Post on 10-Apr-2018
8/8/2019 CPC Kurban IV
1/131
CPC IV
1) Minocycline Pigmentation
2) Kyrle Disease
3) Molloscum contagiosum
4) Acrokeratoelastoidosis ofCosta
5) Tungiasis
6) Trichomycosis axilaris
7) Pseudocyst of the auricle
8) Subcorneal PustularDermatosis
9) Acrokeratosis Verruciformisof Hopf
10) NecrobioticXanthogranuloma
11) Elastosis PerforansSerpiginosum
12) Angiokeratoma
13) Syringoma
14) Urticaria
15) Poikiloderma
16) Keratosis LichenoidesChronica
17) Hyperkeratosis LenticularisPerstans (Flegels)
18) Infantile myofibromatosis
19) Papular acrodermatitis ofchildhood (Gianotti-Crosti)
8/8/2019 CPC Kurban IV
2/131
Case 1
8/8/2019 CPC Kurban IV
3/131
8/8/2019 CPC Kurban IV
4/131
8/8/2019 CPC Kurban IV
5/131
8/8/2019 CPC Kurban IV
6/131
8/8/2019 CPC Kurban IV
7/131
Minocycline pigmentation
8/8/2019 CPC Kurban IV
8/131
Clinical-minocycline
pigmentation Blue-black discoloration in areas of priorinflammation-acne scars (type I).
Blue-black on shins (type II).
Generalized muddy brown hyperpigmentation,accentuated in sun-exposed areas (type III)-uncommon.
Teeth-grey or grey green on midportion of tooth
(different than tetracycline). Can also affect sclera, ears,bone, thyroid,
nailbed.
8/8/2019 CPC Kurban IV
9/131
Path: Minocycline pigmentation
Brown dermal pigment
Positive with iron and melanin stains
Pigment granules within dermalmacrophages.
8/8/2019 CPC Kurban IV
10/131
8/8/2019 CPC Kurban IV
11/131
8/8/2019 CPC Kurban IV
12/131
Case 2
8/8/2019 CPC Kurban IV
13/131
8/8/2019 CPC Kurban IV
14/131
8/8/2019 CPC Kurban IV
15/131
8/8/2019 CPC Kurban IV
16/131
8/8/2019 CPC Kurban IV
17/131
Kyrle Disease
(Acquired perforatingdermatoses)
8/8/2019 CPC Kurban IV
18/131
Clinical-Kyrle
Associated with renal failure and/or diabetes
4-10% of dialysis patients-usu legs
Variable itchiness.
Felt to be a response to trauma-the scratching in
reponse to pruritis of renal failure.
Tx:PUVA, UVB, Hydration, retinoids, renal
transplantation.
8/8/2019 CPC Kurban IV
19/131
Path-Kyrles
Hyperkeratotic plug, sometimesassociated with follicular orifices.
Parakeratosis and dyskeratosis.
Epidermal hyperplasia
No elastic fibers or collagen fibers withinplug.
Foreign body giant cells in the dermis atperforation site.
8/8/2019 CPC Kurban IV
20/131
8/8/2019 CPC Kurban IV
21/131
8/8/2019 CPC Kurban IV
22/131
Case 3
8/8/2019 CPC Kurban IV
23/131
8/8/2019 CPC Kurban IV
24/131
8/8/2019 CPC Kurban IV
25/131
8/8/2019 CPC Kurban IV
26/131
8/8/2019 CPC Kurban IV
27/131
Molloscumcontagiosum
8/8/2019 CPC Kurban IV
28/131
Case 4
8/8/2019 CPC Kurban IV
29/131
8/8/2019 CPC Kurban IV
30/131
8/8/2019 CPC Kurban IV
31/131
8/8/2019 CPC Kurban IV
32/131
Acrokeratoelastoidosis of Costa
Inheritance
Clinical
Path: Focal acral hyperkeratosis
Acrokeratoelastoidosis of Costa
Treatment
8/8/2019 CPC Kurban IV
33/131
Case 5
8/8/2019 CPC Kurban IV
34/131
8/8/2019 CPC Kurban IV
35/131
8/8/2019 CPC Kurban IV
36/131
8/8/2019 CPC Kurban IV
37/131
8/8/2019 CPC Kurban IV
38/131
8/8/2019 CPC Kurban IV
39/131
8/8/2019 CPC Kurban IV
40/131
Case 6
8/8/2019 CPC Kurban IV
41/131
8/8/2019 CPC Kurban IV
42/131
8/8/2019 CPC Kurban IV
43/131
8/8/2019 CPC Kurban IV
44/131
Trichomycosis axilaris
8/8/2019 CPC Kurban IV
45/131
8/8/2019 CPC Kurban IV
46/131
8/8/2019 CPC Kurban IV
47/131
8/8/2019 CPC Kurban IV
48/131
Path
Cavity within the auricular cartilage (without an epithelial lining) that
contains clear fluid.
Fibrous tissue and granulation tissue may be found in the cavity aswell.
The cartilage lining the cavity may show degenerative changes.
However, no inflammation is seen in the cartilage; this is helpful inexcluding ___________, which is frequently in the clinical differential
diagnosis.
8/8/2019 CPC Kurban IV
49/131
Pseudocyst of the auricle
Endochondral pseudocyst; cystic chondromalacia; intracartilaginous cyst
Clinical
Etiology
Treatment
DDX: Relapsing polychondritis
8/8/2019 CPC Kurban IV
50/131
8/8/2019 CPC Kurban IV
51/131
8/8/2019 CPC Kurban IV
52/131
Case 8
8/8/2019 CPC Kurban IV
53/131
Inframammary Fold
8/8/2019 CPC Kurban IV
54/131
Inframammary Fold
8/8/2019 CPC Kurban IV
55/131
8/8/2019 CPC Kurban IV
56/131
Pathology
search
Subcorneal
vesiculation filled with
neutrophils and
eosinophils
8/8/2019 CPC Kurban IV
57/131
Subcorneal Pustular Dermatosis
(Sneddon-Wilkinson) A benign, chronic-relapsing, sterile pustular eruption, usually
involving flexural sites and proximal extremities
Idiopathic. Some cases are thought to be a variant of pustular
psoriasis. Some cases are associated with IgA pemphigus.
Known association with paraproteinemia, monoclonal gammopathy,and lymphoproliferative disorders, such as multiple myeloma
Usually occur in people >40 years of age
Classically, pt are found to have flaccid bullae on the intertriginous
area, especially axilla, inframammary folds, and groin
Studies: SPEP, bacterial culture (usually negative) Tx: dapsone, acitretin, phototherapy, infliximab
8/8/2019 CPC Kurban IV
58/131
Case 9
8/8/2019 CPC Kurban IV
59/131
8/8/2019 CPC Kurban IV
60/131
8/8/2019 CPC Kurban IV
61/131
8/8/2019 CPC Kurban IV
62/131
8/8/2019 CPC Kurban IV
63/131
8/8/2019 CPC Kurban IV
64/131
Pathology
Hyperkeratosis,
regular acanthosis,
and papillomatosis
Regular undulatingappearance of the
surface
No parakeratosis
Very little
inflammatory infiltrate
8/8/2019 CPC Kurban IV
65/131
Acrokeratosis Verruciformis of Hopf
Genodermatosis characterized by multiple warty lesions foundtypically on the dorsal hands and feet
Autosomal dominant
Clinically very similar to keratosis follicularis of Dariers disease.However, no dyskeratosis on the pathology.
Usually present at birth or since childhood Clinically present as multiple skin-colored to red/brown flat, warty
papules on the dorsal hands and feet.
One family found with mutation in ATP2A2 (possibly an allelicmutation to Dariers)
Tx with superficial ablation, e.g. Nd:YAG laser
8/8/2019 CPC Kurban IV
66/131
Case 10
8/8/2019 CPC Kurban IV
67/131
8/8/2019 CPC Kurban IV
68/131
8/8/2019 CPC Kurban IV
69/131
8/8/2019 CPC Kurban IV
70/131
8/8/2019 CPC Kurban IV
71/131
Pathology
Duffuse predominantly
granulomatous infiltrate of
foamy and multinucleated
histiocytes.
Touton giant cells
Also can see numerous
neutrophils with focal abscess
formation
Necrobiosis in the center with
degradation of collagen.
8/8/2019 CPC Kurban IV
72/131
Necrobiotic Xanthogranuloma
Rare, progressive histiocytosis, with close association withparaproteinemia.
Adult onset, with average in the 6th decade.
May begin as a papule/nodule and slowly enlarge.
Clinically with red/orange or yllow color.
Most common site of involvement is the face, particularly periorbitalregion (85%). Can have ophthalmologic complication in 50% ofcases
May involve extracutaneous sites, e.g. heart, lungs, kidney.
Associate with paraproteinemi. 80% with monoclonal gammopathy
on SPEP. Also, multiple myeloma and Hodgkins lymphoma. Anecdotal treatment with chlorambucil, melphalan, localized
radiotherapy, interferon, and plasmapheresis withhydroxychloroquine.
8/8/2019 CPC Kurban IV
73/131
Case11
8/8/2019 CPC Kurban IV
74/131
Post. Cervical
8/8/2019 CPC Kurban IV
75/131
8/8/2019 CPC Kurban IV
76/131
8/8/2019 CPC Kurban IV
77/131
8/8/2019 CPC Kurban IV
78/131
Pathology
Increased elastic fiber in the
papillary dermis
Elastic fiber can be seen as
clumped and disorganized.
Abnormal elastic fiber, alongwith collagen fiber,
inflammatory cells, and cellular
debris are extruded through
the epidermis
8/8/2019 CPC Kurban IV
79/131
8/8/2019 CPC Kurban IV
80/131
Case12
8/8/2019 CPC Kurban IV
81/131
8/8/2019 CPC Kurban IV
82/131
8/8/2019 CPC Kurban IV
83/131
8/8/2019 CPC Kurban IV
84/131
Angiokeratoma
8/8/2019 CPC Kurban IV
85/131
Case13
8/8/2019 CPC Kurban IV
86/131
8/8/2019 CPC Kurban IV
87/131
8/8/2019 CPC Kurban IV
88/131
8/8/2019 CPC Kurban IV
89/131
Syringoma
8/8/2019 CPC Kurban IV
90/131
8/8/2019 CPC Kurban IV
91/131
Case14
8/8/2019 CPC Kurban IV
92/131
8/8/2019 CPC Kurban IV
93/131
8/8/2019 CPC Kurban IV
94/131
Urticaria
8/8/2019 CPC Kurban IV
95/131
Urticaria
8/8/2019 CPC Kurban IV
96/131
Case15
8/8/2019 CPC Kurban IV
97/131
8/8/2019 CPC Kurban IV
98/131
8/8/2019 CPC Kurban IV
99/131
8/8/2019 CPC Kurban IV
100/131
8/8/2019 CPC Kurban IV
101/131
8/8/2019 CPC Kurban IV
102/131
Case16
8/8/2019 CPC Kurban IV
103/131
8/8/2019 CPC Kurban IV
104/131
8/8/2019 CPC Kurban IV
105/131
8/8/2019 CPC Kurban IV
106/131
8/8/2019 CPC Kurban IV
107/131
P th l
8/8/2019 CPC Kurban IV
108/131
Pathology
Orthokeratotichyerperkeratosis
Irregular acanthosis
Superficial lymphocytic
infiltrate with a feweosinophils
Melanin pigment isobserved
Follicular plugging and
thinning of stratumspinosum is locallyobserved
search
K t i Li h id Ch i
8/8/2019 CPC Kurban IV
109/131
Keratosis Lichenoides Chronica
A rare disorder consists of progressive development ofasymptomatic verrucous papules on the limbs and trunk, with lineardistribution. A small reticulated network can be seen
In 75% of cases, an erythematous eruptions can occur on themediofacial area.
Pt may have ulceration, infiltration, and inflammation of the mucous
membrane. Histologically may mimick hypertrophic lichen planus. However,
KLC has linear/reticulated pattern and pruritus is usually absent.
Several reports associate KLC with visceral pathology, such ashepatitis, chronic lymphoid leukemia, or nephropathy.
Topical therapy is ineffective. So far, some cases report
improvement with oral etretinate or isotretinoin, PUVA therapy, orphotodynamic therapy.
8/8/2019 CPC Kurban IV
110/131
Case17
8/8/2019 CPC Kurban IV
111/131
8/8/2019 CPC Kurban IV
112/131
8/8/2019 CPC Kurban IV
113/131
8/8/2019 CPC Kurban IV
114/131
Pathology
8/8/2019 CPC Kurban IV
115/131
Pathology
Abrupt and discreteareas of compactlamellarhyerperkeratosis
Epidermal atrophy
Band-like infiltrate
Hyperkeratosis Lenticularis
8/8/2019 CPC Kurban IV
116/131
yp
Perstans (Flegels) Originally described as red-brown papules with scales of irregular
outlines measuring 1-5mm in idameter and up to 1mm in depth.
Lesions are primarily located on dorsal feet and lower legs.
Unclear etiology
Ulstrastructurally, there is a loss or decrease of membrane-coatinggranules (Odland bodies), resulting in decreased desquamation ofthe stratum corneum, with subsequent retention hyperkeratosis.
Treatment with Calcipotriol is reported to be effective.
Other successful treatments include topical fluorouracil cream, localexcision, and dermabrasion.
8/8/2019 CPC Kurban IV
117/131
Case18
8/8/2019 CPC Kurban IV
118/131
8/8/2019 CPC Kurban IV
119/131
8/8/2019 CPC Kurban IV
120/131
8/8/2019 CPC Kurban IV
121/131
8/8/2019 CPC Kurban IV
122/131
Pathology
8/8/2019 CPC Kurban IV
123/131
Pathology
Path shows whorls and fascicles of smooth muscle and
spindle-shaped myofibroblastic cells in a fibrous stroma.
The leison is surrounded by vascular channels, which is
characteristic of these tumors, resembling
hemangiopericytoma.
A trichome stain confirms the presence of smooth muscle.
Infantile myofibromatosis
8/8/2019 CPC Kurban IV
124/131
Infantile myofibromatosis
Benigh fibrous spindle cell tumor This is the most common juvenile fibrous tumor, classified into three forms:
Solitary (>50%)
Multicentric (
8/8/2019 CPC Kurban IV
125/131
Case19
8/8/2019 CPC Kurban IV
126/131
8/8/2019 CPC Kurban IV
127/131
8/8/2019 CPC Kurban IV
128/131
8/8/2019 CPC Kurban IV
129/131
8/8/2019 CPC Kurban IV
130/131
Papular acrodermatitis of childhood
8/8/2019 CPC Kurban IV
131/131
(Gianotti-Crosti) A self-limited childhood exanthem that manifests characteristically in an
acral distribution
In the US, most commonly associated with EBV. The original report waswith HBV.
Affect mostly children from 3months to 15 years of age, with average at 2years of age
In children, both genders are equally affected. However, in adults, reportedcases have been mostly women.
Monomorphous skin-colored papules or papulo-vesicles localizedsymmetrically and acrally over the extensor surfaces of extremities,buttocks and face.
Eruption lasts from 10 days to 6 weeks. Complete resolution often takes
more than 2 months. May have associated constitutional symptoms such as fever,
lymphadenopathy.