DERMATOPATHOLOGY CASES: Self-Assessment Cases: Editor - Dr Sampurna Roy MD

Digital Images of interesting cases that will include the full spectrum of Dermatopathology, presented in the form of quiz.

The answer of the cases include related links and recent abstracts of articles.

Friday, February 5, 2010

Answer of Dermatopathology Case 40

Acute Febrile Neutrophilic Dermatosis or Sweet's Syndrome

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Sweet's syndrome: an update and review.G Ital Dermatol Venereol. 2009 Oct;144(5):603-12.

Sweet's syndrome, or acute neutrophilic dermatosis, is an unusual dermatologic disorder that may serve as a marker of leukemia or lymphoma, other malignancy or another serious systemic disorder. It is characterized by the sudden eruption of tender discrete erythematous nodules or plaques which are sometimes associated with fever, neutrophilic leukocytosis, and a dense dermal infiltrate of mature neutrophils. It may occur as a hypersensitivity reaction with cytokines playing a pivotal role. It may remit after the treatment of an underlying cancer or discontinuation of an offending pharmacologic agent, although it con also resolve spontaneously without therapeutic intervention. The gold standard of treatment is with systemic corticosteroids; however, potassium iodide, colchicine and other therapeutic agents have been utilized successfully.

Acute febrile neutrophilic dermatosis - Sweet's syndrome. Acta Reumatol Port. 2009 Jul-Sep;34(3):536-40.

In Rheumatology there are several diseases that frequently develop cutaneous manifestations creating diagnostic difficulties. The Sweet's syndrome appears as archetype of the neutrophilic dermatosis,which is a group of not infectious illnesses, characterized for a dermic neutrophilic and angiocentric infiltrated. The four main features that define this syndrome are: cutaneous eruption, fever, peripheral neutrophilia and dermic neutrophilic infiltrated without vasculitis on skin biopsy. The authors describe a typical clinical case of Sweet's syndrome, pointing out the multiplicity of clinical situations that can simulate this pathology, making difficult its diagnosis and highlighting the need for suspicious in patients with both musculoskeletal and cutaneous involvement.

Neutrophilic dermatoses: a review of current treatment options.Am J Clin Dermatol. 2009;10(5):301-12.

Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis are neutrophilic dermatoses - conditions that have an inflammatory infiltrate consisting of mature polymorphonuclear leukocytes. The neutrophils are usually located within the dermis in Sweet syndrome and pyoderma gangrenosum; however, in subcorneal pustular dermatosis, they are found in the upper layers of the epidermis. Sweet syndrome, also referred to as acute febrile neutrophilic dermatosis, is characterized by pyrexia, elevated neutrophil count, painful erythematous cutaneous lesions that have an infiltrate of mature neutrophils typically located in the upper dermis, and prompt clinical improvement following the initiation of systemic corticosteroid therapy. Classical, malignancy-associated, and drug-induced variants of Sweet syndrome exist. Pyoderma gangrenosum is characterized by painful, enlarging necrotic ulcers with bluish undermined borders surrounded by advancing zones of erythema; its clinical variants include: ulcerative or classic, pustular, bullous or atypical, vegetative, peristomal, and drug-induced. Subcorneal pustular dermatosis is an uncommon relapsing symmetric pustular eruption that involves flexural and intertriginous areas; it can be idiopathic or associated with cancer, infections, medications, and systemic diseases. Since Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis share not only the same inflammatory cell but also similar associated systemic diseases, it is not surprising that the concurrent or sequential development of these neutrophilic dermatoses has been observed in the same individual. Also, it is not unexpected that several of the effective therapeutic interventions - including systemic drugs, topical agents, and other treatment modalities - for the management of these dermatoses are the same. The treatment of choice for Sweet syndrome and idiopathic pyoderma gangrenosum is systemic corticosteroids; however, for subcorneal pustular dermatosis, dapsone is the drug of choice. Yet, tumor necrosis factor-alpha antagonists are becoming the preferred choice when pyoderma gangrenosum is accompanied by inflammatory bowel disease or rheumatoid arthritis. Potassium iodide and colchicine are alternative first-line therapies for Sweet syndrome and indomethacin (indometacin), clofazimine, cyclosporine (ciclosporin), and dapsone are second-line treatments. Cyclosporine is effective in the acute management of pyoderma gangrenosum; however, when tapering the drug, additional systemic agents are necessary for maintaining the clinical response. In some patients with subcorneal pustular dermatosis, systemic corticosteroids may be effective; yet, systemic retinoids (such as etretinate and acitretin) have effectively been used for treating this neutrophilic dermatosis - either as monotherapy or in combination with dapsone or as a component of phototherapy with psoralen and UVA radiation. Topical agents can have an adjuvant role in the management of these neutrophilic dermatoses; however, high-potency topical corticosteroids may successfully treat localized manifestations of Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis. Intralesional corticosteroid therapy for patients with Sweet syndrome and pyoderma gangrenosum, hyperbaric oxygen and plasmapheresis for patients with pyoderma grangrenosum, and phototherapy for patients with subcorneal pustular dermatosis are other modalities that have been used effectively for treating individuals with these neutrophilic dermatoses.

Dermatopathology Case 40


Case 40

A 52 year old female with fever, an elevated neutrophil count, and tender, dark-red plaques on the upper extremities.

Thursday, February 4, 2010

Answer of Dermatopathology Case 39


Procollagen 1 and Melan-A expression in desmoplastic melanomas.Am J Dermatopathol. 2009 Apr;31(2):173-6.

The histopathologic diagnosis of desmoplastic melanoma (DM) is usually based on typical conventional microscopic findings coupled with expression of S100 protein by neoplastic cells. Important differential diagnostic considerations include atypical fibroxanthoma (AFX) and spindle cell squamous carcinoma. Spindle cell squamous cell carcinoma is characterized by positivity for cytokeratin, whereas the diagnosis of AFX has been one of exclusion. Procollagen 1 (PC1) has been identified as a relatively sensitive marker of AFX. In this study, we sought to analyze the expression of PC1, S100 protein, and Melan-A in a series of 37 DMs (27 males and 10 females; ages 36-92 years, mean age 74 years). All lesions displayed a spindle cell morphology with varying degrees of desmoplasia. The neoplastic cells avidly expressed S100 protein in 37 of 37 neoplasms. A complete lack of PC1 expression was noted in 24 of 37 (64.9%). There was a weak PC1 expression by 9 DMs (24.3%) and a moderate expression by 4 DMs (10.8%). Melan-A expression was found in 19 of 37 DMs (51.4%), but in 10 lesions, the expression was only faint and focal. We conclude that PC1 labeling of DM is not uncommon but poses little risk for misdiagnosis, provided the stain is performed as part of panel that includes S100 protein. Melan-A offers little for the diagnosis of DM, as less than a quarter of lesions exhibit a strong reaction with this antibody. It is critical to employ a broad panel of antibodies, including S100 protein, Melan-A, cytokeratin, PC1, and others, in the immunohistochemical evaluation of spindle cell neoplasms of the skin.

Desmoplastic malignant melanoma: a clinicopathologic analysis of 113 cases.Am J Dermatopathol. 2008 Jun;30(3):207-15.

Desmoplastic melanoma (DM) is a rare variant of spindle cell melanoma, which usually develops in sun-damaged skin of elderly patients. Often the lesion is nonpigmented and frequently mistaken for a nonmelanocytic proliferation, which delays diagnosis and treatment and therefore worsens the prognosis. The spindle shape of neoplastic melanocytes, the prominent desmoplasia, and the frequent neurotropism of neoplastic melanocytes are its most characteristic histopathological features. We have studied the clinicopathologic features of 113 cases of DM. The mean age of the patients was 71.1 years; 48% of the patients were males and 52% were females. The neoplasm was located on the head in 72% of the cases. Malignant melanoma was the initial clinical diagnosis in only 27% of the cases. Histopathologically, all lesions appeared as poorly demarcated neoplasms that involved the entire dermis and often extended into the subcutaneous tissue. The neoplasms were composed of ill-defined fascicles of spindle cells. Desmoplasia was defined as the presence of spindle cells associated with a fibrotic stroma. Fifty-one cases (45%) were classified as "pure DM" when the lesion was entirely desmoplastic, and 62 cases (55%) were considered as "combined DM" when a recognizable desmoplastic component was seen in an otherwise conventional malignant melanoma. In 81% of the cases, an atypical intraepidermal melanocytic component (in situ malignant melanoma) was identified, whereas in the remaining 19% of the cases the intraepidermal component was lacking. Seventy-one percent of the cases were histologically amelanotic, 23% showed a small amount of pigment, and only 6% were heavily pigmented. Neural involvement was identified in 40/113 cases (35%), predominantly in the thickest tumors. Lymphoid nodules, found in 42/113 cases (37%), were significantly more frequent in pure DM than in combined DM (53% vs 24%). The null hypothesis of homogeneity of the "pure" and "combined" subgroups should be rejected (P <0.002).>Solar elastosis, with variable intensity, was seen in 82% of the cases.

Mean Breslow thickness was 4.1 mm (4.6/3.7 mm, in the pure/combined subgroups, respectively), median was 4.0 mm (4.0/3.0 mm); Breslow thickness ranged from 0.3 to 11.0 mm, with half of the cases thicker than 4 mm. Only 4% of the cases showed Clark level below IV. The predominant neoplastic cells consisted of spindle-shaped melanocytes in 85% of the cases, whereas the remaining 15% of the cases demonstrated round neoplastic cells forming the main mass of the neoplasm. The mitotic rate of the neoplastic cells was low in 72% of the cases, 23% had an intermediate mitotic rate, and 5% showed a high mitotic rate. On follow-up, 55/113 patients (49%) (with an average of 55 months) demonstrated persistence of the disease. About 4% had local recurrences, 2% of lymph node invasion, 9% systemic metastases, and 12% died from the disease (2 cases of pure DM and 5 cases of combined DM). Although a better prognosis has been postulated for DM when compared with conventional cutaneous malignant melanomas of the same thickness, in most cases, a DM is diagnosed only in established long-standing and thick melanomas. Therefore, dermatologists and dermatopathologists should be more aware of this clinicopathologic variant of cutaneous malignant melanoma.

Dermatopathology Case 39


Case 39

An indurated nodule on the forehead of a 70 year old male.

Tuesday, February 2, 2010

Answer of Dermatopathology Case 38


Visit: Pathology of Trichoblastoma
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Cytologic features of trichoblastoma in fine needle aspiration biopsies.Acta Cytol. 2009 Nov-Dec;53(6):679-82.
OBJECTIVE: To review the cytologic features of trichoblastoma in order to define criteria that may aid in identification of these tumors at the time of aspirationand allow a definitive diagnosis. STUDY DESIGN: A 58-year-old male presented witha mass lesion on the thigh. On fine needle aspiration, the patient was diagnosed as having a benign skin adnexal tumor. Histology showed the presence of atrichoblastic fibroma, and a retrospective analysis of the cytology wasperformed. RESULTS: The cytologic features of trichoblastoma resembled a cellular fibroadenoma/phyllodes tumor on aspiration, not previously described in theliterature. If the cytomorphology of a skin or subcutaneous aspirate appears to resemble that of a fibroadenoma, the diagnosis of a trichoblastoma should be entertained. Peripheral palisading of nuclei at the edges of the basaloid cellsheets and squamous eddy formation are clues to the diagnosis but may be very focal and could be overlooked. If the tumor occurs in the region of the breast, distinction from a fibroadenoma would be difficult if these additional featureswere not prominent. CONCLUSION: Knowledge of the cytologic features of trichoblastoma will allow correct management of the patient and prevent misdiagnosis as other benign or malignant tumors.

Bcl-2 and CD10 expression in the differential diagnosis of trichoblastoma, basal cell carcinoma, and basal cell carcinoma with follicular differentiation. Int J Dermatol. 2009 Jul;48(7):713-7.
BACKGROUND: Both trichoblastoma and basal cell carcinoma (BCC) of the skin are characterized morphologically by the proliferation of basaloid cells; however,BCCs are clinically associated with a more aggressive behavior. An accurate diagnosis of these lesions is essential for effective, timely treatment and appropriate therapeutic decisions. METHODS: This study includes 40 lesions. Bcl-2and CD10 immunohistochemistry were performed in all cases and the patterns ofexpression were analyzed. RESULTS: Bcl-2 is useful for the detection of BCC with diffuse expression in nests of basaloid cells, but cannot distinguish between BCC with follicular differentiation and trichoblastoma, as both lesions show the samepattern with positive and negative areas. Conversely, CD10 expression can distinguish between trichoblastomas with peritumoral stromal staining and BCCswith epithelial staining. If both stromal and epithelial areas are stained, thesecases are classified as BCC with follicular differentiation. CONCLUSIONS: CD10 is useful for distinguishing between BCC with widespread follicular differentiation and trichoblastomas.

Dermatopathology Case 38


Case 38

A well cicumscribed solitary lesion (1.5cm in diameter) on the scalp of a 35 year old male.