Background

The dermis is a dynamic environment with complex interactions of collagen, blood vessels, elastic fibers, and mucopolysacchardies and ground substance. The family of enzymes known as Matrix Metalloproteinases (MPP) play a major role in maintaining the tight control of events.

From the diagnostic standpoint, the pathologist determines the location of inflammatory cells within the dermis and examines any alterations within the collagen. Putting together a combination of these changes leads to a differential diagnosis. Finally, combining these changes with the clinical history and appearance of the lesion leads to a diagnosis.

• Anetoderma
• Dermal mucinosis
• Eosinophilic Fasciitis
• Fibroblastic Rheumatism
• Keloid (Hypertrophic Scar)
• Lichen Sclerosis et Atrophicus (LSEA)
• Mid-dermal Elastolysis
• Nephrogenic Fibrosing Dermopathy
• Porphyria
• Pseudoxanthoma elasticum
• Restrictive Dermopathy
• Sarcoidosis
• Scleroderma/Morphea

OUTLINE

Gross Appearance and Clinical Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Commonly Used Terms
Internet Links

DISEASE VARIANTS	CHARACTERIZATION
ACROKERATO-ELASTOIDOSIS
Acrokeratoelastoidosis. Bogle MA, Hwang LY, Tschen JA. St. Joseph Hospital, and the Department of Dermatology, Baylor College of Medicine.	J Am Acad Dermatol 2002 Sep;47(3):448-51 Abstract quote Acrokeratoelastoidosis is a genodermatosis characterized by firm papules or plaques on the sides of the hands and feet. Although poorly understood, the lesions may result from an abnormality in the secretion or excretion of elastic material by fibroblasts in the dermis. In this report, we will present a patient with this rare condition and review the clinical and histopathologic features, cause, and differential diagnosis.
ELASTIC TISSUE DISORDERS
Acquired disorders of elastic tissue: Part II. decreased elastic tissue. Lewis KG, Bercovitch L, Dill SW, Robinson-Bostom L. Department of Dermatology, Brown Medical School-Rhode Island Hospital, Providence, RI 02903, USA	J Am Acad Dermatol. 2004 Aug;51(2):165-85; Abstract quote Elastic fibers in the extracellular matrix are integral components of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin are attributable to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studying the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood owing to the paucity of reported cases. Several acquired disorders in which loss of dermal elastic tissue produces prominent clinical and histopathologic features have recently been described, including middermal elastolysis, papular elastorrhexis, and pseudoxanthoma-like papillary dermal elastolysis, which must be differentiated from more well-known disorders such as anetoderma, acquired cutis laxa, and acrokeratoelastoidosis. Learning objective At the conclusion of this learning activity, participants should have an understanding of the similarities and differences between acquired disorders of elastic tissue that are characterized by a loss of elastic tissue.
Acquired disorders of elastic tissue: Part I. increased elastic tissue and solar elastotic syndromes. Lewis KG, Bercovitch L, Dill SW, Robinson-Bostom L. Departments of Dermatology and Pathology, Brown Medical School-Rhode Island Hospital. USA.	J Am Acad Dermatol. 2004 Jul;51(1 Pt 1):1-21. Abstract quote   Elastic fibers in the extracellular matrix are an integral component of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin may be attributed to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studying the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood due to the paucity of reported cases. Several acquired disorders in which accumulation or elastotic degeneration of dermal elastic fibers produces prominent clinical and histopathologic features have recently been described. They include elastoderma, linear focal elastosis, and late-onset focal dermal elastosis and must be differentiated from better-known disorders, among them acquired pseudoxanthoma elasticum, elastosis perforans serpiginosa, and Favre-Racouchot syndrome. Learning objective At the conclusion of this learning activity, participants should understand the similarities and differences between acquired disorders of elastic tissue that are characterized by an increase in elastic tissue, as well as the spectrum of solar elastotic dermatoses.
OSTEOMA CUTIS
Osteomas of the skin revisited: a clinicopathologic review of 74 cases. Conlin PA, Jimenez-Quintero LP, Rapini RP.	Am J Dermatopathol 2002 Dec;24(6):479-83 Abstract quote Cutaneous ossification is an unusual event that may be primary or secondary to either inflammatory or neoplastic processes. It is classified as primary when it occurs in the absence of a demonstrable preexisting lesion. Secondary lesions have been most commonly reported occurring with pilomatricoma, basal cell carcinoma, acne vulgaris, and melanocytic nevi (nevus of Nanta). Histologically, the osteomas are composed of well-formed bony spicules with prominent cement lines and calcification. They may demonstrate osteoblasts, osteoclasts, and osteocytes and occasionally may even demonstrate bone marrow elements. We searched the files of a reference dermatopathology laboratory to identify cases of either primary or secondary cutaneous ossification. We present a series of 74 cases of primary and secondary cutaneous ossification. Most cases were secondary in nature. Lesions were more common on the head and neck and in whites. Lesions were also more commonly identified in female patients. In addition, included in our series are 19 cases of nevus of Nanta. To our knowledge, this represents the largest series of such cases in the English literature. Cutaneous ossification is seen both in primary and, more commonly, in secondary conditions involving the skin. Benign neoplasms, especially melanocytic nevi, represent the most common cause of secondary osteoma formation. Women are more commonly affected than men, but the reason for this is unclear. The exact reason why osteoma formation occurs is unclear and requires further study.

SPECIAL STAINS/ IMMUNOPEROXIDASE/ ELECTRON MICROSCOPY

CHARACTERIZATION

HSP47 is a useful marker for skin fibroblasts in formalin-fixed, paraffin-embedded tissue specimens K. Kuroda and S. Tajima

Journal of Cutaneous Pathology Volume 31 Issue 3 Page 241 - March 2004 Abstract quote Aim: This study was undertaken to assess whether heat-shock protein (HSP)47 is a useful cell marker for skin fibroblasts in formalin-fixed, paraffin-embedded skin specimens. Background: HSP47, a 47-kDa HSP, is a collagen-specific molecular chaperone localized in the endoplasmic reticulum. HSP47 plays an essential role in collagen biosynthesis in skin fibroblasts. Methods: Immunohistochemistry was performed to detect HSP47 in skin fibroblast cultures and skin tissue sections. Results: Immunostaining for HSP47 clearly detected skin fibroblasts in paraffin tissue sections as well as in fibroblast cultures and frozen tissue sections. HSP47 staining on paraffin sections from diseased skin specimens revealed that skin ulcer, keloid, nodular fascitis, spindle cell lipoma, and dermatofibroma had strong signals for HSP47 compared with the signals obtained from normal skin. Dermatofibrosarcoma protuberans had many HSP47 positive cells, but signals on individual cells were not as strong as those seen from the above benign proliferative disease samples. In neurofibroma, a small number of faintly positive cells were detected. Our double-immunostaining studies also demonstrated that HSP47 staining distinguished skin fibroblasts from CD68-positive histiocytes/macrophages, factor VIII-related antigen-positive endothelial cells, or factor XIIIa-positive dermal dendritic cells. CD34-positive interstitial cells coexpressed HSP47 in spindle cell lipoma. Conclusions: These findings indicate that HSP47 staining can detect skin fibroblasts in routine, paraffin-embedded specimens. A panel approach using HSP47 and other cell markers on paraffin sections may help the identification of the cell type involved with mesenchymal proliferative disorders.

Weedon D. Weedon's Skin Pathology. Churchill Livingstone. 1997.
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.

Collagen

Matrix Metalloproteinases (MPP)

Skin

Basic Principles of Disease
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Commonly Used Terms
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Diagnostic Process
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Surgical Pathology Report
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Special Stains
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