0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Observation |

Systemic Sarcoidosis With Unique Vulvar Involvement FREE

Stacey Watkins, BA1; Aimen Ismail, BS1; Kristopher McKay, MD1; Kathleen Beckum, MD1; Vlada Groysman, MD1
[+] Author Affiliations
1Department of Dermatology, University of Alabama, Birmingham
JAMA Dermatol. 2014;150(6):666-667. doi:10.1001/jamadermatol.2013.7204.
Text Size: A A A
Published online

Sarcoidosis is a complex, multisystem disease with an unclear cause.1 Research suggests that the pathogenetic mechanism of sarcoidosis is dysregulation of the immune system in individuals with a genetic predisposition who are subsequently exposed to inciting environmental agents.2

Cutaneous sarcoidosis is often one of the earliest clinical signs of the disease and can be divided into 2 subclasses: specific and nonspecific lesions. Specific lesions are characterized by granulomas identified histologically and include macules, papules, plaques, annular lesions, lupus pernio, infiltration of scars, and subcutaneous nodules.3 Alternatively, nonspecific sarcoid lesions, including erythema nodosum, prurigo, or calcifications, are reactive inflammatory processes.3

REPORT OF A CASE

A woman in her 40s presented with erythematous papules with perinasal and periocular distribution involving both the upper and lower eyelids and an atrophic plaque on the dorsal surface of her neck. The patient complained of vaginal changes including itching, burning, tearing, pain with intercourse, and a painful lesion in the perianal area. Examination revealed an erythematous scaly plaque on the mons pubis with atrophic, white, discolored patches in the vaginal area without evidence of tearing (Figure 1).

Place holder to copy figure label and caption
Figure 1.
The Vaginal Area of the Patient With Cutaneous Sarcoidosis

White, discolored patches are apparent in the posterior fourchette, which is the site from which the second biopsy specimen was taken (arrowhead).

Graphic Jump Location

The patient presented with a 5-week history of productive cough with exertional dyspnea, and her chest radiograph revealed upper-lobe calcified granulomas but no bilateral hilar lymphadenopathy. The patient’s pulmonary function tests revealed no airflow limitations, normal lung volume, and a mild reduction in the diffusing capacity of the lungs for carbon monoxide. The results of blood tests, including complete blood cell count, comprehensive metabolic panel, angiotensin-converting enzyme level, and erythrocyte sedimentation rate, were within normal limits.

Biopsies of the vulva and mons pubis revealed pauci inflammatory nodular granulomas consistent with sarcoidosis. On examination of the epidermis, vaguely psoriasiform epidermal acanthosis with hyperkeratosis was found. Within the dermis, tissue biopsy specimens demonstrated numerous, well-circumscribed nodular granulomas with multinucleated giant cells and lacking significant numbers of neutrophils (Figure 2A). Most of the granulomas were pauci inflammatory, but some had a cuff of lymphoplasmacytic inflammation. Finally, in areas of the epidermis there was transepidermal elimination (TEE) of the granulomas, a unique finding in the vulvar area of a patient with sarcoidosis (Figure 2B). All specimens tested negative for microorganisms by Grocott-Gomori methenamine silver and acid-fast bacilli stains and were not consistent with lichen sclerosus or lichen simplex chronicus. Hydroxychloroquine therapy had previously failed for this patient, and she was therefore prescribed mycophenolate (500 mg, twice a day) by her pulmonologist. In addition, we recommended 3-times-daily application of topical clobetasol ointment and nightly hydrocortisone suppositories.

Place holder to copy figure label and caption
Figure 2.
Pathologic Specimens of Vulva in Patient With Cutaneous Sarcoidosis

A, Numerous, well-formed hypoinflammatory granulomas in the dermis (hematoxylin-eosin, original magnification ×40). B, Transepidermal elimination of granulomas in areas of epidermal acanthosis (hematoxylin-eosin, original magnification ×100).

Graphic Jump Location

DISCUSSION

Although it is rare, sarcoidosis may occur in other regions of the female genital tract, including the uterus, ovaries, fallopian tubes, cervix, placenta, and vaginal wall.4 The differential diagnosis of granulomatous disease of the vulva includes tuberculosis, Crohn disease, syphilis, foreign body reactions, and lymphogranuloma venereum.5

Our case demonstrates TEE, a histologic phenomenon that, to our knowledge, has never been reported in a case of vulvar sarcoidosis. During TEE, the epidermis or the follicular epithelium undergoes a process of pseudohyperplasia to encompass the targeted material, which is eventually eliminated by maturing keratinocytes.6 Importantly, the epithelium does not undergo major structural remodeling and returns to normal after the targeted material is successfully removed.6 In the dermis of our patient’s vulva, the granulomas were the targeted material to be removed.

In summary, our patient presented with sarcoidosis of the vulva with histologic evidence of TEE. This case highlights that sarcoidosis can have an uncommon presentation, warranting extensive physical examination of patients presenting with possible sarcoidosis.

ARTICLE INFORMATION

Corresponding Author: Vlada Groysman, MD, Department of Dermatology, University of Alabama at Birmingham, 1530 Third Ave S, EFH 414, Birmingham, AL 35294 (VGroysman@cahabaderm.com).

Published Online: March 5, 2014. doi:10.1001/jamadermatol.2013.7204.

Conflict of Interest Disclosures: None reported.

REFERENCES

Iannuzzi  MC, Rybicki  BA, Teirstein  AS.  Sarcoidosis. N Engl J Med. 2007;357(21):2153-2165.
PubMed   |  Link to Article
Baughman  RP, Lower  EE, du Bois  RM.  Sarcoidosis. Lancet. 2003;361(9363):1111-1118.
PubMed   |  Link to Article
Cardoso  JC, Cravo  M, Reis  JP, Tellechea  O.  Cutaneous sarcoidosis: a histopathological study. J Eur Acad Dermatol Venereol. 2009;23(6):678-682.
PubMed   |  Link to Article
Klein  PA, Appel  J, Callen  JP.  Sarcoidosis of the vulva: a rare cutaneous manifestation. J Am Acad Dermatol. 1998;39(2 Pt 1):281-283.
PubMed   |  Link to Article
Ezughah  FI, Ghaly  AF, Evans  A, Green  CM.  Vulval sarcoid: a systemic presentation of sarcoidosis. J Obstet Gynaecol. 2005;25(7):730-732.
PubMed   |  Link to Article
Batres  E, Klima  M, Tschen  J.  Transepithelial elimination in cutaneous sarcoidosis. J Cutan Pathol. 1982;9(1):50-54.
PubMed   |  Link to Article

Figures

Place holder to copy figure label and caption
Figure 1.
The Vaginal Area of the Patient With Cutaneous Sarcoidosis

White, discolored patches are apparent in the posterior fourchette, which is the site from which the second biopsy specimen was taken (arrowhead).

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.
Pathologic Specimens of Vulva in Patient With Cutaneous Sarcoidosis

A, Numerous, well-formed hypoinflammatory granulomas in the dermis (hematoxylin-eosin, original magnification ×40). B, Transepidermal elimination of granulomas in areas of epidermal acanthosis (hematoxylin-eosin, original magnification ×100).

Graphic Jump Location

Tables

References

Iannuzzi  MC, Rybicki  BA, Teirstein  AS.  Sarcoidosis. N Engl J Med. 2007;357(21):2153-2165.
PubMed   |  Link to Article
Baughman  RP, Lower  EE, du Bois  RM.  Sarcoidosis. Lancet. 2003;361(9363):1111-1118.
PubMed   |  Link to Article
Cardoso  JC, Cravo  M, Reis  JP, Tellechea  O.  Cutaneous sarcoidosis: a histopathological study. J Eur Acad Dermatol Venereol. 2009;23(6):678-682.
PubMed   |  Link to Article
Klein  PA, Appel  J, Callen  JP.  Sarcoidosis of the vulva: a rare cutaneous manifestation. J Am Acad Dermatol. 1998;39(2 Pt 1):281-283.
PubMed   |  Link to Article
Ezughah  FI, Ghaly  AF, Evans  A, Green  CM.  Vulval sarcoid: a systemic presentation of sarcoidosis. J Obstet Gynaecol. 2005;25(7):730-732.
PubMed   |  Link to Article
Batres  E, Klima  M, Tschen  J.  Transepithelial elimination in cutaneous sarcoidosis. J Cutan Pathol. 1982;9(1):50-54.
PubMed   |  Link to Article

Correspondence

CME
Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

391 Views
1 Citations

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections
PubMed Articles
Jobs
×