Multiple eccrine hidrocystomas in a patient of sarcoidosis.

A 45-year-old female patient known case of sarcoidosis presented in skin department with chief complaint of skin-colored papulonodular lesions over face with exacerbation in summer season since last 4 years. On examination lesions were multiple, firm and skin colored. Biopsy was done showing features of eccrine hidrocystoma. Patient was treated with electrocautery and showed improvement.

What was known?

Both Sarcoidosis and EH are known to occur on face and sometimes in a patient of sarcoidosis with papular shiny lesions on the face we generally take them to be lesions of sarcoid and are managed on the same lines.

Introduction

Hidrocystomas are rare, benign, cystic lesions of the skin - can be either eccrine or apocrine, and are often found on the head and neck region. Eccrine hidrocystomas are small and tense thin-walled cysts, ranging from 1 to 6 mm in diameter, and can occur as single or multiple lesions.[1] They are found predominantly in adult females and are located mostly on the periorbital and malar regions.

Apocrine hidrocystomas arise from the proliferation of apocrine glands and are usually solitary, with a diameter of 3-15 mm.[1] Apocrine lesions are also found mostly on the head and neck and along the eyelid margin near the inner canthus.[2]

Eccrine hidrocystomas are prevalent in adults between 30 and 70 years of age.[1] Solitary eccrine hidrocystomas are equally prevalent among males and females; however, the multiple eccrine hidrocystomas are mainly seen in female patients.[3] We report a case of multiple hidrocystoma on face in a patient of sarcoidosis which was previously misdiagnosed as cutaneous sarcoidosis.

Case Report

A 45-year-old female patient was referred to us for evaluation of multiple, papulonodular lesions over malar area of face which were present since last 4 years. Patient had history of exacerbation of lesions in summer season. Lesions were itchy but painless. Patient was a known case of sarcoidosis with interstitial lung disease. She was on regular treatment with rheumatologist and was on methotrexate and hydrxychloroquin for the last 7 years. She had also received courses of systemic steroids intermittently for exacerbations. She was being given topical steroids for cutaneous lesions as the lesions were misdiagnosed as cutaneous sarcoidosis. On examination, multiple, tense, skin colored, firm papulonodular lesions were seen over malar area of face in a symmetrical fashion. Size of the lesions ranged from 5 mm to 1cm in diameter [Figure 1]. Biopsy was done from one of these lesions which showed single cystic cavity lined by flattened epithelial cells. Overlying epidermis was thinned and eccrine tubules and ducts were seen close to cavity consistent with a diagnosis of eccrine hidrocystoma. [Figures 2a and b] Patient was treated with electrocautery. Patient showed improvement in the lesions and she was relieved that these are benign lesions and are not due to sarcoidosis as she was afraid she might have to take systemic steroids again. She did not come for further treatment following this.

Figure 1

The grouped skin-colored dome-shaped papules on the malar area and nose

Figure 2

(a) A dilated cystic structure in the middle dermis is shown (H and E, ×40), and (b) the cystic wall is lined by layers of cuboidal and flattened epithelial cells without decapitation (H and E, ×200)

Discussion

Eccrine hidrocystomas are benign tumors of sweat gland origin and are classified into two major groups: the Smith type, which is the most prevalent solitary type, and the Robinson, or multiple, type. They are typically dome-shaped, have an amber, brown, or bluish tint, and range from 1 to 6 mm in diameter. During hot or humid weather, these lesions will grow in size and/or multiply in number. Eccrine hidrocystomas usually do not involve the eyelid margin but rather are distributed around the eyelid skin; this, along with a lighter color presentation, typically differentiates it from the apocrine type.[1]

The clinical presentation of apocrine hidrocystomas is very similar to that described above. They appear as dome-shaped, solitary, clear, cystic nodules with a smooth surface, and the color varies, ranging from flesh-colored to blue-black. Although the solitary hidrocystoma is the most common clinical presentation, multiple lesions on the face and neck have been reported.[4] The cysts tend to stay asymptomatic and will follow a gradual course until a certain size is reached. There is no evidence of seasonal variations associated with apocrine hidrocystoma, whereas patients tend to present with eccrine hidrocystomas mostly during hot, humid weather.[5] Under the microscope, eccrine hidrocystomas appear as unilocular cysts, which usually contain a single cystic cavity composed of one or two layers of cuboidal cells. They are located within the mid-dermal to superficial layers of the skin, especially around the eyes. Unlike their apocrine counterpart, there are no secretory cells seen and decapitation of cells is not viewed under the microscope.[1] Histochemically, they stain positive for S100 protein (solitary type), whereas apocrine hidrocystomas do not. The cyst and epidermis do not communicate, and the periodic acid-Schiff (PAS)-positive granules are not observed.[1]

Apocrine hidrocystomas appear as unilocular or multilocular under the microscope. The cyst wall is composed of an inner layer of secretory columnar epithelium.

The etiology of multiple EH remains unknown. A suggested cause for EH involves occlusion of the intradermal portion of the eccrine duct. However, Murayama et al. suggest that EH is a hamartoma-like disorder that becomes more pronounced as a result of retaining sweat. We thought that the latter hypothesis might explain the atypical presentation of our case.

Although a solitary EH can be easily treated with surgical excision, eliminating multiple hidrocystomas is difficult because of their number and cosmetic location.[4] The therapeutic options can be divided into medical and surgical modalities. Multiple lesions have been treated with topical scopolamine, topical atropine and botulinum toxin type A.[56] Simple needle puncture has not been shown to produce lasting effects. Using a 585-nm pulsed dye laser has resulted in good outcomes when treating multiple EHs.[7] Most importantly, avoiding hot temperatures or humid conditions will help prevent worsening of symptoms in patients. In our case, due to nonavailability of topical preparations and poor socioeconomic status, we chose electrodessication of the lesions. Gupta and colleagues[8] reported the use of electrodessication to be successful in the treatment of multiple apocrine tumors of less than 1 cm in diameter.

There have been no reported cases of EH in patients of sarcoidosis and there is no causal relationship between the two; in our case too the association is coincidental rather than causal. We reported this case to stress on the importance of biopsy in facial lesions in case of sarcoidosis because in our case the lesions were being treated as sarcoidosis and the patient was about to be started on systemic steroids as she was not responding to topical steroids. The patient was more concerned about restarting systemic steroids rater than the lesions per se as she knew the lesions would improve in winters and was relieved of her worry after knowing the diagnosis.

What is new?

1. There have been no reported cases of EH in patients of sarcoidosis and there is no causal relationship between the two; in our case too the association is coincidental rather than causal.

2. We want to stress on the importance of biopsy in the lesions on face because many a times both the patient and the dermatologist are hesitant in biopsying a facial lesion.