Classic Example

Congenital smooth muscle hamartoma

A one-year-old Japanese boy presented with brown macules on the waist.

 

Diagnosis: 
Congenital smooth muscle hamartoma
Comment: 

This lesion showed a positive pseudo-Darier sign (which is a transient piloerection and elevation of a lesion induced by rubbing and is observed especially in congenital smooth muscle hamartomas)

Immunohistochemical staining for α-SMA was positive in the tumor cells.

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Right arm lesion, 53-year-old woman

Contributor(s): 

53-year-old woman.  Biopsy from right arm.  No clinical history provided.

Diagnosis: 
Plexiform schwannoma
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54 year-old HIV-positive male with upper back thickenning.

Contributor(s): 

Biopsy sumbitted to rule out "lipodystrophy, necrobiosis and acanthosis".

Diagnosis: 
Scleredema
Comment: 

pending

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Dermpedia textbook chapter: 

Inverted follicular keratosis

Contributor(s): 

45 year old female with suspected squamous cell carcinoma on the tip of the nose.

Diagnosis: 
Inverted follicular keratosis
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Right cheek lesion, 12-year-old girl

Contributor(s): 

Right cheek lesion in a 12-year-old girl.

Diagnosis: 
Dermal spindled and epithelioid cell (Spitz) nevus with moderate atypia
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Dermpedia textbook chapter: 

11-year-old girl with brown-tan papules on her back and chest

Contributor(s): 

11-year-old girl with brown-tan papules on her back and chest.

Part A (images 1-4) is from the upper back.  Part B (images 5-8) is from the upper chest.

Diagnosis: 
Halo nevi
Comment: 

Halo nevi are so called because they clinically surrounded surrounded by a white halo.  They tend to occur in children and younger adults and, as in this case, are most common on the trunk.

Histologically, one sees a dense lichenoid infiltrate of lymphocytes and histiocytes, which may obscure the nevus cell nests, potentially causing them to be missed if they are not carefully sought.  Immunohistochemical stains may aid in their detection.

Halo nevi may be junctional, compound or dermal.

 

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Pilomatrixoma

85 year old male with an excision of the posterior scalp.  Gross examination included a well circumscribed, cystic structure containing yellow-red grainy material.

Diagnosis: 
Pilomatrixoma
Comment: 

Pilomatrixoma presents as a slow growing, firm to hard nodule (0.5- 3 cm) appearing, in decreasing frequency, on the head (cheek most common), upper limbs, neck, trunk, lower limbs.

Its pathogenesis is associated with the Wnt/wingless signal transduction pathway and mutated B-catenin protein, which plays a structural role in cell-cell formation between adherens junctions and the actin cytoskeleton.

There have been rare reports of pilomatrix carcinoma arising out of benign pilomatrixomas, and sites treated for pilomatricoma.

Who is affected: young (<20 years old, many before10 years old) with second peak in 6-7th decades of life.

Treatment: surgery is curative. Local recurrence in 2-3% of cases.

Microscopic features: 

- Lobules of basaloid and ghost cells (biphasic cell population

- Early lesions show brisk mitoses (indicating rapid growth and not a malignancy) and sheets of germinative basaloid cells with uniform nuclei and small nucleoli.

- As cells mature, they become larger with abundant eosinophilic cytoplasm, and pyknotic nuclei

- Melanin pigment in basaloid and tumor cells

- Foreign body giant cell reaction common

- Calcification, granular and basophilic, may be seen

- Atypical features in the elderly such as basloid cell pleomorphism, loss of polarity, nuclear hyperchromatism, and marked mitotic activity.

Differential diagnosis: infundibular cysts, trichoepithelioma.

Alopecia Case #1 for discussion at Scottsdale 2010 Event

Contributor(s): 

6 year old AA girl with 1 month history of hair loss. Areas on scalp that are shiny, smooth and without hair Few scattered scaly papules with few excoriated papules, pruritis.

Diagnosis: 
Pending
Comment: 

2 punch biopsies were performed. Both vertical and horizontal sections were evaluated.

70 year old woman with nose lesion

70 year old woman with punch biopsy of left lateral nose with question of blue nevus vs. pigmented basal cell carcinoma vs. melanoma.

Diagnosis: 
Venous lake
Comment: 

Venous lakes are fairly common vascular ectasias presenting on sun damaged skin of the elderly, showing a predilection for the lip.

Lesions may be multiple and measure up to 1 cm in diameter.

The pathogenesis may be due to possible defective stromal support.

 

Microscopic features: dilated and congested veins in the superficial dermis.  No evidence of vascular proliferation.  

68 year old male with suspected psoriasis: a surprise!

Left thigh biopsy of a 68 year old man with suspected psoriasis versus hypersensitivity reaction

Diagnosis: 
Scabies
Comment: 

Scabies is a highly contagious dermatogical condition caused by the mite, Sarcoptes scabiei. The female mite burrows into the epidermis to lay eggs. Larvae hatch in 3-5 days, mature about 2 weeks later, and then migrate to the surface and mate to continue the cycle. Papules and burrows are often found in the webbing between the fingers and toes, or the sides of fingers.

The host immunologic response (immune complex mediated)  to the mite or its feces (seen microscopically as scybala when hardened) produces the intense itching experienced by patients. Secondary infection is common. 

Scabies transmitted by prolonged skin to skin contact contact.

Who is affected: those living in poor socioeconomic conditions, overcrowded areas (eg nursing homes, jails), and have poor personal hygiene. Scabies can be transmitted during sexual contact.

Microscopic features: acanthosis, hyperkeratosis, and often spongiosis with lymphocytic infiltrate.  The mite is usually subcorneal, at the junction between the epidermis and stratum corneum.

Diagnosis: is made by visualizing the mite, mite parts or its feces.

Treatment of choice: topical permethrin cream.

Prevention measures: good personal hygiene; machine wash infected laundry with hot water; seal infected objects (toys) in a plastic bag for 3 days.

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Another case masquerading as a pilomatix carcinoma

None as yet.  Case discovered on review of appendage tumor collection.  Clinical information to be added if and when it becomes available

Diagnosis: 
Melanocytic matricoma
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Congenital nevus with proliferative nodules

Contributor(s): 
Diagnosis: 
Congenital nevus with proliferative nodules
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Early invasive melanoma

Contributor(s): 
Diagnosis: 
Early invasive melanoma
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15-year-old with "suspicious conjunctival lesion"

Contributor(s): 

15-year-old boy with a pigmented lesion on the conjunctiva of his left eye.

Diagnosis: 
Conjunctival melanocytic nevus
Comment: 

Key features include the small cells of the melanocytes and their nuclei (similar in appearance to the overlying conjunctival epithelial cells) and the highly characteristic induction of cysts formed by the conjunctival epithelial cells.  The S-100 stain confirms the melanocytic nature of the lesion.

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51-year-old female with a recurrent blistering eruption involving the hands and feet

51-year-old female. The clinical information provided on the requisition was one of a recurrent blistering eruption of undetermined etiology involving the hands and feet.

Diagnosis: 
Pending
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Trichofolliculoma with sebaceous elements

Contributor(s): 
Diagnosis: 
Trichofolliculoma with sebaceous elements
Comment: 

Trichofolliculoma is a rare pilar tumor which typically presents on the head or neck.  Histologically, it shows one to several dilated hair follicles usually containing keratin, from which numerous small follicles branch off.

As in this case, focal areas of sebaceous differentiation may be seen within the follicles (the vacuolated cells seen at high power).  Sebaceous elements are more common in late-stage trichofolliculomas.  A variant termed "sebaceous trichofolliculoma" has also been described.  This designation should be reserved for cases in which large sebaceous follicles connect to a central cavity.

Atypical acral nevus

Contributor(s): 

44-year-old woman with an atypical pigmented lesion on toe.

Diagnosis: 
Atypical acral nevus
Comment: 

Nevi of acral skin (aka MANIAC: melanocytic nevus with intraepidermal ascent of cells) tend to be small macular or slightly elevated lesions.  Histologically, they are junctional nevi whose distinguishing feature is their characteristic upward migration of melanocytes into the epidermis.

This case displays neoplastic melanocytes extending upward into the stratum corneum.  The relatviely severe melanocytic atypia and irregularity of the nests warrant a diagnosis of atypical acral nevus.  The degree of atypia, however, falls short of that required for a diagnosis of acral lentiginous melanoma, the principal entity that must be excluded.  Additional features which are characteristic of acral melanoma are contiguous basilar proliferation and more pronounced pegetoid spread.

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Pretibial myxedema

60 year old female with biopsy of skin of right pretibia. Clinical question of panniculiltis vs. deep granuluma annulare vs B-cell lymphoma.

Diagnosis: 
DERMAL MUCINOSIS CONSISTENT WITH MYXEDEMA
Comment: 

Pretibial myxedema is an infiltrative dermopathy and rare complication of Graves disease (incidence 1-4% of patients).

Clinical symptoms: 

  • limited to anterior aspect of lower extremities and dorsa of feet, rarely other location
  • waxy (pink, yellow) induration of the skin or non pitting edema
  • orange peel (peau d'orange) skin, sometimed nodules

Biopsy reveals mucin deposition in the mid to lower dermis, confirmed by Alcian blue and collagen stains. There is no increase in fibroblasts.

Case 10

62 year-old female with a slowly growing eyelid nodule

Diagnosis: 
Pending
Comment: 

This is a classic example of this entity.

It is a precursor of mucinous carcinoma of the eyelid. 

 

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Kaposi sarcoma, early patch stage

44-year-old man with HIV.  Left elbow lesion clinically suspicious for Kaposi sarcoma.

Diagnosis: 
Kaposi sarcoma, early patch stage
Comment: 

This is a very early patch stage lesion. Key diagnostic features include:

  • dissecting capillaries
  • inflammation with plasma cells
  • focal red cell extravasation of blood cell with hemosiderin deposition

 

 

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