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Symptomatic hypercalcemia and scarring alopecia as presenting features of sarcoidosis.

Sarcoidosis is a multisystemic granulomatous disease with a peak onset between the ages of 20 and 40 years. It is slightly more common in women, and the incidence is especially high in African Americans (40 to 70 per 100,000 population per year) compared to Caucasians (5 to 19 per 100,000). (1) Though cutaneous involvement is a common extrathoracic manifestation (20%-35%), sarcoidosis of the scalp is a rare clinical entity. (2) Aberrant calcium metabolism is also associated with sarcoidosis and other granulomatous diseases, presenting most commonly with hypercalciuria. Symptomatic hypercalcemia is seen in a small proportion of cases (approximately 5%) and rarely causes kidney damage. (3,4) We report an unusual case of sarcoidosis presenting with symptomatic hypercalcemia, scarring alopecia, and acute-on-chronic kidney failure.

CASE REPORT

A 60-year-old black woman with hypertension, chronic bronchitis, intellectual impairment, and schizophrenia presented to the emergency department for motor weakness and altered mental status. The patient's caregiver reported a worsening nonproductive cough, confusion, and progressive muscle weakness preventing the patient from getting out of bed. There was no fever, chills, night sweats, hemoptysis, sick contacts, recent travel, or chronic kidney disease. Her medications included quetiapine, citalopram, bupropion, hydrochlorothiazide, lisinopril, and clonidine.

Physical examination was significant for a large (20-cm) scaly plaque with central depression and scarring alopecia on the right parietotemporal scalp, plus a smaller 4-cm scaly plaque with associated alopecia on the left frontal scalp (present for >15 years) (Figure 1). Bilateral wheezing and crackles over the right lung were also noted. Bacterial, fungal, and sputum cultures for tuberculosis were negative. Serum and protein electrophoresis demonstrated an M spike with subsequently normal kappa and lambda light chains and immunofixation panel. Other laboratory results are shown in Table 1. Computed tomography of the chest revealed pulmonary nodules, calcified bilateral hilar and mediastinal lymph nodes, and tracheobronchial stenosis.

The patient was started on intravenous fluids (0.9% normal saline) and calcitonin (200 units daily). Nephrotoxic agents (e.g., hydrochlorothiazide and lisinopril) were withheld, and the patient was admitted to the medicine floor. Subsequent bronchoscopy with transbronchial biopsy showed multinucleated giant cells associated with laminated concentric calcification (Schaumann bodies). Biopsy of the large scalp lesion demonstrated dermal granulomatous inflammation without caseation (Figure 2). Fungal and acid-fast stains were negative, and no polarizable foreign material was present. Renal ultrasound showed bilateral renal cortical echogenicity without hydronephrosis.

The diagnosis of sarcoidosis was confirmed. The patient was managed with Solu-Medrol (40 mg intravenously every 8 hours) along with continued gentle hydration plus calcitonin (200 units daily). Over the 11-day hospital course, her serum calcium improved to 10.6 mg/dL (2.64 mmol/L) and creatinine to 2.48 mg/dL (219.23 [micro]mol/L) at discharge. The patient was discharged on a tapering dose of prednisone initiated at 60 mg. She was referred to pulmonology and her primary care physician for follow-up.

DISCUSSION

Sarcoidosis of the scalp can present with variable morphologies, including scarring and nonscarring alopecia, indurated plaques, and nodules. Areas of hair loss can be localized or diffuse with associated infiltrated borders, atrophy, erythema, and scaling. The differential diagnosis includes lichen planopilaris, discoid lupus erythematosus, and central centrifugal cicatricial alopecia. The diagnosis is best supported by the presence of noncaseating granulomas on biopsy with appropriate clinical and radiologic findings. (5) The prevalence of scalp lesions appears to be highest in African American women. Concomitant systemic disease, especially of the lungs and lymph nodes, is common. (6)

Aberrant calcium metabolism is related to sarcoidosis and other granulomatous disorders as a result of increased levels of active 1,25-dihydroxy vitamin D (OHD). Hypercalcemia is reported in 10% to 20% of patients with sarcoidosis and is generally asymptomatic. (7) Clinically significant calcium elevations are much less frequent (5%). (8) Hypercalcemia can occur in the setting of inappropriatel y normal OHD concentrations. An appropriately suppressed parathyroid hormone level and inappropriately normal OHD level in our patient suggest extrarenal production of OHD, likely from activated macrophages in the lungs and lymph nodes. Hypercalcemia was likely exacerbated by her dehydration, thiazide diuretics, and renal insufficiency.

Longstanding hypercalcemia may lead to nephrocalcinosis and nephrolithiasis and, less frequently, tubular necrosis, tubulointerstitial nongranulomatous inflammation, and renal failure. Up to one-third of patients with renal dysfunction have granulomatous interstitial nephritis. (7) A renal ultrasound of our patient revealed changes consistent with kidney disease involving primarily the renal parenchyma, likely attributed to hypercalcemic renal damage, granulomatous deposition, or simply chronic hypertension. Unfortunately, no renal biopsy was obtained to confirm sarcoidosis of the kidneys.

Severe symptomatic hypercalcemia in association with sarcoidosis requires prompt treatment with hydration and corticosteroids. Both granulomatous interstitial nephritis and renal dysfunction from extrarenal sarcoidosis are also responsive to corticosteroids. Other therapeutic options include ketoconazole, hydroxychloroquine, methotrexate, and azathioprine. (8) Treatment of scalp lesions is difficult, with intralesional and/ or systemic corticosteroids most frequently implemented.

Unique features of this case include scarring alopecia secondary to sarcoidosis and severe and symptomatic hypercalcemia in association with normal urinary calcium excretion, serum angiotensin-converting enzyme, and 1,25-dihydroxy vitamin D levels. Though our patient's hypercalcemia and renal dysfunction were responsive to corticosteroids and hydration, renal function was not restored to normal, likely due to chronic renal damage. Relapses frequently occur, despite an initial response to corticosteroids, requiring lifelong monitoring of disease activity.

FUNDING

Dr. Menter reports grants and honoraria from AbbVie, Allergan, Amgen, Boehringer Ingelheim, Janssen Biotech, Inc., LEO Pharma, Novartis, Pfizer, and Xenoport; grants from Anacor, Celgene, Dermira, Merck, Neothetics, Regeneron, and Symbio/Maruho; and honoraria from Eli-Lily, Galderma, and Vitae, all outside of the submitted work. None of the authors have any conflicts of interest to disclose that are pertinent to this article. https://doi.org/10.1080/08998280.2018.1435118

(1.) Ungprasert P, Crowson CS, Matteson EL. Epidemiology and clinical characteristics of sarcoidosis: an update from a population-based cohort study from Olmsted County, Minnesota. Reumatismo. 2017; 69:16-22.

(2.) Bhushan P, Thatte SS, Singh A. Key messages from a rare case of annular sarcoidosis of scalp. Indian Dermatol Online J. 2016; 7:192-194.

(3.) Khurana A, Cherian S, Majumder S, et al. Retinal detachment and symptomatic hypercalcemia in a patient with sarcoidosis: unusual presentation of a granulomatous disease. Conn Med. 2016; 80:11-14.

(4.) Mahfoudhi M, Mamlouk H, Turki S, et al. Systemic sarcoidosis complicated of acute renal failure: about 12 cases. Pan Afr Med J. 2015; 22:75.

(5.) Fernandez-Faith E, McDonnell J. Cutaneous sarcoidosis: differential diagnosis. Clin Dermatol. 2007; 25:276-287.

(6.) Katta R, Nelson B, Chen D, et al. Sarcoidosis of the scalp: a case series and review of the literature. J Am Acad Dermatol. 2000; 42:690-692.

(7.) Berliner AR, Haas M, Choi MJ. Sarcoidosis: the nephrologist's perspective. Am J Kidney Dis. 2006; 48:856-870.

(8.) Conron M, Young C, Beynon HL. Calcium metabolism in sarcoidosis and its clinical implications. Rheumatology (Oxford). 2000; 39:707-713.

Jillian Frieder, MD, Dario Kivelevitch, MD, and Alan Menter, MD

Division of Dermatology, Baylor University Medical Center, Dallas, Texas

Corresponding author: Jillian Frieder, MD, Division of Dermatology, Baylor University Medical Center, 3900 Junius Street, Suite 125, Dallas, TX 75246 (e-mail: jhfrieder@gmail.com)

Received November 8, 2017; Revised December 4, 2017; Accepted December 5, 2017.

Caption: FIGURE 1. (a) Giant scalp plaque with atrophy, hypopigmentation, and scarring alopecia plus prominent scaling at the borders of the lesion. (b) A smaller crusted plaque with scarring alopecia on the left frontal scalp.

Caption: FIGURE 2. (a) Skin biopsy of the scalp showing sarcoidal granulomas with multinucleated giant cells in the dermis with absence of hair follicles (x20 view). (b) Skin biopsy of the scalp, x40 view of sarcoidal granulomas with multinucleated giant cells in the dermis and absence of hair follicles.
Table 1. The patient's initial laboratory results

Test                                     Result      Normal range

Serum calcium (mg/dL)                     14.5          8.6-10
Serum albumin (g/dL)                       3.3         3.9-5.1
Corrected serum calcium (mg/dL)           15.06         8.6-10
Ionized serum calcium (mg/dL)             6.37        4.64-5.28
24-hour urine calcium (mg/day)            10.4          0-300
Serum creatinine (mg/dL)                  5.98         0.8-1.3
Parathyroid hormone (pg/mL)                11           15-65
Parathyroid hormone-related protein        3.3          14-27
  (pg/mL)
Phosphorus (mg/dL)                         5.1         2.7-4.5
25-Hydroxy vitamin D (ng/mL)              18.7          20-40
1,25-Dihydroxy vitamin D (pg/mL)          19.6          18-72
Angiotensin-converting enzyme (U/L)        12            9-67
Antinuclear antibody                    Negative
Antineutrophil cytoplasmic antibody     Negative
Serum IgG, IgA, IgM                      Normal
HIV antigen and antibody               Nonreactive
CD4:CD8 ratio                              7.4           1-4
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Author:Frieder, Jillian; Kivelevitch, Dario; Menter, Alan
Publication:Baylor University Medical Center Proceedings
Article Type:Clinical report
Date:Apr 1, 2018
Words:1407
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