dermatol 1972;87:383385.
4. Sehgal VN. Lichen planusan appraisal of 147 cas
es. Ind J Dermatol Venereol. 1974; 40: 104.
5. SehgalVN.Twentynaildystrophytrachyonychia:
anoverview.J Dermatol 2007;34:361366.
Lichen nitidus is an asymptomatic papulo
squamous eruption characterized by numerous, tiny, discrete, flesh colored papules. It affects children and young adults of both the sexes.
CliniCal Features
The lesions are pinheadsized, flat, with a shiny surface. Some lesions are dome shaped and may have a central depression. They are discrete, and occasionally may coalesce to form plaques.
Koebner’s phenomenon may be elicited. They are distributed over the penis, arms, forearms, and abdomen.
Diagnosis
It is made clinically and confirmed by histopa
thological examination. Hematoxylin and eosin stained section reveals (Figs 29.1A and B; 29.2) the following:
• Focal accumulation of inflammatory cells in the widened dermal papilla. The infiltrate con sists of epithelioid cells, lymphocytes, and an occasional multinucleate giant cell
• The infiltrate is grasped by elongated, ‘claw
like’ rete ridges
• Hydropic degeneration of basal cells
• Thin/absent granular layer
• Parakeratotic epidermis over the dermal infil trate.