29 - Pênfigo

Question 1

Apresentações incomuns de pênfigo vulgar ? (5)

Answer 1

-placas crostosas isoladas na face e escalpe;
-paroníquia e/ou onicomadese;
-úlcera de pé;
-eczema desidrótico ou pompholyx;
-macroglossia.

Question 2

Achados histológicos Pênfigo vulgar?
E no Pênfigo vegetante?

Answer 2

-bolha intraepidérmica com acantólise supra-basal;
-SEM QUERATINÓCITOS NECRÓTICOS;
-linha de células basais unidas à MB com aspeto “row of tumbstones”;
-bolha pode conter células inflamatórias (+eosinofilos);
-papilas dérmicas mantidas e podem fazem providência para a cavidade da bolha.
-derme com infiltrado mononuclear e alguns eosinofilos.
-raramente: achado inicial - espongiose eosinofilica.

No pênfigo vegetante: acantólise suprabasal + papilomatose + acantose.

Question 3

Causas de Espongiose eosinofílica?
a) d. bolhosas
b) d. não bolhosas

Answer 3

a)
Pemphigus vulgaris and its variants
Pemphigus foliaceus
Bullous pemphigoid and its variants
Pemphigoid gestationis (herpes gestationis)
Mucous membrane (cicatricial) pemphigoid
Linear IgA bullous dermatosis.

b)
Insect-bite reaction
Drug eruptions
Id reactions
Wells syndrome
Polymorphic eruption of pregnancy
Erythema toxicum neonatorum
Incontinentia pigmenti (first stage)
Atopic dermatitis
Contact dermatitis
Eosinophilic folliculitis
Mycosis fungoides
Prurigo pigmentosa
Still disease.

Question 4

Histologia do pênfigo foliáceo?

Answer 4

-acantólise na epiderme superior, ao nível da camada granulosa.
-histologia = ao impétigo bolhoso e síndrome pele escaldada estafilocócica.
-inicialmente tb pode ter espongiose eosinofilica.

Question 5

Histologia do pênfigo paraneoplásico?

Answer 5

1-acantólise suprabasal;
2-necrose individual de queratinócitos;
3-linfócitos na epiderme;
4-dermatite de interface;

Question 6

Histologia do pênfigo IgA?

Answer 6

-pustula/vesícula intraepidérmica;
-conteúdo da pústulas: +++ neutrófilos;
-ACANTÓLISE AUSENTE.

a) tipo dermatose pustulosa subcornea: pustula subcorneana;
b) neutrofílico intraepidérmico: pustula suprabasal (epiderme inf ou toda).

Question 7

Exame de diagnóstico mais sensível e confiável para dx de qualquer pênfigo?

Answer 7

DIF.

Question 8

Qual o gold-standard para dx de pênfigo?

Answer 8

Demonstração de IgG (ou IgA no pênfigo IgA) contra a superfície dos queratinócitos.

Question 9

DD de pênfigo vulgar?

Answer 9

a) lesões mucosas:
-acute herpetic stomatitis,
-aphthous stomatitis,
-erythema multiforme major or Stevens–Johnson syndrome,
-lichen planus,
-systemic LE,
-mucous membrane (cicatricial) pemphigoid.

b) lesões cutâneas:
-other forms of pemphigus,
-bullous pemphigoid,
-linear IgA bullous dermatosis,
-erythema multiforme,
-Hailey–Hailey disease,
-transient acantholytic dermatosis (Grover disease).

Question 10

DD de pênfigo foliáceo?

Answer 10

-other forms of pemphigus,
-bullous impetigo,
-subcorneal pustular dermatosis,
-subacute cutaneous LE,
-seborrheic dermatitis

Question 11

DD de pênfigo vegetante?

Answer 11

-d Hailey-Hailey,
-penfigoide vegetante,
-pioderma blastomicose-like,
-piodermatite-piostomatite vegetante,
-halogenodermia,
-infeções (granuloma inguinal, condilomas latas, fungos dimorficos, cromomicose, condiloma acuminado, herpes simples vegetaste, herpes zoster verrugoso, granuloma ameba),
-d Paget extramamário.

Question 12

DD de pênfigo paraneoplásico?

Answer 12

-PV,
-penfigoide das membranas mucosas,
-EM ou SSJ,
-liquen plano,
-GVHD,
-infeção HSV persistente,
-outras infeções víricas,
-estomatite por QT (duração 7-14 dias).

Question 13

Doenças com crostas hemorrágicas nos lábios?

Answer 13

-Herpes simplex
-Herpes zoster
-Erythema multiforme major
-Stevens–Johnson syndrome/TEN spectrum
-Pemphigus vulgaris
-Paraneoplastic pemphigus
-Contact cheilitis

Question 14

Gold-standard para dx de pênfigo paraneoplásico?

Answer 14

Demonstração da combinação de auto-Acs IgG anti-plaquina (imunoprecipitação, imunoblot, ELISA) e anti-desmogleína (ELISA).

Question 15

DD de pênfigo IgA?

Answer 15

-dermatose pustulosa subcornea (histologia =),
-PF,
-impétigo bolhoso,
-DH,
-DBAL,
-psoríase pustulosa.

Question 16

Achado clínico que ajuda na distinção entre pênfigo paraneoplásico e pênfigo vulgar?

Answer 16

Bolhas e lesões eritema multiforme-like nas palmas e plantas ajudam a diferenciar o p. paraneoplásico do p. vulgar, em que as palmas e plantas estão habitualmente poupadas.

Question 17

Fala do pênfigo IgA.

Answer 17

IgA pemphigus represents a more recently characterized group of autoimmune intraepidermal blistering diseases presenting with a vesiculopustular eruption, neutrophilic infiltration of the skin, and in vivo bound and circulating IgA autoantibodies against the cell surface of keratinocytes; no IgG autoantibodies are present.

IgA pemphigus usually occurs in middle-aged or elderly persons.

Two distinct types of IgA pemphigus have been described: the subcorneal pustular dermatosis type and the intraepidermal neutrophilic type.

Patients with both types of IgA pemphigus present with flaccid vesicles or pustules on either erythematous or normal skin. In both types, the pustules tend to coalesce to form an annular or circinate pattern with crusts in the center of the lesion, although a sunflower-like configuration of pustules is a characteristic sign of the intraepidermal neutrophilic type.
The most common sites of involvement are the axilla and groin, but lesions can also develop on the trunk and proximal extremities.
Mucous membrane involvement is rare, and pruritus is often a significant symptom.

Because the subcorneal pustular dermatosis type of IgA pemphigus is clinically and histologically indistinguishable from classic subcorneal pustular dermatosis (Sneddon–Wilkinson disease), immunologic evaluation is essential to differentiate the two diseases.

IgA deposition on cell surfaces of epidermal keratinocytes is present in all cases, as shown by direct
immunofluorescence (DIF) microscopy, and many patients have detectable circulating IgA autoantibodies, as shown by indirect immunofluorescence (IIF) microscopy.

In the subcorneal pustular dermatosis type, IgA autoantibodies tend to react against upper epidermal surfaces, while in the intraepidermal neutrophilic type, IgA autoantibodies are found throughout the entire epidermis.

The subclass of IgA autoantibodies is exclusively IgA1. IgA autoantibodies in the subcorneal pustular dermatosis type were shown to recognize desmocollin 152, while the autoimmune targets of the intraepidermal neutrophilic type remain to be identified. A subset of IgA pemphigus patients have IgA autoantibodies directed against Dsg1 or Dsg3, making the autoimmune targets of IgA pemphigus more heterogeneous. The exact pathogenic role of IgA autoantibodies in inducing pustular formation in IgA pemphigus remains to be elucidated.

Question 18

Pênfigo herpetiforme?

Answer 18

• Variante de PF (+++) ou de PV;
• Caracteriza-se por:
  1) placas urticariformes eritematosas e vesícula tensas em distribuição herpetiforme;
  2) espongiose eosinofílica e pústulas subcórneas com acantólise mínima ou ausente;
  3) auto-Acs IgG contra a superfície dos queratinócitos.