Quiz 42: What is your diagnosis?

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Diagnosis: Quiz 42

Quiz 42

Answer: Fibrous papule of the face

Criteria for diagnosis clinically: A smooth-surfaced pink papule on a nose is typical of fibrous papule.

Differential diagnosis clinically: This could be a Miescher's nevus or a benign adnexal neoplasm and, that being so, biopsy is essential to coming to a diagnosis with precision.

Criteria for diagnosis histopathologically: In addition to an increase in venules dilated markedly in a stroma characterized by coarse bundles of collagen in lamellar arrangement and in company with an increase in number of fibrocytes, there are distinctly abnormal hair follicles in the reticular dermis, they, too, being enveloped by lamellar fibroplasia that, in turn, is separated from the adjacent fibrotic dermis by clefts. Those findings, in toto, are ones of fibrous papule of the face.

Differential diagnosis histopathologically: There is none.

Clinicopathologic correlation: The papule is formed mostly of the elements angiofibromatous in the dermis, the pink hue is a result of the numerous dilated venules in the dermis having, in vivo, been stuffed with erythrocytes, and the surface is smooth because the stratum corneum is normal.

Options for therapy predicated on knowledge of histopathologic findings: A diagnosis rendered by a histopathologist of fibrous papule  should telegraph to a clinician that the lesion is benign (a hamartoma) and, that being the case, no more surgery is indicated.

1) Fibrous papule of the face, known also as fibrous papule of the nose, is a hamartoma in which the rudimentary constituents are aberrant hair follicles, an exaggerated perifollicular sheath, and elements angiofibromatous within the dermis. In many an example of "fibrous papule," there is a notable increase in number of melanocytes disposed as solitary units at the dermoepidermal junction, a phenomenon well in evidence here. It was that change which prompted Richard J. Reed to name the lesion  melanocytic angiofibroma. In fact, however, it is not basically an angiofibroma, but a hamartoma made up always of elements follicular, as well as ones angiofibromatous and, episodically, an increase in the number of melanocytes at the dermoepidermal junction.

2) Many fibrocytes scattered throughout the dermis of a fibrous papule tend to be binucleate and multinucleate and those, as well as ones mononuclear, are given to sport cytoplasm copious and stellate. Sometimes some of those fibrocytes house a basophilic round inclusion body in their cytoplasm, the meaning of which has yet to be elucidated. Fibrocytes are increased in number not only in the interfollicular dermis, but in widened perifollicular sheaths.

3) When fibrous papules are present on a face in number legion, sometimes many hundreds of them, the condition is known as adenoma sebaceum, it being a manifestation of the "tuberous sclerosus complex" typified by epiloia, a mnemonic for epilepsy, low intelligence, and a denoma sebaceum. In short, each lesion of adenoma sebaceum is a fibrous papule of the face.

4) It is well established that a hamartoma or a benign neoplasm that presents itself as a solitary lesion may express itself also as lesions innumerable, they often being situated on a face. Examples in addition to "fibrous papule" are conventional trichoepithelioma, that when encountered in number was known in times past as "epithelioma adenoides cysticum," fibrofolliculoma/trichodiscoma, that when countless and widespread is a manifestation of Burt-Hogg-Dube syndrome, and neurofibroma, that when innumerable and generalized is known as neurofibromatosis (von Recklinghausen's disease).

5) What has just been written about hamartomas and benign neoplasms applies equally to some malignant neoplasms, e.g., solitary basal-cell carcinoma vis-à-vis ones innumerable in nevoid basal-cell carcinoma syndrome and solitary sebaceous carcinoma vis-à-vis the ones countless in Muir-Torre syndrome.

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