Dermatopathology Cases

Answer of Dermatopathology Case 114

2012-01-23T22:23:00.000-08:00

Deep Penetrating Nevus

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Abstract:

Deep penetrating nevus: a review.Arch Pathol Lab Med. 2011 Mar;135(3):321-6.
CONTEXT:
Deep penetrating nevus is a distinctive melanocytic lesion that may simulate melanoma both clinically and histologically.
OBJECTIVE:
To review clinical and histologic features of deep penetrating nevi and discuss their differential diagnosis, especially regarding melanoma.
DATA SOURCES:
The literature on deep penetrating nevi is reviewed and supplemented by our experiences with deep penetrating nevi.
CONCLUSIONS:
One or more disturbing histologic features may frequently be found in deep penetrating nevi, including asymmetry, plump but fairly regular nests of melanocytes in the dermis, cytologic atypia with some nuclear pleomorphism, a small to medium-sized eosinophilic nucleolus, absence of maturation, occasional presence of normal dermal mitoses, and a patchy mononuclear inflammatory cell infiltrate. Although unusual, such histologic features should not be regarded as a sign of malignancy in deep penetrating nevi.

Pigmented lesion pathology: what you should expect from your pathologist, and what your pathologist should expect from you.Clin Plast Surg. 2010 Jan;37(1):1-20.
The first part of this review examines the reliability of histologic diagnosis in pigmented lesions, as measured by concordance studies and medicolegal analysis. It emphasizes the role of clinicians in maximizing that reliability, by providing adequate clinical descriptions, using appropriate biopsy technique, and critically interpreting pathology reports. It identifies those entities that are especially problematic, either because they cannot be reliably recognized by the histopathologist or because their histology is a poor guide to their biologic behavior. The second part of the review is a guide to some of the more difficult and controversial pigmented lesions, including dysplastic nevus, spitzoid nevi and melanomas, cellular blue nevus, animal-type melanoma, and deep penetrating nevus.

Dermatopathology Case 114

2012-01-23T21:36:00.000-08:00

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Case 114

A dark pigmented papule on the neck of a 25 year old female.

Diagnosis

Answer of Dermatopathology Case 113

2012-01-23T03:03:00.000-08:00

Extramammary Paget's Disease

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Abstract:

Extramammary Paget's disease: Evaluation of the histopathological patterns of Paget cell proliferation in the epidermis.J Dermatol. 2011 Nov;38(11):1054-7. doi: 10.1111/j.1346-8138.2011.01268.x. Epub 2011 Sep 23.
Extramammary Paget's disease (EMPD) is a rare malignant skin neoplasm. The prototypical pattern of tumor cell proliferation in the epidermis includes single cells and/or nest arrangements, mainly in the lower epidermis. Although other patterns have been recognized, they have not previously been investigated in detail. We aimed to examine the patterns of tumor cell proliferation in the epidermis. Surgical specimens were obtained from 38 patients with primary EMPD. We defined six patterns, in addition to the prototypical one: (i) glandular; (ii) acantholysis-like; (iii) upper nest; (iv) tall nest; (v) budding; and (vi) sheet-like. There were 26 males and 12 females (mean age, 75.0 years). Lesions were located on the scrotum (26 cases) and vulva (12). There were 22 in situ EMPD and 16 invasive EMPD. The frequencies of the different proliferation patterns were: glandular, 36.8%; acantholysis-like, 73.7%; upper nest, 68.4%; tall nest, 28.9%; budding, 47.4%; and sheet-like, 23.7%. Upper nest pattern and the presence of more than three patterns were significantly more frequent in invasive EMPD than in situ EMPD (P < 0.05). We identified the histopathological patterns of Paget cell proliferation in the epidermis in EMPD, and suggest that the characteristic patterns and the diversity of patterns could be associated with progression and dermal invasion in EMPD.

Primary Extramammary Paget's Disease Combined with Bowen's Disease in Vulva.Ann Dermatol. 2011 Oct;23(Suppl 2):S222-5. Epub 2011 Oct 31.
Extramammary Paget's disease (EMPD) is a uncommon neoplastic condition of apocrine gland-bearing skin and its occurrence in combination with Bowen's disease is very rare. The most common site of involvement is the vulva, although perineal, perianal, scrotal and penile skin may also be affected. EMPD is usually not combined with Bowen's disease. We report an interesting case of EMPD combined with Bowen's disease, which was confirmed by immunohistochemical stain.

Paget's disease of the vulva: Diagnosis and follow-up key to management; a retrospective study of 50 cases from Queensland.Gynecol Oncol. 2011 Jul;122(1):42-4. Epub 2011 Apr 17.
OBJECTIVE:
To review the clinical features, diagnosis, management, and outcomes for the 50 cases of Paget's disease (PD) of the vulva referred to Queensland Centre for Gynaecological Cancer between 1986 and 2009.
METHODS:
Vulvar PD cases from QCGC were reviewed and analyzed using the computer software Statistical Package for the Social Sciences (SPSS) 11.0.
RESULTS:
Paget's disease (PD) of the vulva is uncommon. Of the 50 patients, 2 have died of their PD, 1 patient that had coexisting PD died of squamous cell vulva cancer, and 11 died of unrelated causes. The mean age at diagnosis was 67.6 years (range, 31 to 91). All cases were Caucasian. Time from onset of symptoms to diagnosis averaged 21 months. Not until a biopsy was performed was the diagnosis made. The most common presenting complaint was pruritis (27 cases, 54%). There was no identifiable "favored" site on the vulva for PD. Positive groin lymph nodes were found in 4 of the 10 cases who underwent node biopsy. Two who had poorly differentiated carcinoma in the nodes and PD died of disease within a year of diagnosis, one is alive three years later. The fourth case had coincidental PD and vulvar squamous cell carcinoma with squamous carcinoma groin nodes. Initial treatment was surgical.
CONCLUSIONS:
The prognosis for primary extra-mammary PD of the vulva confined to the epidermis (IEP) is excellent. Early diagnosis and long term follow-up are the keys to successful management. The status of disease at the margins of surgical specimens does not reliably equate to patient long term outcomes.

Dermatopathology Case 113

2012-01-23T02:30:00.000-08:00

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Case 113

A 54 year old woman with a thickened plaque on the vulva. The lesion is crusted with scale covering its surface.

Diagnosis

Answer of Dermatopathology Case 112

2012-01-12T07:58:00.000-08:00

Pleomorphic Fibroma

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Abstract:

Pleomorphic fibroma of the skin: a benign neoplasm with cytologic atypia. A clinicopathologic study of eight cases.Am J Surg Pathol. 1989 Feb;13(2):107-13.
A clinicopathologic study of eight examples of polypoid and dome-shaped cutaneous fibrous lesions with sparse cellularity but striking nuclear atypia and rare mitotic figures is presented. Positive immunohistochemical staining for vimentin and actin supported the fibroblastic nature of these lesions. All eight cases were adults whose ages ranged from 33 to 67 years (mean 52 years). Five were women and three were men. Five lesions were located on extremities, two on the trunk, and one on the face and they measured from 4 to 16 mm in greatest dimension. The lesions were clinically followed from 4 months to 5 years. They all showed benign clinical behavior, with only one local recurrence in a lesion that had been incompletely removed. The nuclear atypia seen in these fibrous lesions may be similar to that which occurs in other benign mesenchymal neoplasms, such as pleomorphic lipoma, pleomorphic leiomyoma, ancient schwannoma, and variants of dermatofibroma with atypical cells. We suggest that "pleomorphic fibroma" is an appropriate term for this lesion based on its histologic differentiation, cytologic atypia, and benign clinical course.

Pleomorphic fibroma of the skin, a form of sclerotic fibroma: an immunohistochemical study.Clin Exp Dermatol. 1998 Jan;23(1):22-4.
We report a new case of pleomorphic fibroma of the skin arising on the face. The lesion was polypoid and fibrous, located on the dermis, and formed by coarse collagen bundles with sparse cells. It was also characterized by the presence of cellular atypia and pleomorphism without mitosis. Immunohistochemistry showed staining for vimentin and actin and negativity for S-100, CD34, CD68, alpha-1-antichymotrypsin and alpha-1-antitrypsin antigens, supporting a myofibroblastic origin. A few isolated cells also showed granular cytoplasmic detection of factor XIIIa. Although the disorder has been previously considered to be a tumour of preferential localization on the trunk or extremities, this does not always hold true as four of the 14 reported cases arose on the head. Pleomorphic fibroma is a benign condition and is we believe a variant of sclerotic fibroma.

Dermatopathology Case 112

2012-01-12T07:42:00.000-08:00

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Case 112

A 51 year old female with a polypoid cutaneous lesion on the left extremity.

Diagnosis

Answer of Dermatopathology Case 111

2011-12-18T02:36:00.000-08:00

Darier's Disease (Keratosis Follicularis)

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Abstract

Darier disease: case report with oral manifestations.Med Oral Patol Oral Cir Bucal. 2006 Aug 1;11(5):E404-6.
Darier disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare autosomal dominant genodermatosis. It is clinically manifested by hyperkeratotic papules primarily affecting seborrheic areas on the head, neck and thorax, with less frequent involvement of the oral mucosa. When oral manifestations are present, they primarily affect the palatal and alveolar mucosa, are usually asymptomatic, and are discovered in routine dental examination.
Histologically, the lesions present suprabasal clefts in the epithelium with acantholytic and dyskeratotic cells represented by corps ronds and corps grains. This paper reports a case of an adult male patient presenting clinical signs of Darier disease in the palatal mucosa and skin on the neck and upper limbs. Intraoral biopsy of the affected area, analysis of family history and evaluation by a multidisciplinary team led to the diagnosis of Darier disease.
Dental professionals and pathologists should be aware of these lesions to allow correct diagnosis and proper management of this disease.

Comedonal, cornifying and hypertrophic Darier's disease in the same patient: a Darier combination.J Dermatol. 2006 Jul;33(7):477-80.
Our 42-year-old patient had comedonal Darier's disease (DD) on the face, comedonal cornifying DD on the upper back, and hypertrophic DD on both legs.
Biopsies taken from face, upper back and medial sides of the legs were found to be compatible with these clinical subtypes. The comedonal type was in the classical place, but the hypertrophic and cornifying types were not in the usual sites.
In addition to the classic histopathology of DD, we noted multiple, warty dyskeratoma-like structures in the comedonal type, marked compact hyperkeratosis in the cornifying type, and marked papillomatosis in the hypertrophic type.

Dermatopathology Case 111

2011-12-18T02:02:00.000-08:00

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Case 111

A 16 year old boy with yellowish brown,warty papules on the forehead. Small pits are noted on the palms and soles of the feet. Red and white bands are noted in the nails along with V-shaped nick at the free margin of the nail.

Diagnosis

Answer of Dermatopathology Case 110

2011-12-17T23:01:00.000-08:00

Dermatofibroma with Monster Cells

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Abstracts:

Giant dermatofibroma with monster cells.Am J Dermatopathol. 2002 Feb;24(1):36-8.
We report a case of a 64-year-old woman with a giant dermatofibroma on her back with the unusual histologic feature of monster cells. The firm, exophytic, 3-cm nodule had purple and yellow components with surface telangiectasia. Histologic examination demonstrated characteristic findings of a dermatofibroma, including rete ridge flattening and bridging; a stroma containing scattered, large, round, eosinophilic collagen bundles; and a polymorphous dermal infiltrate of spindle and xanthomatous cells with scattered siderophages. Some xanthomatous cells demonstrated features consistent with monster cells, including huge bizarre nuclei and one or more nucleoli. Immunohistochemical staining for factor XIIIa was positive. A diagnosis of giant dermatofibroma with monster cells (DFMC) was made. Giant dermatofibromas are rare, with monster cells being an uncommon finding in dermatofibroma. To our knowledge, this is the first report of DFMC.

Case of dermatofibroma with monster cells: a review and an immunohistochemical study.Am J Dermatopathol. 1997 Jun;19(3):312-5.
We report a dermatofibroma with monster cells. The patient was a 79-year-old woman who had a dark-brown nodule of her left leg for approximately 3 years. The lesion was composed of spindle-shaped fibroblastic cells, histiocytic cells, and multinucleated giant cells. Most of the histiocytic cells had foamy cytoplasm with numerous hemosiderin deposits. In addition to these cells, bizarre multinucleated cells with markedly hyperchromatic nuclei and xanthomatous cells with very large nuclei (monster cells) were also noted. No mitotic figures of the cellular components were present. This lesion has been shown to be completely benign despite the presence of pleomorphic or bizarre cells. From a clinical standpoint, recognition of a benign lesion of this type is very important since an incorrect histologic interpretation could result in inappropriate treatment.

Dermatofibroma with monster cells.Am J Dermatopathol. 1987 Oct;9(5):380-7.
Nineteen cases of dermatofibroma associated with monster cells are reported. The term "monster" (an animal with a strange or terrifying shape, one unusually large for its kind) implies a strikingly atypical cell with an extremely large nucleus. Except for monster cells, these 19 lesions had all of the typical histopathological findings of dermatofibroma. The clinical diagnosis for 16 of these lesions was dermatofibroma (or histiocytoma). Three lesions were submitted without any clinical diagnosis. Eighteen of 19 lesions occurred on the extremities. One was on the back. Monster cells are seen in the early, histiocytic stage of dermatofibroma when lipophages and/or siderophages are usually present in large numbers. Only rarely were mitotic figures seen in dermatofibromas with monster cells, and they were neither numerous nor atypical. It is important for histopathologists to distinguish dermatofibroma with monster cells from cutaneous malignant fibrous histiocytoma and radiation sarcoma. The criteria for differentiation concern primarily the architectural pattern of the lesion rather than its cytological features.

Dermatopathology Case 110

2011-12-17T22:50:00.001-08:00

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Case 110

A 55 year old female with a brownish firm nodule on the back. The nodule is 2.5 cm in diameter.

Diagnosis

Answer of Dermatopathology Case 109

2011-12-03T11:46:00.000-08:00

HMB-45 : Positive

Balloon Cell Melanoma

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Dermatopathology Case 109

2011-12-03T11:37:00.000-08:00

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Case 109

A 72 year old male with a skin lesion on the forehead.

Diagnosis

Answer of Dermatopathology Case 108

2011-11-30T08:04:00.000-08:00

Median Raphe Cyst

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Abstract:
Median raphe cyst of the penis.Dermatol Online J. 2005 Dec 1;11(3):37.
Cysts of the median raphe are uncommon. We describe a 43-year-old man with an asymptomatic nodule on the glans penis. Excision of the lesion was performed followed by histological and immunohistochemical studies. Histopathological examination revealed a solitary unilocular cystic cavity in the corium, lined by a pseudostratified columnar epithelium, among which mucinous cells where identified. Hints of decapitation secretion occurred at the apex of the luminal layer. An immunohistochemical study using a standard avidin-biotin peroxidase method disclosed CK7 and CK13 reactivity in the columnar cells. Epithelial membrane antigen (EMA) and carcinoembryonic (CEA) antigen immunoreactivity occurred at the apical border of the luminal cells. No staining was obtained with anti-CK20, human milk fat globulin 1 (HMFG1) and anti- S100 protein. The study supports its histogenetic relationship with the urothelium.

Dermatopathology Case 108

2011-11-30T07:57:00.000-08:00

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Case 108

A 38 year old male with a nodule on the glans penis.

Diagnosis

Answer of Dermatopathology Case 107

2011-11-29T21:36:00.000-08:00

Naevoid Melanoma

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In general, common naevi are diagnosable at low power.
It is important to exclude the possibility of naevoid melanoma.
This is a rare variant that mimics benign naevi and is difficult to recognise.
The correct diagnosis is frequently made only after the patient has developed a metastasis.
At low power, naevoid melanoma may have a nodular architecture, and exhibit other features of a common banal naevus, including circumscription and at least relative symmetry.
Naevoid melanoma lacks the prominent junctional activity and pagetoid spread usually associated with superficial spreading melanoma.
Common intradermal naevus “matures” (there is an overall decrease in nest size and cellular and nuclear size with depth).
On low power examination, naevoid melanoma may appear to mature with depth, yet closer inspection reveals that the cells at the base of the lesions are similar in size to those of the superficial dermal component.
Only at higher power the distinguishing characteristics of naevoid melanoma can be appreciated.
These features include :
1) A monotonous population of small round cells with prominent nucleoli.
2) From a few to numerous mitoses any where in the lesion.
3) Other features that may be present include individual cell necrosis and atypical mitoses.

Dermatopathology Case 107

2011-11-29T21:13:00.000-08:00

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Case 107

A 45 year old female with a pigmented lesion on the left side of the chest.
Diagnosis

Answer of Dermatopathology Case 106

2011-11-28T05:32:00.000-08:00

Desmoplastic Spitz Naevus

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Abstract:

Desmoplastic Spitz nevus: a histopathological review and comparison with desmoplastic melanoma. Ann Dermatol Venereol. 2009 Oct;136(10):689-95.
INTRODUCTION: Desmoplastic Spitz nevus is a rare variant of spitzoid tumours characterized by dermal proliferation of large epitheliod and/or fusiform melanocytes within a desmoplastic stroma, comprising thick, eosinophilic collagen bundles. It occurs most frequently in young adults and children, and exhibits a predilection for the limbs. Histologically, this entity may be mistaken for cutaneous fibrous tumours or desmoplastic melanoma. To establish useful histological criteria for differential diagnosis of desmoplastic Spitz nevus, we conducted a retrospective study comparing data concerning the clinical, demographic and histological characteristics of desmoplastic Spitz nevus and desmoplastic melanoma.
PATIENTS AND METHODS: We reviewed the slides for 28 cases of desmoplastic Spitz nevus and for 13 cases of desmoplastic melanoma obtained from the files of Pinkus Dermatopathology Laboratory (Monroe, MI, USA) during the period 1993-2005.
RESULTS: Desmoplastic Spitz nevus was more common in women (sex ratio M/F=0.4) and younger patients (mean age: 28.2 years) whereas the mean age of patients with desmoplastic melanoma was significantly higher (mean age: 75 years). From a histopathological point of view, symmetry, circumscription, melanocytic maturation and involvement of adnexal structure were significantly more frequent in desmoplastic Spitz nevi. The presence of melanocytic junctional nests associated with discohesive cells, variations in size and shape of the nests, lentiginous melanocytic proliferation, actinic elastosis, pagetoid spread, dermal mitosis, perineural involvement and brisk inflammatory infiltrate were significantly more frequent in desmoplastic melanoma. No significant difference was found concerning epidermal hyperplasia, presence of Kamino bodies or moderate inflammatory infiltrate.
CONCLUSION: The combination of architectural and cytological features was useful in differentiating desmoplastic Spitz nevus from desmoplastic melanoma. A complete excision biopsy is mandatory in such tumors as the architectural criteria are necessary for a correct diagnosis.

Dermatopathology Case 106

2011-11-28T05:22:00.001-08:00

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Case 106

A 28 year old female with a reddish brown nodule on the left leg.
Diagnosis

Answer of Dermatopathology Case 105

2011-11-27T05:25:00.000-08:00

Immunostaining, staining reveals that the epithelial cells are positive for cytokeratin , estrogen receptor (ER) and progesterone receptor (PR).

PR (Progesterone Receptor):Positive

ER (Estrogen Receptor): Positive

Cytokeratin: Positive

Diagnosis: Cutaneous Ciliated Cyst

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Abstract:

Cutaneous ciliated cyst of the right lower leg. Pathol Int. 1999 Apr;49(4):354-7.
A 23-year-old Japanese woman with a cutaneous ciliated cyst on her right lower leg is reported. A subcutaneous cyst, measuring 2.5 cm in diameter with papillary projections into the lumen, was lined with ciliated cuboidal to columnar epithelia with partial stratification, histologically. These lining cells did not produce mucin. Immunohistochemically, the ciliated lining cells of the cyst were diffusely positive to epithelial membrane antigen and cytokeratin. In addition, positive immunoreaction with anti-desmin monoclonal antibody was observed in the body of the cilia. Less than 10% of the epithelial cells revealed positive immunoreaction to S-100 protein and estrogen receptor.

Dermatopathology Case 105

2011-11-27T05:08:00.000-08:00

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Case 105
A 38 year old female with a cystic lesion on the lower part of the left leg. The lesion is 2.5cm in diameter.

Diagnosis

Answer of Dermatopathology Case 104

2011-11-26T03:05:00.000-08:00

Allergic Granulomatosis (Churg-Strauss Syndrome)

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The three major microscopic features of Allergic Granulomatosis are:
1) Necrotizing vasculitis
2) Tissue infiltration by eosinophils
3) Extravascular granulomas
It should be noted that the 3 features mentioned above do not always coexist.

In Case No 104 there is small-vessel necrotizing vasculitis together with extensive tissue infiltration by eosinophils. No granulomas were present in the multiple sections taken. Hence in this case detailed clinical history played a very important role to reach the final diagnosis.

Dermatopathology Case 104

2011-11-26T02:34:00.000-08:00

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Case 104

A 42 year old female with history of asthma and allergic rhinitis. There is marked peripheral blood eosinophilia. Erythemous macules are present on the extremities. Biopsy from the skin lesion.

Diagnosis

Answer of Dermatopathology Case 103

2011-11-24T09:13:00.000-08:00

Infantile Hemangioma

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Abstract:

Comparative study on pathology of noninvoluting congenital hemangioma and infantile hemangioma.Zhonghua Zheng Xing Wai Ke Za Zhi. 2011 May;27(3):178-81

OBJECTIVE: To distinguish noninvoluting congenital hemangioma (NICH) and infantile hemangioma (IH) by comparing the pathological structure and marker antigen expression.
METHODS: From Jan. 2005 to Aug. 2010, 39 paraffin-embedded samples, including 13 cases of NICH, 13 cases of proliferating IH and 13 cases of involuting IH, were collected from operation. Hematoxylin-eosin staining was used to observe the pathological structure. Immunohistochemical analysis was also performed to investigate the expression of Glut-1.
RESULTS: The lobules of capillaries were well-defined in NICH. The lobules were surrounded by abundant fibrous tissue. The capillaries were often large and integrity in NICH. There were few mitosis and apoptosis in endothelial cells and stromal cells in NICH. While in IH, the pathologic findings were totally different. Immunochemistry revealed that the Glut-1 was expressed in endothelial cells of IH, but not in NICH.
CONCLUSIONS: NICH has a steady histologic structure and low proliferation, while the endothelial cells in proliferative IH has a high proliferation. Glut-1 can be used as the reliable marker antigen for differential diagnosis of NICH and proliferative infantile hemangiomas.

Dermatopathology Case 103

2011-11-24T08:59:00.000-08:00

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Case 103

A 9 month old child with a reddish nodule on the forehead.

Diagnosis

Answer of Dermatopathology Case 102

2011-11-23T10:17:00.001-08:00

Acrodermatitis Chronica Atrophicans

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There is superficial and deep chronic inflammatory cellular infiltrate in the dermis.
It is moderately heavy and composed mainly of lymphocytes together with histiocytes and plasma cells.
There is telangiectasia and the cellular infiltrate is prominent around the blood vessels.
Inflammatory cells are also present around the adnexae.
Thickened collagen bundles are haphazardly present in the upper half of the dermis.
There is some atrophy of the dermis and of the subcutis. The epidermis also shows variable atrophy with loss of rete pegs.