CPC III

• 1) Deckchair sign • 9) Sweet Syndrome

( Ofuji's Papuloerythroderma)
• 2) Axillary Granular • 10) Granuloma Fasciale
Parakeratosis • 11) Secondary Syphllis
• 3) Mastocytosis • 12) Hidrocystoma
• 4) PIEZOGENIC PEDAL • 13) Chromomycosis
PAPULES
• 14) Syringocystadenoma
• 5) Scleromyxedema Papilliferum
• 6) PPD • 15) PCT
• 7) PMLE
• 8) CTCL
Case 1
JAAD 2001 Iotaderma (#84)

The “deck chair sign” refers to sparing of the creases in

skin folds in an erythroderma consisting of confluent flat-
topped pink papules and associated with a peripheral
eosinophilia.
What is the eponymic name for the disease in which the
“deck chair sign” occurs?
Answer: Papuloerythroderma of Ofuji

Ofuji S, Furukawa F, Miyachi Y, Ohno S. Papuloerythroderma.

Dermatologica 1984;169:125-30.
Ofuji's Papuloerythroderma
Ofuji's Papuloerythroderma

• Diffuse, papular erythroderma which spares the skin

folds, creating the is characteristic ‘deckchair sign’
• Many of these patients have a peripheral eosinophilia,
and some lymphadenopathy
• Recently thought to be not a single entity but instead a
pattern of expression of various inflammatory
dermatoses, including lymphoma, hypereosinophilic
syndrome, cancers, atopic dermatitis, tinea versicolor,
and drug reactions
• The work-up should include the exclusion of the above-
mentioned entities, especially lymphoma.
Case 2
Case #1: Axilla
Case #1: Axilla
Case #1: Post. Cervical
Case #1 Inguinal Folds
 Axillary Granular Parakeratosis
distinct retention of basophilic keratohyaline granules
within areas of parakeratosis in the stratum corneum
Axillary Granular Parakeratosis
• The primary lesions are brownish-red keratotic papules
that can coalesce into plaques
• It occurs almost exclusively in women
• In most cases, lesions are localized to the axilla, but
other intertriginous sites can be affected
• A defect in processing profilaggrin to filaggrin is a
proposed mechanism
 Pathology
• Psoriasiform
hyperplasia.
• Thickened stratum
corneum with
parakeratosis and
retention of
keratohyaline
granules.
 (Axillary) Granular Parakeratosis
• Benign condition, with hyperpigmented and hyperkeratotic papules
and plaques in the flexural folds
• Uncertain etiology: may be associated with use of topical agents,
e.g. antiperspirants and occlusion
• Mostly affect women from 40-50 years of age, but may affected
children as well.
• May associate with pruritus.
• Effective treatment include topical and oral retinoids,topical
calcipotriene, and topical ammonium lactate.
Case 3
Mastocytosis
• It is a disease in which there is an increased
number of mast cells in various organs of the
body, the most frequent site of organ
involvement is the skin.
• Mast cells contain : histamine (urticaria, Gi
symptoms), prostaglandinD2 (flush, CVS, GI
symptoms), heparin (bleeding into lesion at
biopsy site), proteases, acid hydrolases (patch
hepatic fibrosis and bone lesions).
• Stroking lesion causes it to itch and to wheal
(Darier’s sign).
Triggers that induce systemic mast cell
degranulation:
• Drugs (opiate analgesics, Vancomycin, Aspirin,
NSAIDs, Muscle relaxants)
• Temperature changes : heat, cold.
• Ingestion of alcohol.
• Mechanical irritation : massage, Friction.
• Infections (bacterial, Viral, Ascaris)
• Insect stings, posion (snakes, Jellyfish).
Cuteanous Mastocytosis:
• Solitary mastocytoma
• Urticaria pigmentosa
• Diffuse cutaneous mastoctosis
• Telengectasia Macularis eruptiva Perstans
(TMEP).
• Telangiectases and a very subtle increase in
mast cell number around superficial vessels may
be the only findings
• Mast cells have round, dark nuclei and moderate
surrounding grayish granular cytoplasm resembling "fried
eggs
• Toluidine
blue stain
confirms
the
diagnosis
• Another helpful stain to confirm mast cells is
chloroacetate esterase
• Giemsa or
toluidine blue
stain shows
metachromasi
a and
amphophilic
mast cell
granules,
confirming the
diagnosis of
mast cell
disease
• Electron
microsco
py
shows
characte
ristic
electron-
dense
granules
 Differential Diagnosis

• Mastocytoma : Juvenile xanthogrnuloma,

Spitz nevus

• UP, DCM, TMEP: histiocytosis X,

secondary syphilis, papular sarcoid,
generalized eruptive histiocytosis.
 Management

• Avoidence of drugs that cause mast cell

degranulation
• Antihistamine
• PUVA
Case 4
 Histology
• Marked decrease in the thickness of the dermis
• Normal epidermis
• Abnormal collagen fibers arranged in thin fibers
rather than in bundles
• Subcutaneous fat extends through the dermis
and encroaches on the epidermis in some areas
• Thin collagen fibers : seen bt. the lobules of
subepidermal adipose tissue.
• EM: fine filamentous structures , normal-
appearing collagen fibers
PIEZOGENIC PEDAL
PAPULES
 PIEZOGENIC PEDAL PAPULES

• herniation of fat through the dermis.

• Common
• Non-hereditary
• not the result of an inherent connective
tissue defect
• Rarely found asso. with EDS
• No racial predisposition
• Sex: women ( obesity) > men
• Age: any age
• asymptomatic
• No treatment required
• If the condition is painful, patients may
report limitation of occupational or sporting
activities.
 Features
• Skin color, compressible papule
• Common : lateral heels,bilaterally; volar
wrists
• Examine patients standing with their full
weight on the heels.
• Papules resolve when the weight is
removed
 Causes
• No specific
• believed to be sporadic.
• more common overweight, prople with
orthopedic problems ( flat feet), & may
occur more commonly in persons with
collagen disorders such as EDS.
 Differential Diagnosis
• Nevus lipomatosus superficialis
• EDS
Case 5
 Histopathology of
Scleromyxedema
Typical triad of
- fibrosis
- proliferation of irregularly arranged
fibroblasts
- interstitial deposits of mucin in the upper
and mid-reticular dermis.
: Mucin deposits splay collagen bundles in
the dermis, but there is only slight
fibroblast proliferation and no sclerosis.
Scleromyxedema
 Scleromyxedema
• = Generalized papular mucinosis
• Adults, M=F
• Chronic, progressive, pruritic
• Multiple waxy/shiny papules, coalesce into
plaques
• Dorsal hands, face, elbows, ears, extensor
extremities, leonine facies
• Doughnut sign
• Visceral: GI, pulm., musculoskeletal, CNS
 Differential Diagnosis
• Mucin deposition
• Fibroblast proliferation
• Fibrosis
• Normal thyroid function tests
• Monoclonal gammopathy, usually IgGλ
type
• Bone marrow: N or incr. plasma cells, or
myeloma
 Differential Diagnosis
• Folliculotropic mycosis fungoides
• Scleroderma
• Amyloidosis
• Nephrogenic fibrosing dermopathy
 Treatment and Prognosis
• Physiotherapy
• Systemic steroids
• Retinoids, plasmapheresis, photopheresis
• IVIG, EBT, PUVA, IFN, CyA, IL kenalog
• Melphalan, cyclophosphamide
• Autologous stem cell transplant
• Prognosis poor
Case 6
Pigmented Purpuric Dermatosis
Schamberg's Disease
 Pigmented Purpuric Dermatoses
Synonyms:
Capillaritis; Purpura pigmentosa chronica
Variants:
• Progressive pigmentary dermatosis of Schamberg; Schamberg's
disease

• Purpura annularis telangiectodes of Majocchi; Majocchi's disease

• Pigmented purpuric lichenoid dermatitis of Gougerot and Blum

• Eczematid-like purpura of Doucas and Kapetanakis

• Lichen aureus

UNIFYING KEY FEATURES:

• Clustered petechial hemorrhage
• Often a background of yellow-brown discoloration due to hemosiderin
deposition
Case 7
 Polymorphous light eruption
• Pseudobulla: Marked
papillary edema

• Tight superficial and

deep lymph. infiltrate

• Occasional
dyskeratosis and
exocytosis

• DDx: Pernio (acral

skin with milder edema,
more diffuse infiltrate)
Case 8
Mycosis fungoides/ CTCL
 Mycosis fungoides/ CTCL

Minimal to moderate spongiosis

Mycosis fungoides/ CTCL

• Lymphocytes adhere to
basal layer but do not
obscure it

• Lymphocytes may have

halos, are not perfectly
round and may be slightly
enlarged

• microabscesses may not

be present

• degree of atypia is
variable, may be low
Case 9
Sweets Syndrome: Clinical
Acute Febrile Neutrophilic Dermatosis
• Erythematous plaques; can be
bullous
• Face, neck, upper extremities
• Uncommon; Female > Male 4:1
• Age 30-60 yrs
• Possibly hypersensitivity reaction or
cytokine dysregulation
• Idiopathic, paraneoplastic (20%), IBD/
autoimmune, drugs, and pregnancy
• Associated fever, leukocytosis,
arthritis/arthralgias, ocular,
pulmonary, renal, hepatitis
Sweet’s Syndrome: Histology
• Edema of the papillary dermis,
severe (can form blister)
• Dense neutrophilic infiltrate
• Leukocytoclasia (but no
vasculitis)
• Epidermal hyperplasia
• Epidermal neutrophilic
microabcesses
• Vascular ectasia
Papillary dermal edema
Neutrophils in their natural habitat
 Neutrophils

Perivascular Nodular Diffuse

Dermatopathology Interactive Atlas, Bhawan et al.

Sweet’s Vasculitis

Fibrinoid Necrosis
Case 10
Granuloma Faciale: Clinical

• Red/brown infiltrated plaques

• Face/nose
• Extrafacial sites: trunk,
extremities, scalp
• White males, middle age
Granuloma Faciale: Histology
• Normal epidermis
• Grenz zone
• nodular and/or diffuse
infiltrate in upper dermis
• Eosinophils,
neutrophils; also
lymphs and plasma
cells
• Leukocytoclastic
vasculitis
eosinophil

neutrophil
 Granuloma
Faciale

• LCV
• Neutrophils
predominate

Dermatopathology Interactive Atlas, Bhawan et al.

Case 11
 Secondary Syphilis: Clinical

• Papulosquamous eruption, “copper-colored”

• Annular on face
• non-pruritic
• Acral: symmetric papules with colarrette of scale
• Condyloma lata
• Moth-eaten alopecia
Secondary Syphilis: Histology
• Psoriasiform hyperplasia
• Hyperkeratosis and Parakeratosis
• Spongiosis/Vaculolization at DE junction
 Secondary Syphilis: Infiltrate
Perivascular Nodular Lichenoid
Plasma Cells
• Endothelial swelling with
pseudo-granulomatous
aggregates

• Silver stain (Warthin-

Starry) shows
spirochetes in epidermis
Case 12
Hidrocystoma
 Hidrocystomas

• 1 to 3mm translucent papules,

occasionally with bluish tint

• Solitary-face, scalp

• Tx-excision, laser, atropine, scopolamine.

 Path: Hidrocystoma
• Cyst-may appear empty from fluid running
out in processing.
• Cyst lining gives diagnosis-
– Cuboidal possibly bilayered-eccrine
– Columnar possibly bilayered with decapitated
secretion-apocrine
Case 13
Chromomycosis
 Clinical-chromomycosis
• Usu lower extremities, 4:1 men (farmers)
• Verrucous, slow growing, 15 yrs to
diagnosis
• Dematiacoius fungi-Fonsecaea pedrosoi
most common.
• Tx: excision, cryo, itraconazole,
terbinafine.
 Path: Chromomycosis
• Pseudoepitheliomatous hyperplasia, occ
intraepidermal neutrophilic abscess
• Mixed dermal infiltrate-neuts, lymphs,
histiocytes, plasma, and giant cells.
• Cluster or chains of brown spores-copper
pennies, medlar bodies
Case 14
Syringocystadenoma
Papilliferum
 Syringocystadenoma
Papilliferum-clinical

• Commonly develop within nevus sebaceus

of Jadassohn

• Rose-red papules of firm consistency.

 Path: Syringocystadenoma
Papilliferum
• Papillomatous epidermis connecting to
underlying tumor
• Cystic space in tumor opens to skin.
• Superficial tumor lined by squamous epithelium,
deeper-sweat gland epithelium.
• Apocrine decapitation usually present
• Plasma cell infiltrate
• Nevus sebaceus usually present
Case 15
Porphyria Cutanea Tarda
 Clinical PCT
• Photosensitivity resulting in bullae-sun exposed.
• No erythema surrounding-rupture to ulcers.
• Hyperpigmentation and hypertrichosis is often
seen.
• Associated with liver disease or estrogen
therapy.
• Deficiency in uroporphyrinogen decarboxylase.
• Tx: antimalarials and phlebotomy.
 Path-PCT
• Subepidermal blister with festooning of dermal
papillae.
• Caterpillar bodies-eosinophilic, linear,
segmented, basement membrane material
resembling dyskeratotic cells.
• Sparse hyalizined material around vessels.
• DFI-IgG and C3 around papillary dermal
vessels.