Letters

Figure 2. Pathologic Specimens of Vulva in Patient With Cutaneous Sarcoidosis A

B

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).

(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 applic ation of topic al clobetasol ointment and nightly hydrocortisone suppositories. 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. Stacey Watkins, BA Aimen Ismail, BS Kristopher McKay, MD Kathleen Beckum, MD Vlada Groysman, MD

Author Affiliations: Department of Dermatology, University of Alabama, Birmingham (Watkins, Ismail, McKay, Beckum, Groysman). Corresponding Author: Vlada Groysman, MD, Department of Dermatology, University of Alabama at Birmingham, 1530 Third Ave S, EFH 414, Birmingham, AL 35294 ([email protected]). Published Online: March 5, 2014. doi:10.1001/jamadermatol.2013.7204. Conflict of Interest Disclosures: None reported. 1. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357 (21):2153-2165. 2. Baughman RP, Lower EE, du Bois RM. Sarcoidosis. Lancet. 2003;361(9363): 1111-1118. 3. Cardoso JC, Cravo M, Reis JP, Tellechea O. Cutaneous sarcoidosis: a histopathological study. J Eur Acad Dermatol Venereol. 2009;23(6):678-682. 4. 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. 5. Ezughah FI, Ghaly AF, Evans A, Green CM. Vulval sarcoid: a systemic presentation of sarcoidosis. J Obstet Gynaecol. 2005;25(7):730-732. 6. Batres E, Klima M, Tschen J. Transepithelial elimination in cutaneous sarcoidosis. J Cutan Pathol. 1982;9(1):50-54.

Hypertensive Emergency, Matlike Telangiectasias, and Calciphylaxis in POEMS Syndrome POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a paraneoplastic syndrome caused by plasma cell dyscrasias. We report hypertensive emergency and acral matlike telangiectasias as novel manifestations of POEMS syndrome (POEMS). Also, to our knowledge, this is the first reported case of calciphylaxis and POEMS occurring in the absence of sclerotic bone lesions or a peripheral monoclonal gammopathy, highlighting the diagnostic challenges posed by this disease. Report of a Case | A woman in her 30s with a 2-year history of distal polyneuropathy and matlike telangiectasias presented with a hypertensive emergency and peak blood pressure of 247/154 mm Hg. Workup revealed a serous pericardial effu-

jamadermatology.com

JAMA Dermatology June 2014 Volume 150, Number 6

Copyright 2014 American Medical Association. All rights reserved.

Downloaded From: http://archderm.jamanetwork.com/ by a University of Georgia User on 05/26/2015

667

Letters

sion, hepatosplenomegaly, ascites, shotty lymphadenopathy, and diffuse subcutaneous edema; the remainder of her workup findings were negative, including those for rheumatoid factor, antineutrophil cytoplasmic antibodies, antinuclear antibodies, extractable nuclear antigen antibodies panel, serum and urine protein electrophoresis with immunofixation, and brain magnetic resonance imaging. After blood pressure was brought under control, prednisone treatment was empirically initiated, and the ascites improved. Over the next 4 months, the patient developed hemangiomas, amenorrhea, refractory ascites, hepatosplenomegaly, weight loss, and fatigue. On readmission, she was found to have hypothyroidism, diabetes mellitus, hypogonadism, and papilledema. Significant laboratory findings included thrombocytosis (platelet level peaking at 2045 × 109/ dL) and transient renal insufficiency (creatinine level peaking at 3.04 mg/dL, then quickly decreasing). (To convert creatinine to micromoles per liter, multiply by 88.4.) Parathyroid hormone level was within normal limits, and the level of calcium phosphate product was 43.12 mg/dL (normal,

Hypertensive emergency, matlike telangiectasias, and calciphylaxis in POEMS syndrome.

Hypertensive emergency, matlike telangiectasias, and calciphylaxis in POEMS syndrome. - PDF Download Free
321KB Sizes 0 Downloads 0 Views