Subepidermal calcified nodule in upper eyelid: A case report and review of the literature.

Calcinosis cutis involves the inappropriate deposition of calcium within the dermis layer of the skin and is often associated with autoimmune diseases. A 3-year-old healthy Omani child presented for evaluation of asymptomatic hard nodule on the left upper eyelid. Pathological examination identified the mass as subepidermal calcified nodule. The patient had no history of trauma or metabolic disturbances. Serum levels of calcium and phosphate were normal. Idiopathic calcinosis cutis should be included in the differential diagnosis for eye lid mass.

Introduction

Calcinosis cutis is a disorder characterized by the deposition of insoluble amorphous calcium salts under the epidermal layer. Classically, the syndrome is separated into five subtypes: Dystrophic calcification, metastatic calcification, idiopathic calcification, iatrogenic calcification, and calciphylaxis.[12]

Case Report

A 3-year-old male Omani child presented for evaluation of asymptomatic left upper eyelid nodule. We got a written approval from the ethics review board of the research committee in North Banta Governorate for this work. Clinical examination was remarkable for solitary nodule at the medial aspect of left upper eyelid measured 6 × 4 mm with slightly verrucous surface and hard consistency. No history of trauma, metabolic disturbances, or systemic diseases was reported. Histopathological examination of the nodule showed hyperplastic epidermis and upper dermal well-circumscribed focally cystic lesion [Figure 1]. It is formed of homogenous basophilic deposits variable in shape and size ranging from large amorphous deposits, small spherules to minute fine granules [Figures 2–4] that consistent with subepidermal calcified nodule (SCN) as a variant calcinosis cutis. This diagnosis is confirmed by Von kossa stain that identifies the calcium in tissues as black deposits within pink background [Figure 5]. This lesion surrounded by chronic inflammatory cells and foreign body giant cells. No pilomatrixoma or preexisting nevus was detected. Laboratory tests revealed normal values of calcium, phosphate, parathyroid hormones, and kidney function tests and 1,25 dihydroxy vitamin D. Therefore, the final diagnosis is SCN, a variant of idiopathic calcinosis cutis.

Figure 1

Hyperplastic epidermis and upper dermal well-circumscribed cystic lesion formed entirely of homogenous basophilic deposits (H and E, ×100)

Figure 2

(a) Variable shaped and sized large amorphous basophilic deposits (H and E, ×200). (b) Closer view of calcification at dermis and epidermis (H and E, ×400)

Figure 4

Calcifications ranged from large amorphous deposits (black arrows), well-formed small spherules (green circles) to small fine granules (brown arrows) (H and E, ×400)

Figure 5

(a) Black calcium deposits with three forms of calcification, (b) Predominance of large amorphous calcium, (c) and the predominance of minute fine granules (Von kossa stain, ×400)

(a) Large amorphous basophilic deposits, (b) Myriads of minute fine basophilic granules (H and E, ×400)

Discussion

Calcinosis cutis is a disorder characterized by the deposition of insoluble amorphous calcium salts under the epidermal layers. The dystrophic type is observed in damaged or devitalized tissues, while the metastatic type is associated with high serum levels of calcium or phosphate, as can be seen with various systemic diseases.[3] The iatrogenic type of calcinosis cutis is generally associated with calcium-containing medical devices or medications,[4] and the idiopathic type occurs in the absence of tissue injury or detectable disease in otherwise healthy individuals.[3] Calciphylaxis presents with small vessel calcification mainly affecting blood vessels of the dermis or subcutaneous fat.[1] Several cases of idiopathic calcinosis cutis involving the scrotum, penis, vulva, breast, or extremities have been described in association with autoimmune diseases.[567] After exclusion of all causes of pathological calcification, the final diagnosis is SCN. SCN is a rare form of idiopathic calcinosis cutis.[8] It occurs most commonly in children as asymptomatic solitary nodule as our case or multiple.[910] SCN presents as congenital or acquired.[10] Evans et al.[11] reported 21 cases of SCN with male predominance as our case. Most of SCN occur at face and ear.[11] Other locations of SCN were scalp, chin, nose, palm, knee, and finger. Recently, sole is reported as a site of SCN.[91213] Eyelid is a rare site of SCN.[11] To best our knowledge, only five reports of SCN on eyelid were reported in the literature; three of them in healthy adult females,[31415] one in healthy adult male[16] and one in adult female with tumoral calcinosis.[17] Nico and Bergonse reported SCN on the eyelids of two healthy Brazilian female patients.[14] Recently, a Korean report of idiopathic calcinosis cutis at tarsus of upper eyelid was published.[3] A 12-year-old girl experienced progressive enlargement of painless nodules on the right upper eyelid and left lower eyelid over a 1 year duration with no history of trauma or evidence of systemic disease that suggestive of bilateral idiopathic SCN.[15] A 20-year-old healthy male with symmetrical nodules on both upper eyelids enlarging over 3 years.[16] A Caucasian middle age female with multiple eyelid calcifications and biochemical features of tumoral calcinosis.[17]

This is the first case report of a healthy male Omani child presented with SCN on upper eyelid. The peculiar characteristics of our case; it is the 6th report of SCN of the eyelid in the literature; it is the 5th report of SCN of the eyelid in idiopathic calcinosis cases. Finally, it is the 1st report of SCN in healthy male Omani child as our patient is the youngest one among those reported previously in the literature. In addition, the calcifications in the present case varied from minute fine granules, spherules to large amorphous irregular deposits that are not previously reported in one case. The pathogenesis of SCN is unknown. It was first described by Winer in 1952[18] and described it as a sweat gland hamartoma and named by Woods and Kellaway, 1963[19] that proposed two possible mechanisms related to degeneration and calcification of the stroma. Evans et al.[11] proposed that SCN represents calcification following dermal damage of unknown cause. The patient treated by surgical excision of the nodule. We concluded that SCN could be considered in the differential diagnosis of the eyelid mass. Moreover, SCN is a rare diagnosis after exclusion of all causes of pathological calcification. Calcinosis cutis is a ring for thorough clinical, radiological, and biochemistry-related calcium metabolism investigations. We recommended further studies to elicit the histogenesis of SCN and the relationship of duration, location, and both size and shape of calcifications.