Interactive Case Study - July 2022

Numerous monomorphic discrete red-brown macules and papules on the trunk and extremities of a 47-year-old male.

Murphy R. Mastin MD

Emma F. Johnson MD

A 47-year-old male with multiple monomorphic discrete red-brown macules and papules with associated telangiectatic components involving the trunk and extremities (Figures 1 and 2) presented to the outpatient dermatology clinic for further evaluation. Punch biopsies of representative lesions were performed. The lesions did have associated pruritis and would urticate with mechanical manipulation.

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Figure 1

Figure 2

Figure 3

Figure 4

Figure 5

What is the best diagnosis?

Telangiectasia macularis eruptive perstans

Generalized essential telangiectasias

Neutrophilic urticarial dermatosis

Pigmented purpuric dermatosis

urticaria

What is the approximate percentage risk for systemic mastocytosis in patients with TMEP?

A, B, C and E

50%

Severino M., et al. Telangiectasia macularis eruptiva perstans (TMEP): A form of cutaneous mastocytosis with potential systemic involvement. J Am Acad Dermatol. 2016; 74;5: p. 885-891
Shukla S.A., et al. Mast cell ultrastructure and staining in tissue. Methods Mol Biol. 2006;315; p. 63-76.
Kelly R., Baker C. Chapter 106. Other vascular disorders. In: Bolognia JL,‎ Schaffer JV,‎ Cerroni L, eds.Dermatology. 4th ed. Philadelphia, PA: Elsevier; 2017.
Broekaert S.M., et. al. Neutrophilic Epitheliotropism is a Histopathological Clue to Neutrophilic Urticarial Dermatosis. Am J Dermatopathol 2016; 38: p. 39-49.
Hercogova J. Persistent pigmented purpuric dermatoses: who are you. J Eur Acad Dermatol Venereol 2001; 15: p. 15.
Sardana K., Sarkar R., Sehgal V.N. Pigmented purpuric dermatoses: an overview.Int J Dermatol. 2004; 43;7: p.482-8.
Peroni A., et. al. Urticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria: Part II. Systemic diseases. J Am Acad of Dermatology 2010; 62;4: p. 557-570
Sánchez J.L., Benmamán O. Clinicopathological correlation in chronic urticaria. Am J Dermatopathol 1992; 14: p. 220-223.
Wilmas K., Wohlmuth-Wieser I., Duvic M. Telangiectasia macularis eruptiva perstans: an old terminology, still frequently used.Dermatol Online J. 2017; 23;8.
Valent P, Akin C, Metcalfe DD. Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood. 2017;129;11: p. 1420-1427

Acknowledgements

Author Profiles

Murphy R. Mastin

Mayo Clinic

Murphy R. Mastin, MD, is a second year (PGY-3) Dermatology Resident at the Mayo Clinic. He will complete a Dermatopathology Fellowship at the University of Virginia after completing his Dermatology Residency in June 2022. Dr. Mastin plans to practice both General Dermatology and Dermatopathology at Citizens Memorial Hospital in rural Bolivar, Missouri once his training is complete.

Emma F. Johnson

Mayo Clinic

Emma F. Johnson, MD, completed her Dermatology Residency and Dermatopathology Fellowship at the Mayo Clinic in Rochester, Minnesota. She is currently practicing as both a staff Dermatologist and Dermatopathologist at the Mayo Clinic. She has a special interest in granulomatous skin disorders, cutaneous lymphomas, and cutaneous vasculitis and vasculopathies.

Question:

Interactive Case Study - July 2022

Acknowledgements

Author Profiles

Numerous monomorphic discrete red-brown macules and papules on the trunk and extremities of a 47-year-old male.

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