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.

Sunday, December 18, 2011

Answer of Dermatopathology Case 111

Darier's Disease (Keratosis Follicularis)

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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




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.

Saturday, December 17, 2011

Answer of Dermatopathology Case 110

Dermatofibroma with Monster Cells

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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




Case 110

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


Saturday, December 3, 2011

Answer of Dermatopathology Case 109

HMB-45 : Positive

Dermatopathology Case 109


Case 109

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