194 DISEASES/DISORDERS
Toxic epidermal necrolysis
Epitheliotropic lymphoma
Lymphoreticular neoplasia
Causes of ulcerative stomatitis
DIAGNOSTICS
CBC/Biochemistry/Urinalysis
Neutrophilia and hyperglobulinemia.
Abnormalities may be related to underlying pathology (i.e., neoplasia producing PP)
or from chronic inflammation or infection.
Baseline values should be obtained prior to initiating immunosuppressive therapy.
Other Laboratory Tests
Antinuclear antibody: may be weakly positive in PE only.
Diagnostic Procedures
Cytology of aspirates or impression smears of pustules or crusts:
Acantholytic keratinocytes, neutrophils and eosinophils
Acantholytic keratinocytes appear as rounded, darkly stained cells with promi-
nent nuclei (“fried egg”) (Figure 12.21)
No acantholytic cells in BP or EBA; rare in PV due to depth of clefting.Bacteriologic culture: identify secondary bacterial infections.
Biopsy of lesions with dermatohistopathologic examination required for diagnosis.
Biopsy samples should be obtained from lesional or perilesional skin.
Pathologic Findings – Pemphigus Complex
PF: acantholytic keratinocytes in crusts; acantholysis and intraepidermal clefting;
microabscess or pustule formation that spans and extends into follicles; acantholytic
keratinocytes may be individual, associated together into “rafts” or adherent to the
overlying epidermis (“cling-ons”); dermal inflammation mixed and perivascular neu-
tophilic and eosinophilic (Figure 12.22).PE: findings similar to PF; lichenoid interface dermatitis with basal cell damage; pig-
mentary incontinence.
PV: suprabasilar clefting with acantholytic keratinocytes; individual keratinocytes
remain attached to the basement zone creating a “tombstone” pattern; secondary
ulceration common; degree of superficial dermal inflammation variable.PEP/Pveg: pustules present throughout the epidermal layers and includes the follic-
ular epithelium.
PP: transepidermal pustulation with suprabasilar and superficial acantholysis; promi-
nent apoptotic keratinocytes; dermal or submucosal infiltrate of lymphocytes,
macrophages, and plasma cells; variable numbers of neutrophils.