Skip to main content

Multiple Clustered Dermatofibromas Associated With Pulmonary Arterial Hypertension.

Publication ,  Journal Article
Komforti, M; Jewell, J; Pavlis, M
Published in: Am J Dermatopathol
March 2016

BACKGROUND: Dermatofibromas (DFs) are common, benign, fibrohistiocytic tumors of the skin. Clinically, if they present in a rapid succession, they are termed multiple eruptive DFs. When they arise in a localized distribution, they are termed multiple clustered dermatofibromas (MCDFs). These DF variants are rare, and the subtype of multiple eruptive DFs has been associated with autoimmune diseases, hypertriglyceridemia, pregnancy, HIV, and primary pulmonary hypertension. Conversely, there is a paucity of published clinical associations with MCDF. METHODS: A 23-year-old white woman with pulmonary arterial hypertension presented with a 7-year history of multiple, asymptomatic, firm, brown, and violaceous papules clustered on the right hip. RESULTS: Histopathologic examination of the biopsies demonstrated acanthotic epidermis, proliferation of cytologically bland fibrohistiocytic spindle cells in a fascicular pattern in the dermis, and collagen trapping on the periphery. Immunohistochemistry was focally positive for factor XIIIa and negative for CD34, thus supporting the clinical diagnosis of MCDF. CONCLUSIONS: A case of MCDF associated with pulmonary arterial hypertension is presented. To date, there have been greater than 25 reported cases of MCDF, but only one other publication has described an associated systemic comorbidity.

Duke Scholars

Altmetric Attention Stats
Dimensions Citation Stats

Published In

Am J Dermatopathol

DOI

EISSN

1533-0311

Publication Date

March 2016

Volume

38

Issue

3

Start / End Page

e30 / e32

Location

United States

Related Subject Headings

  • Young Adult
  • Skin Neoplasms
  • Immunohistochemistry
  • Hypertension, Pulmonary
  • Humans
  • Histiocytoma, Benign Fibrous
  • Female
  • Dermatology & Venereal Diseases
  • Biomarkers, Tumor
  • 3202 Clinical sciences
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Komforti, M., Jewell, J., & Pavlis, M. (2016). Multiple Clustered Dermatofibromas Associated With Pulmonary Arterial Hypertension. Am J Dermatopathol, 38(3), e30–e32. https://doi.org/10.1097/DAD.0000000000000423
Komforti, Miglena, Jolene Jewell, and Michelle Pavlis. “Multiple Clustered Dermatofibromas Associated With Pulmonary Arterial Hypertension.Am J Dermatopathol 38, no. 3 (March 2016): e30–32. https://doi.org/10.1097/DAD.0000000000000423.
Komforti M, Jewell J, Pavlis M. Multiple Clustered Dermatofibromas Associated With Pulmonary Arterial Hypertension. Am J Dermatopathol. 2016 Mar;38(3):e30–2.
Komforti, Miglena, et al. “Multiple Clustered Dermatofibromas Associated With Pulmonary Arterial Hypertension.Am J Dermatopathol, vol. 38, no. 3, Mar. 2016, pp. e30–32. Pubmed, doi:10.1097/DAD.0000000000000423.
Komforti M, Jewell J, Pavlis M. Multiple Clustered Dermatofibromas Associated With Pulmonary Arterial Hypertension. Am J Dermatopathol. 2016 Mar;38(3):e30–e32.

Published In

Am J Dermatopathol

DOI

EISSN

1533-0311

Publication Date

March 2016

Volume

38

Issue

3

Start / End Page

e30 / e32

Location

United States

Related Subject Headings

  • Young Adult
  • Skin Neoplasms
  • Immunohistochemistry
  • Hypertension, Pulmonary
  • Humans
  • Histiocytoma, Benign Fibrous
  • Female
  • Dermatology & Venereal Diseases
  • Biomarkers, Tumor
  • 3202 Clinical sciences