Management of genital hidradenitis suppurativa and lymphoedema with the restoration of erectile function.

Hidradenitis suppurativa (HS) involving the anogenital and inguinal regions can cause considerable morbidity. Genital lymphoedema is an under‐recognised complication of long‐standing severe HS. Despite the advent of immunomodulatory therapy, surgical management is still required in some situations. We present a case of anogenital HS with associated genital lymphoedema and discuss the multidisciplinary management of this disease.

A 40‐year‐old man developed scrotal and penile lymphoedema after a 20‐year history of treatment‐resistant, anogenital and inguinal, Hurley Stage III HS (Fig. 1a,b). His disease had caused impotence, notwithstanding weekly adalimumab 40 mg with intermittent intralesional steroid injections, doxycycline and a combination of clindamycin and rifampicin for 2 years. Given extensive induration and lymphoedema, he proceeded to surgery without interruption to adalimumab.

Figure 1

Pre‐, and postoperative Images. (a) The extensive mass of inflammation and infection affecting the scrotum, groins and penis preoperatively. (b) 2 weeks postoperatively showing good take of the skin grafts. (c and d) 6 months postoperatively showing mature skin grafts free of disease and a less lymphoedematous penis. [Colour figure can be viewed at wileyonlinelibrary.com]

Circumcision was performed to enable the visualisation of the buried and obscured glans and urethral meatus, necessary to facilitate urinary catheterisation. Excision of the inguinal and scrotal disease began in the upper inguinal regions and progressed to the perineal body, with the use of a preoperative MRI (Fig. 2a) to identify the location of the testicles that were impalpable. Following the excision of the inguinal and scrotal disease (Fig. 2b,c), the defects were reconstructed with fenestrated sheets of split‐thickness skin graft (Fig. 2d) and covered with a vacuum‐assisted closure dressing (Fig. 2e).

Figure 2

(a) Preoperative MRI showing the locations of the impalpable testicles, with measurement from the anterior superior iliac spine (coronal image) and skin surface (axial image). (b) Following excision of the groin, scrotal and foreskin disease, with preservation of the testicles. (c) The excised scrotal/groin tissue weighing 1.2 kg and measuring approximately 30 cm long. (d) Following application of fenestrated sheets of split‐thickness skin grafts from the thigh to the groins and testicles. (e) Site following application of VAC dressing. [Colour figure can be viewed at wileyonlinelibrary.com]

Promptly following the procedure, the patient reported regained erectile function. A marked functional and cosmetic improvement was achieved (Fig. 1c,d), including a reduction in penile lymphoedema and induration. The residual perineal HS lesions resolved over next the 6 weeks, while the surgical wounds were healed by 2 months postoperatively.

Hidradenitis suppurativa significantly impairs patients' quality of life, more so than many other dermatological conditions. Persistent poorly controlled HS results in diffuse scarring, fibrosis and lymphoedema that can impact micturition, social and sexual functioning.

Tumour necrosis factor‐alpha inhibitors, namely adalimumab, have significantly improved the management of severe HS. Preoperative medical optimisation can reduce the burden of active disease, thereby reducing the extent and technical difficulty of surgery, and minimising the risk of complications. However, once lymphoedema becomes severe, further inflammation and infection may result, producing a positive feedback loop. Consequently, HS often requires a combination of medical therapy and surgical intervention to maximally control the disease and restore function and cosmesis. This is particularly true in the setting of genital lymphoedema. Furthermore, there is animal evidence to suggest that the lymphangiogenic properties of 9‐cis retinoic acid may be useful in the management of lymphoedema.

Surgery, in this case, resulted in improved mentation and resolution of the residual anogenital disease. We postulate this was likely due to a reduced chronic inflammatory burden resulting in enhanced biological efficacy of the adalimumab.

Penile lymphoedema is known to often improve with scrotectomy, as occurred in this case, hence it was not excised initially. However, there was likely some fat deposition and fibrosis, which may require further surgery.

This case highlights the need for combined medical and surgical approaches in treating male anogenital HS with lymphoedema. Surgery may improve disease control, sexual function and enhance biological efficacy by lessening the systemic inflammatory burden.