Perianal Pseudoverrucous Papules and Nodules in an Elderly Patient

Introduction

Diaper dermatitis encompasses a range of acute, inflammatory reactions that occur as a result of irritant contact dermatitis caused by exposure to feces, urine or both. In uncomplicated cases, the affected areas exhibit diffuse erythema, with occasional presence of micropapules and scaling. Several uncommon clinical patterns, regarded as complications of diaper dermatitis, have also been described: granuloma gluteale infantum/adultorum, Jacquet erosive diaper dermatitis, and perianal pseudoverrucous papules and nodules (PPPN).¹

PPPN is an uncommon manifestation of irritant contact dermatitis that was first reported around urostomy sites.² PPPN consisted of flat-topped, erythematous-brown papules and nodules that develop in the diaper and perianal areas in patients of any age whose skin is chronically exposed to moisture, including the elderly and children with prolonged urinary or fecal incontinence.³ These lesions are relatively rare and occasionally misdiagnosed, which may even result in their surgical removal. Only a limited number of case reports exist documenting the occurrence of PPPN on the perianal skin or in the elderly.^1,4,5

Case

An 84-year-old woman visited the dermatologic department with a 3-month history of multiple verrucous papules and nodules on the perianal area, which were causing discomfort and pain. She had a previous medical history of hypertension and spinal stenosis. The patient was bedridden due to her medical condition. Despite frequent defecation and urination, her caregiver had changed her diapers only twice a day, using wet wipes and povidone. A month ago, the patient was prescribed topical antibiotics at a local clinic with limited improvement and was referred to our clinic under the impression of viral infection. The patient’s laboratory test results were nonspecific, and polymerase chain reaction did not detect human papillomavirus. Treponema pallidum hemagglutination (TPHA) tests were negative/non-reactive. No bacterial or fungal growth was observed on culture. On physical examination, multiple, 3–12 mm sized flat-topped moist verrucous papules and nodules with some lesions coalescing to form plaques were noticed at the perianal area (Figure 1). Punch biopsy specimen revealed prominent hyperkeratosis, parakeratosis, irregular epidermal hyperplasia (Figure 2A) and mild lymphocytic infiltration in the dermis (Figure 2B). Periodic Acid-Schiff with diastase (PAS-D) staining did not reveal any pathogenic organism. The lesions were diagnosed as perianal pseudoverrucous papules and nodules based on the clinical and histopathologic features. The patient was treated with daily saline wet dressings and 0.25% prednicarbate lotion. The diapers were changed on more regular and frequent intervals. After 6 weeks of treatment, the lesions improved significantly (Figure 3).

Figure 1 Multiple eroded hyperpigmented flat-topped papules and nodules with few verrucous papules and nodules on the perianal area.

Figure 2 Histopathologic findings. (A) Prominent hyperkeratosis, parakeratosis and irregular hyperplasia in the epidermis (Hematoxylin and eosin; x 40). (B) Mild lymphocytic infiltration in the dermis (Hematoxylin and eosin; x 100).

Figure 3 The lesions improved after 6 weeks of treatment.

Discussion

Goldberg et al introduced the term PPPN for the first time in 1992.³ PPPN predominately affects children in the diaper area and around surgical ostomies, related to gastrointestinal or urologic diseases.³ PPPN presents as multiple, wart-like erythematous-brown papules and nodules in perianal, genital, suprapubic, buttocks, and peristomal areas.⁶ Histopathological examination of these lesions reveals psoriasiform epidermal hyperplasia with acanthosis, broad hyperparakeratosis, papillary edema, prominent dermal blood vessels, and mild-to-moderate dermal perivascular infiltrate. It is controversial whether PPPN constitutes a distinct form of primary irritant diaper dermatitis¹ or that granuloma gluteale infantum/adultorum, Jacquet’s erosive dermatitis, and PPPN are all part of the same entity coined under the term “erosive papulonodular dermatosis”.⁶ Granuloma gluteale adultorum is usually manifested as reddish-purple, firm, oval, or elongated plaques and nodules in the pubic area, buttocks, scrotum, and medial aspects of the thighs.⁶ This is a different feature from the observed papules and small nodules located in the perianal area of our patient. Moreover, PPPN tends to exhibit a higher number of verrucous lesions compared to granuloma gluteale adultorum.⁷ Compared to PPPN, the histology of granuloma gluteale adultorum is relatively nonspecific. The histopathological features show epidermal hyperplasia and a dense dermal inflammatory cell infiltration, with proliferation of blood vessels and a variable degree of dilation. The term “granuloma” can be misleading because histologically, granulomatous inflammation is not observed in cases of granuloma gluteale adultorum.^6,7 In Jacquet’s erosive diaper dermatitis, well-demarcated, 2 to 5 mm punched-out ulcers or erosions with elevated borders typically occur on the peri-vulvar or penis area.^1,8,9 Biopsy findings usually show a nonspecific inflammatory pattern.^6,7,9 Thus, the term PPPN is preferred in our case because it is more clinically descriptive (Table 1).

Table 1 Characteristic Features in Various Types of Irritant Diaper Dermatitis

PPPN is an uncommon type of irritant contact dermatitis and sometimes can be misdiagnosed. Due to clinical similarities, several dermatoses, infections and neoplasms may be considered in the differential diagnosis including condyloma lata, condyloma acuminata, bacterial infections, candidiasis, scabietic nodules, anogenital warts, Langerhans cell histiocytosis and cutaneous Crohn’s disease.^3,5,10 The sexual history, rapid plasma reagin (RPR), venereal disease research laboratory (VDRL), bacterial culture, fungus culture and skin biopsy may be helpful in differentiating from other diseases. In histology, condyloma lata caused by Treponema pallidum reveals spirochetes in silver stain.

The mechanism of development of PPPN is not clear. However, the most important factor is presumed to be the humid environment resulting from repeated or continuous contact with urine or loose feces. This state can exacerbate maceration and increase the vulnerability to the damaging effects of fecal irritants such as pancreatic proteases and lipases.^1,10 The urinary ammonia produced by intestinal bacteria ureases in feces makes an alkaline environment, and in this high pH environment, these enzymes become more active and have a stronger damaging effect.¹¹ Other suggested contributing pathogenetic factors include occlusion by nylon or cloth diapers, contact with detergents, starch containing powders, halogenated steroids, plastic pants, Candida infection, and urinary infection.^5,6,11 The treatment should primarily focus on removing the precipitating factors and restoring the skin barrier function to reduce irritation and prevent secondary infection. Thus, timely changing of diapers is important. Superabsorbent disposable diapers are recommended over cloth diapers as cloth diapers wet more easily and may contain remnant detergents, a potential pathogenic factor.¹² There have been reports that shave excision, and repeated cryotherapy sessions are effective in PPPN treatment.^5,13 However, these destructive or surgical methods only provided temporary relief. Additional treatment options are applying topical steroid and topical zinc oxide as a protective barrier.⁴ In a recent study, applying potato protease inhibitors was tested as a novel treatment for preventing perianal dermatitis caused by protease.¹⁴

Recognition and correct diagnosis of PPPN is important as topical steroids and starch containing powders, two most commonly used treatments for diaper rash, may aggravate PPPN. As the elderly population increases and the number of patients with colostomies and urostomies rises, PPPN in the elderly is expected to increase in the future. In order to minimize unnecessary investigations and treatments, clinicians should be aware of various manifestations of peristomal and diaper area dermatitis.