Successful treatment of areolar Fox‐Fordyce disease with surgical excision and 1550‐nm fractionated erbium glass laser

Abstract

Fox‐Fordyce disease (FFD) is a rare chronic disorder characterised by persistent inflammation because of the obstruction of apocrine sweat glands, which is a key factor of pathogenesis. The treatment of FFD is known to be difficult, and the modalities of treatment have not yet been widely studied. We report the successful treatment of a case of bilateral areolar FFD by a combination of surgical excision and 1550‐nm fractionated erbium glass laser in an 18‐year‐old woman. The patient presented with a bilateral areolar eruption of multiple, severely pruritic, 3–4 mm skin‐ to grey‐coloured folliculocentric dome‐shaped papules. The initial treatment plan was for bilateral surgical excision of the larger and more highly elevated papules via circumferential dermal excision, which was intended to maintain the areolar contour and minimise distortion. A 1550‐nm fractional erbium glass laser was then used to control the remnant lesions. The patient was recurrence‐free at 14 months after the final laser treatment, and she was fully satisfied with the treatment results.

Introduction

Fox‐Fordyce disease (FFD), also known as apocrine miliaria, is a rare chronic inflammatory disorder. The obstruction of apocrine sweat glands is a key factor in the pathogenesis of FFD 1, which presents in cutaneous areas that normally bear apocrine glands, including the axilla, the areola, the periumbilical area, the breast and the perianal and genital regions.

Because the exact pathogenesis of FFD remains unclear, treatment is known to be difficult and, to date, has generally not been successful 2. In this report, we describe a case of areolar FFD that had an excellent outcome after treatment by surgical excision and 1550‐nm fractionated erbium glass laser.

Case report

An 18‐year‐old woman presented to our hospital with multiple bilateral 3–4 mm skin‐ to grey‐coloured folliculocentric dome‐shaped areolar papules (Figure 1). The lesions were severely pruritic, and the patient had aesthetic and functional concerns related to the eruption. Lesions were also present in both axillae, but the degree of severity was much less. No other regions were involved. The symptoms had been present for 5 years and were aggravated in the summer. The patient was otherwise healthy and without comorbidity.

Figure 1.

An 18‐year‐old woman presented with multiple 3–4 mm skin‐ to grey‐coloured folliculocentric dome‐shaped papules on both areolas.

The initial treatment plan involved bilateral surgical excision under local anaesthesia with the largest and most elevated papules as the main targets. Circumferential dermal excisions were planned, and in order to maintain the areolar contour and minimise distortion, we ensured that the range of excision did not exceed half of the width of the areola (Figure 2A). The excision was performed based on this surgical design with resection of the full dermal layer to prevent recurrence, taking special precaution to avoid injuring the glandular tissue of the breasts (Figure 2B, C). The skin was closed with #5‐0 nylon sutures (Figure 2D).

Figure 2.

(A) Preoperative surgical design; (B) clinical photo after surgical excision; (C) clinical photo after skin closure. The original areola shape is well maintained. (D) The specimen after excision.

Histopathological analysis of the surgical specimens revealed infundibular dilatation and hyperkeratosis consistent with FFD (Figure 3). After excision of the larger lesions, a 1550‐nm fractional erbium glass laser (MOSAIC; Lutronic Corporation, Seoul, South Korea) was used for the ablation of the remnant lesions. Three laser treatments were given at 1‐month intervals beginning one month after the surgery. Laser was applied by two modes: two passes were performed in stamping mode at 50 mJ followed by one pass in moving mode at 25 mJ.

Figure 3.

Histopathological findings. (A) Dilatation of follicular infundibulum (Haematoxylin and Eosin [H and E] stain, ×100) with (B) hyperkeratosis (H and E stain, ×200).

A hypertrophic scar remained at the site of the surgical wound at 14 months after the last laser treatment, but the patient was free of newly developed papules, pruritus and other signs and symptoms of relapse, and she was fully satisfied with the treatment results (Figure 4).

Figure 4.

At 14 months after the last laser treatment, the initial areolar contour is well preserved, and no new lesions are present.

Discussion

The treatment of FFD is known to be difficult, and the treatment modalities have not yet been widely studied because of the rarity of the disease. Treatment methods including topical steroids, calcineurin inhibitors, retinoids, clindamycin, oral contraceptives and oral isotretinoin have been reported, but success of each of these has been limited because the lesions tend to recur once treatment is stopped, and all of these methods have been associated with high recurrence rates 3.

The use of fractional CO₂ lasers in the treatment of FFD has also been described, but only short‐term results have been reported; the long‐term outcome was not evaluated because the patient was lost to follow‐up 1. However, in the final analysis, laser treatment was considered fairly effective and safe, and there were minimal complications.

Surgical treatment has generally been considered a last option for FFD because of the potential for complications and scarring. It can be difficult to decide whether surgical excision is indicated, especially in cases affecting the areola. Unlike in other regions, such as the axilla, which is the most commonly involved, excision of lesions in the areola may lead to the serious distortion of the areolar contour 2. Extreme caution is needed in these cases as the excision of the whole lesion may cause marked areolar distortion or even loss of the entire areola. With this in mind, we designed a combination therapy of surgical excision of the largest lesions followed by laser ablation of remnant, less severe lesions. The final result was good.

So far, a fractional CO₂ laser has been the only laser modality reported in the treatment of FFD 1. We selected a newer laser modality, 1550‐nm fractionated erbium glass laser, based on the utility of this device for the treatment of active acne vulgaris 4. The 1550‐nm infrared erbium glass laser effectively infiltrates the deep skin layer, where the target sweat glands exist, and causes thermal injury to the glands 5, 6. On the basis of these principles, the disease was improved as residual lesions after partial excision were affected with adequate thermal injury to the associated apocrine gland. As our method, minimal invasive surgery combining laser treatment, which results in minimal wound but leads to the best result, has been used widely for other dermatological diseases 7, 8.

We did not perform a post‐laser therapy pathological evaluation in the present case, but histopathological findings showing decrease and shrinkage of associated glands and reduction of secretions after infrared erbium glass 1550‐nm laser treatment have been previously reported 4, 9.

As mentioned above, conventional medical therapies for FFD have had only a marginal effect. Surgical excision can be curative, with no recurrence, but both the patient and the surgeon may hesitate to choose surgery because of concerns about hypertrophic scarring and other potential surgical complications, especially in cases such as the one reported here, where there was a risk of marked areolar distortion. However, using the two principles described herein – firstly, that only the most obvious lesions should be excised and secondly, that the excision should be circumferential – areolar FFD can be treated by surgical excision with excellent results and without severe deformity of the areola.

In summary, to minimise surgical complications and the risk of recurrence of areolar FFD, a combination of infrared erbium glass 1550‐nm laser treatment for less severe lesions and surgical excision of major lesions can be a safe and highly effective treatment modality.