Idiopathic calcinosis cutis of the vulva.

BACKGROUND: In the present study, calcinosis cutis (CC) is defined as the deposition of amorphous calcium and phosphate salts under epidermis and it may be caused by a pre-existing event such as extravasation injury or hypercalcemic conditions. Idiopathic CC cases have no underlying disease or pre-existing cause. AIM: A demostrative vulvar idiopathic CC case presentation and review of the related literature.
METHODS: A 42-year-old multiparous female presented with vulvar nodular masses. She was keen on surgical removal of the lesions, as the masses caused dyscomfort during sexual intercourse. The lesions were removed and sent for histopathological examination. There was neither a history of trauma nor any inflammatory process in the vulvar skin prior to the development of lesions and no systemic abnormality was detected. RESULTS AND
CONCLUSIONS: The histhopathologic evaluation of the biopsy specimen showed amorphous calcium deposits without any inflammatory infiltration in the dermis. There was no recurrence at 1 year's follow-up. This case shows that idiopathic CC may develop slowly at labio-vulvar region in a sexually active female with normal systemic or laboratory findings.

What was known?

ICC comprises tumoral calcinosis, subepidermal calcified nodules, and scrotal or vulvar calcinosis. The calcification is most commonly localized in one general area, widespread calcinosis has been reported in the absence of tissue injury or detectable disease in otherwise healthy individuals.

Introduction

Calcinosis cutis (CC) is defined as the deposition of insoluble calcium salts in cutaneous tissues.[1] Idiopathic CC is caused by no underlying disease.[2] CC includes, the following forms, metastatic, dystrophic, idiopathic, and iatrogenic calcinosis. In cases of iatrogenic CC, there is a pre-existing cause such as extravasation injury. Tumoral CC is a special form of ICC characterized by large periarticular deposits of calcium, mainly found around the hip, knee, shoulder, and elbow joints. Primary normophosphatemic or hyperphosphatemic tumoral calcinosis shows no evidence of disorders associated with soft-tissue calcification, although, secondary tumoral CC cases have a concurrent disease capable of dermal calcification. Another soft-tissue calcification disorder is calciphylaxis or calcifying pannucilitis, which is defined as small vessel calcification mainly affecting blood vessels of dermis and sub-cutaneous fat. Obesity, liver disease and high-serum calcium (Ca) × parathormone (P) levels, combined therapies of calcium salts with vitamin D and corticosteroids have been observed to increase the likelihood of this disease. Idiopathic CC of the vulva is very rare, we present case of idiopathic CC of the vulva.

Case Report

The patient was a 42-year-old female with two children. On physical examination, there were firm, non-tender, pedunculated nodules measuring approximately 1 cm × 6 cm alongside both vulva without any skin ulceration. It had slowly grown in size during the last 5 years and then remained static. The laboratory investigations revealed within normal serum calcium levels of 9.5 mmol/dL, magnesium levels of 2.4 mg/dL and phosphorus levels of 2.9 mmol/dL. Complete hemogram showed following results; Hb 13.5 g/dL, WBC 5.2 10 3/mL, PLT 270 10 3/mL, and sedimentation rate 25 mm. Blood sugar, uric acid, electrolyte, and liver function and kidney function tests, and routine urine examination were normal. Anti- nuclear cytoplasmic antibodies, serum C3, C4, and CH50 levels, and rheumatoid arthritis test were within normal limits. C-reactive protien (CRP) and antistreptolysine O (ASO) levels were 0.6 mg/dL and 221, respectively. Parathormone level was 63.7 pg/mL. The enyzme linked isosorbent assay (ELISA) tests for anti-human immune deficiency viruse (HIV), anti-hepatit C virıse (HCV), and hepatit B surface antigen (HBSAG) were negative.

It caused discomfort during intercourse as the solid nodular masses mobile. The masses were excised under general anesthesia and there was no recurrence at 1-year follow-up [Figures 1 and 2]. Skin biopsy specimen demonstrated dermal calcification with a diagnosis of idiopathic calcification [Figures 3 and 4]. Any special staining except H and E, was not applied.

Figure 1

Pre-operative view of the vulvar nodular firm masses on both side is seen

Figure 2

Late follow-up, the scar of the previous surgery and it is remarkeble that no recurrence present

Figure 3

Low-power view showing hypekeratozis and pseudoepitheliomatous hyperplasia of the epidermis with calcium deposits in the dermis, there are extruded calcium deposits also in the keratin layer (H and E, ×40)

Figure 4

Calcium deposits within the lesion are amorphous without any inflammatory infiltration (H and E, ×100)

Discussion

Distinct anatomical areas, vulva, scrotum, penis, neck, and breast have been reported to develop idiopathic CC.[3] There are cases with labial lesions at adolescent onset in the literature.[4] In all cases, the main observation is absence of any abnormality in phosphorus or calcium metabolism and to make a diagnosis of idiopathic CC, one should also work-up to rule out collagen vascular disease, as dystrophic CC is caused usually by connective tissue disease. This type of calcinosis is common in dermatomyositis, scleroderma and even it can be seen in lupus erythematosus.[56] Dystrophic CC may also occur following to skin damage. There may be numerous large deposits of calcium or only a few foci. One hypothesis suggests that cutaneous calcification may preferentially ocur at anatomical sites where tissue integrity has been compromised or that contain damaged elastic fibers.[7] The onset of occurence of the mass may be in childhood or adulthood. Nodular sub-cutaneous forms of CC with congenital origin has been reported.[8] This type of nodular idiopathic CC was also seen at infrapatellar region in an athlete of marathon runner, which minds that the occurrence of CC may be related with chronic repeated physical activity.[9]

When reviewing the literature, it can be seen that the definition of idopathic CC with several different forms and association with different clinical statements makes it ill-defined clinical entity. Strong evidences is needed for a clear description of ethiopathologic mechanism. An interesting clinical form of idiopathic CC is milia-like form of which two thirds of the reported cases have been associated with Down syndrome.[10] In particular, this case shows that idiopathic CC may develop slowly at labio-vulvar region in a sexually active female with normal systemic or labarotory findings. Additionally, we did not find out any remarkable event in her past medical history.

At now, histopathological detection of large calcium deposits within sub-cutaneous tissue with no other pathology is enough to make the diagnosis of CC. Histhopathologic findings include calcific deposits beneath epidermis without any inflammatory infiltration. There may be histopathologically vascular changes lead to fibrosis, intimal hyperplasia, and occasionally thrombosis of the vessel in severe cases of calciphlaxis. Von Kossa and Alizain stain is used to confirm the presence of calcium encrusting degenerated collagen bundles throughout the reticular dermis. In early reports, some authors used immunohistochemical stain to demonstrate whether there was an increased number of mast cells in the infiltrate. Now, it is clear that mast cells have no role in the development of the lesions. H and E, stain is usually enough to demonstrate calcific deposits within the dermis. Idiopathic CC is caused by no underlying disease and a diagnosis of exclusion. Its chemical composition has recently been evaluated by vibrational micro-spectroscopy, and according to the results of this study, type B carbonated apotite, and beta caroten interspersing sub-cutaneous tissue present in CC.[11] Many case reports of idiopathic CC of penis, scrotum and vulva have been reported worldwide.[1213] Although, surgical excision is the main choice of treatment, electrodessication or CO2 laser ablation may be performed for small, multiple lesions.[14] Recurrence is uncommon following the current treatment modalities. Colchicine, warfarin, bisphosphonates, probenecid, and diltiazem have been used for treatment of dystrophic form with varying degrees of success.[15]

What is new?

Idiopathic vulvar calcinosis may develop slowly in a sexually active patient and may need surgical removal as it may cause discomfort during intercourse.