2023 Case Studies – Derm In-Review

February 2023 Case Study

By 2023 Case Studies

February 2023 Case Study

by Alexis E. Carrington, MD

A 52 year old female with a past medical history of vulvar lichen sclerosus presents to the clinic for a rash on the back and legs for 8 months. She reports the rash is itchy and the areas on the back are worse during the summer months and with sweating. She is using Aquaphor ointment and a topical antibiotic cream with no improvement. She denies any family history of similar findings or exposure to new personal or household items. Punch biopsy of a lesion on the back is consistent with lichen sclerosus.

Which of the following is true about lichen sclerosus?

A.) It is associated with HLA-DQ2
B.) It is associated with anti-extracellular matrix protein 1 antibodies
C.) Genital lesions can be associated with development of BCC
D.) Treatment of extragenital disease involves the use of a low-potency topical steroid


Extragenital lichen sclerosus typically favors the neck, shoulders, trunk, proximal extremities, flexor wrists and sites of trauma. It is associated with xerosis and mild pruritus, but is usually asymptomatic. The lesions start as white, shiny, polygonal papules that coalesce into plaques and evolve into atrophic patches and plaques. Lesions may also affect the skin around the eye and other areas of the face.

Lichen sclerosus is usually a clinical diagnosis but a biopsy is reasonable to confirm diagnosis. Topical steroids are the gold standard for this condition. Patients should be referred to a dermatologist and gynecologist or urologist for management of genitourinary complications.

Correct answer: B.) It is associated with anti-extracellular matrix protein 1 antibodies

Answer A is associated with Dermatitis Herpetiformis, whereas lichen sclerosus is associated with HLA-DQ7. There is an increased risk of genital squamous cell carcinoma in both men and women with lichen sclerosus, not basal cell carcinoma (Answer C). Treatment of extragenital disease involves the use of high potency topical steroids, rather than low-potency (Answer D).



  1. Bolognia, Jean Dermatology volume 2. Mosby, 2018.
  2. Fistarol SK, Itin Diagnosis and treatment of lichen sclerosus: an update. Am J Clin Dermatol. 2013 Feb;14(1):27-47. PubMed ID: 23329078
  3. Lewis FM, Tatnall FM, Velangi SS, et al. British Association of Dermatologists guidelines for the management of lichen sclerosus, Br J Dermatol. 2018 Apr;178(4):839-853. PubMed ID: 29313888

January 2023 Case Study

By 2023 Case Studies

January 2023 Case Study

by Adam Rosenfeld, MD

61-year-old male with history of HTN, DM, HLD who presented for a new growth on his mid abdomen. He noted a history of a trauma to the area with a metal blade 20 years prior while in active duty, with the area initially healing with a raised scar. He endorsed significant increase in size of the area over the last year, but it had been slowly growing for several years. The patient had been told this was a keloid. He denies any significant pain or associated symptoms. On examination, there was a large approximate 4x5cm protuberant skin colored to pink mass on the left mid abdomen (figure 1). A punch biopsy was obtained of this tumor demonstrating a dense dermal infiltrate of spindle cells (figure 2).

A diagnosis of a malignant dermal proliferation was made, often mimicking the diagnosis the patient had been told before. What stain was confirmatory in making this diagnosis?

A.) CD207+
B.) Factor XIIIA+
C.) S100+
D.) CD34+
E.) SMA+

Correct answer: D.) CD34+

This multi-loculated tumor is suspicious for dermatofibrosarcoma protuberans, or DFSP which classically stains diffusely positive for CD34. CD34 is a marker for vascular endothelium and hematopoietic progenitor cells4. It is positive in DFSP and negative in dermatofibromas. Dermatofibrosarcoma protuberans is a fibroblast derived intermediate-grade soft tissue sarcoma that can be mistaken for a keloid3. It is often slow growing and asymptomatic. It can present as a bulky multi-loculated tumor as in our patient but can also have much more innocent appearances. Its pathophysiology is unclear, but some have proposed prior injury to the skin as a triggering event1. Evidence does support these tumors arising from fibroblastic, histiocytic or neuroectoderm tissue although a fibroblastic origin seems most likely. The growth of the tumor is driven in most cases by a translocation of chromosome 17 and 22 forming the t(17,22) COLA1A-PDGFG fusion protein1. On histopathology, a dense dermal infiltrate of spindle cells is seen infiltrating into the deeper fat causing the “honey combing” appearance of the adipose tissue4. These cells stain diffusely positive for CD34+, while staining weakly or not at all for Factor XIIIA+ (answer choice B), the stain pathognomonic for dermatofibromas4. Importantly, it is crucial to rule out other spindle cell neoplasms including a spindle cell melanoma which would stain positive for S100 (answer choice C)4. While DFSPs are malignant, they typically do not demonstrate significant atypia histologically. Fibrosarcomatous change represents a subset of DFSPs that demonstrate much more atypical features, indicating tumor progression3. The differential diagnosis of DFSP includes but is not limited to a keloid, dermatofibroma, spindle cell melanoma, squamous cell carcinoma, Kaposi sarcoma.

The standard of care for treatment is surgical excision with every effort to achieve clear surgical margins both peripherally and deep2. Although wide local excision can be performed, Mohs Micrographic Surgery (MMS) is now recommended for these tumors. Given that it demonstrates unpredictable microscopic, tentacle like extensions, the directionality afforded by MMS makes it uniquely fit for DFSPs3. No set margins exist but wide local excision typically includes 2-4cm. MMS initial margins vary but the literature suggest 0.5-1.3cm3. Unresectable or metastatic tumors can be treated with radiation or Imatinib, a tyrosine kinase inhibitor5,7. NCCN guidelines do not recommended extensive systemic work up of these patients unless there are red flags for metastasis on exam or through history. Imaging with CT or MRI can be performed to assess for tumor depth but is based on a case-by-case basis8. These tumors have a low risk of metastasis (1-4%) but a high chance of local recurrence. Surveillance includes observation of primary site every 6 months for the first 5 years with yearly exams following3. The overall prognosis of DFSPS is good, with a 10-year survival rate of 99.1%3.

Incorrect stains in answer choices4

CD 207 (choice A) – surrogate marker for presence of Birbeck granules in Langerhans cells

Factor XIIIA (choice B) – stains dermal dendritic cells, positive in dermatofibroma and negative in DFSP

S100 (choice C) – stains melanocytes, Langerhans cells, sweat glands, nerves, Schwann cells, myoepithelial cells, fat, muscle and chondrocytes

SMA (choice E) – positive in smooth muscle, including vascular smooth muscle; negative in skeletal muscle; expressed in myofibroblasts and myoepithelial cells



  1. Bolognia, J., Schaffer, J., & Cerroni, L. (2018). Dermatology (Fourth edition.). Philadelphia,Pa: Elsevier.
  2. Dermatofibrosarcoma protuberans – statpearls – NCBI bookshelf. Dermatofibrosarcoma Protuberans. (n.d.). Retrieved August 11, 2022, from https://www.ncbi.nlm.nih.gov/books/NBK513305/
  3. Hao X;Billings SD;Wu F;Stultz TW;Procop GW;Mirkin G;Vidimos AT; (n.d.). Dermatofibrosarcoma protuberans: Update on the diagnosis and treatment. Journal of clinical medicine. Retrieved August 11, 2022, from https://pubmed.ncbi.nlm.nih.gov/32516921/
  4. Elston, D. M. (2019). Dermatopathology. Elsevier.
  5. Cristian Navarrete-Dechent, M. D. (2019, March 1). Imatinib treatment for locally advanced or metastatic dermatofibrosarcoma protuberans. JAMA Dermatology. Retrieved August 11, 2022, from https://jamanetwork.com/journals/jamadermatology/article-abstract/2719525
  6. Raman K Madan, M. D. (2021, July 15). Dermatofibrosarcoma protuberans. Background, Pathophysiology, Etiology. Retrieved August 11, 2022, from https://emedicine.medscape.com/article/1100203-overview
  7. Ugurel, S., Mentzel, T., Utikal, J., Helmbold, P., Mohr, P., Pföhler, C., Schiller, M., Hauschild, A., Hein, R., Kämpgen, E., Kellner, I., Leverkus, M., Becker, J. C., Ströbel, P., & Schadendorf, D. (2014, January 16). Neoadjuvant imatinib in advanced primary or locally recurrent dermatofibrosarcoma protuberans: A multicenter phase II decog trial with long-term follow-up. American Association for Cancer Research. Retrieved August 15, 2022, from https://aacrjournals.org/clincancerres/article/20/2/499/78307/Neoadjuvant-Imatinib-in-Advanced-Primary-or
  8. Bichakjian, C. K., Olencki, T., Alam, M., Andersen, J. S., Berg, D., Bowen, G. M., Cheney, R. T., Daniels, G. A., Glass, L. F., Grekin, R. C., Grossman, K., Ho, A. L., Lewis, K. D., Lydiatt, D. D., Morrison, W. H., Nehal, K. S., Nelson, K. C., Nghiem, P., Perlis, C. S., … Ho, M. (2014, June 1).Dermatofibrosarcoma protuberans, version 1.2014. JNCCN. Retrieved January 9, 2023, from https://jnccn.org/view/journals/jnccn/12/6/article-p863.xml