Disseminate and Recurrent Infundibular Folliculitis: Case Report and Literature Review

Abstract

We retrospectively analyzed the clinical data of 1 case of disseminate and recurrent infundibular folliculitis (DRIF) in our hospital and 31 cases of DRIF reported in previous domestic and international studies. There were 32 cases, including 22 males and 10 females, with a mean age of onset of 24.72 ± 13.68 years. Skin lesions were more likely to occur on the trunk and proximal extremities and may involve the face, neck, and genitals. Typical histopathology revealed mild thickening and spongy edema of the spinous layer, different degrees of pigmentation increase in the basal layer, and mild to moderate spongy edema and inflammatory cell infiltration (predominantly lymphocytes and neutrophils) in the infundibulum and around the hair follicle. DRIF is a chronic disease more prevalent in young men of African descent and individuals with Fitzpatrick skin types 4–6. This disease is diagnosed based on clinical features and histopathology and is preferentially treated with retinoids.

Introduction

Disseminate and recurrent infundibular folliculitis (DRIF) is a rare skin disease of unknown etiology first reported in 1968 by Hitch and Lund.[1] This disease often occurs in young African men and individuals with Fitzpatrick skin types 4–6.[2] DRIF is characterized by skin-colored papules centered on hair follicles on the trunk and proximal extremities, which are often accompanied by pruritus. Pustules and hairs can also be seen on the papules. The skin lesions in DRIF are usually chronic and recurrent and may also be persistent. Most patients have neither personal or family history of the disease nor abnormal laboratory test results. Here, we reported a case of DRIF and reviewed 31 cases of DRIF in the literature.

Case report

A 31-year-old female patient presented to our hospital due to widespread skin-colored papules with itching on the trunk and extremities for 4 years. Four years ago, the patient developed a needle tip-to grain-sized skin-colored papule on the back with no obvious cause, accompanied by severe itching. The rash gradually increased and spread to the entire body [Figure 1a-d]. The patient was sequentially diagnosed with “pityriasis rubra pilaris,” “follicular eczema,” and “porokeratosis” at other hospitals and was treated with emollients, topical glucocorticoids, antihistamines, and traditional Chinese medicine. However, none of the treatments was effective for the patient. The patient was healthy and had neither special personal or family history nor systematic abnormalities. Dermatological examination revealed grain-sized skin-colored papules (about 1–2 mm diameter) centered on hair follicles that are densely distributed on the trunk and extremities. The papules were polygonal or hemispherical without pus or fusion of rashes and resulted in a bumpy chicken skin-like texture that was dry, rough, and slightly hard with no scales on the surface. The rashes on the extremities are slightly sparser than those on the trunk and those on the back are flaky with pigmentation and unclear boundary. The skin of the entire body is dry without scales. Laboratory tests showed no abnormalities in hematology, urinalysis, and blood biochemistry tests, and fungal microscopy and culture showed negative results. Histopathology suggested hyperkeratosis and columnar parakeratosis with thickened acanthocyte layer, dyskeratosis cells, some lymphocyte infiltration around blood vessels in the superficial dermis, scattered chromophagocytes, and spongy edema of the infundibulum of the hair follicle with a small amount of lymphocyte infiltration [Figure 2a and b]. Therefore, the patient was diagnosed with DRIF. However, due to the patient’s desire to become pregnant, no treatment was provided.

Figure 1.

Widely distributed skin-colored grain-sized papules centered on hair follicles present on the abdomen (a), back (b), upper arm (c), and forearm and back of the hand (d) of the patient

Figure 2.

Histopathological features of DRIF. (a) Hyperkeratosis and columnar parakeratosis with thickened acanthocyte layer, dyskeratotic cells, trace amount of lymphocyte infiltration around the blood vessels of the superficial dermis, and scattered chromophagocytes; (b) Spongy edema in the infundibulum of the hair follicle with some lymphocyte infiltration (40× magnification)

Literature review

Materials and methods

One case of DRIF was reported, and the data of DRIF cases reported in previous literature were retrospectively analyzed. All reported cases of DRIF were searched in CNKI and PubMed using the keywords “disseminate and recurrent infundibular folliculitis” and “DRIF.”. Patient gender, age, skin color, distribution of skin lesions, histopathology, and treatment outcomes were extracted from studies with a clear diagnosis and relatively complete clinical data for analysis.

General information

Thirty-two DRIF cases were included in this review, including 22 males and 10 females, five of which were children. One case was reported in this study, and 31 cases were reported in previous literature (22 males and 9 females). One case was also comorbid with progressive macular hypopigmentation.[3] These DRIF cases were reported in various regions including North America, Asia, Australia, and Europe. There were 27 patients with colored skin, three White patients, and two patients with unspecified skin color. One case was suspected to be related to familial heredity.[4] Twenty-seven cases had pruritic symptoms, and five cases were asymptomatic. Eight cases mentioned that itching was exacerbated in the summer or after sweating, and five cases mentioned that the skin lesions were more severe in summer and alleviated in the winter. Four studies reported that their patients had persistent skin lesions (including the present case).

Age of onset and course of disease

The age of onset ranged from 2 to 65 years (mean 24.72 ± 13.68 years), with a mean age of 25.64 ± 15.63 years for males and 22.70 ± 8.27 years for females. When stratified by age (in increments of 10 years), it was found that most of the patients were aged between 11 and 40 years (26 cases accounting for 81.25%) at the time of onset. The duration of the disease ranged from 14 days to 40 years, with a mean of 50.24 ± 91.16 months. The course of the disease was ambiguous for one case,[5] and hence this patient was removed from the analysis.

Clinical features

Skin lesions mostly affect the trunk and proximal extremities and may involve the face, neck, and genitals.[5] A case of persistent DRIF with an atypical distribution pattern was reported in India where papules and pustules are linearly arranged within the folds of the neck.[6] For most cases, skin lesions appear as grain-sized skin-colored polygonal or hemispherical papules (about 1–2 mm) centered on hair follicles that are densely distributed with a smooth or rough texture and may be accompanied by pustules. Pruritus is the key factor that plagues DRIF patients.

Histopathological features

The lesions mainly develop around the hair follicles, and there are generally no specific changes in the epidermis. The stratum corneum is generally hyperkeratotic but may sometimes exhibit atypical manifestations such as hyperkeratosis with parakeratosis in our case. The spinous layer may have mild edema and thickening, with hyperpigmentation of the basal layer. The blood vessels in the superficial dermis are slightly dilated and infiltrated by a small number of inflammatory cells, predominantly lymphocytes and eosinophils.[7] DRIF lesions are mainly found around the hair follicles and appendages and are characterized by mild to moderate spongy edema and inflammatory cell infiltration (predominantly lymphocytes and neutrophils with diffused monocytes) in the infundibulum and around the hair follicles.[6,8,9] Keratin formation can be seen at the hair follicle orifice.

Treatment

At present, there is no definitive treatment for DRIF. However, there are various therapies that have shown varying degrees of improvement or even resolution of skin lesions and itching. These treatments include oral doxycycline, vitamin A supplementation, retinoid medications as well as topical potent steroids, tacrolimus, tretinoin cream, and phototherapy. Retinoic acid drugs, in particular, are frequently used and have shown notable effects in managing the condition.

Discussion

DRIF is a benign disease that causes itching and aesthetic concerns for patients. Accurate diagnosis is crucial for dermatologists to effectively address the issue. For instance, in the case of the patient reported here, the pathology can be perplexing. If we solely consider the changes in the epidermis without taking into account the concurrent alterations in the hair follicle appendages and clinical manifestations, we may misdiagnose or overlook the condition. It is important to distinguish DRIF from other diseases such as Lichen spinulosus, Keratosis pilaris, Hypovitaminosis A, and Lichen nitidus. Lichen spinulosus commonly affects children, appearing on the neck, buttocks, and extensor sides of the limbs. It is characterized by the presence of filamentous horny spines on the apex of follicular papules. Clinical features of Keratosis pilaris include follicular papules with angular plugs, and small cup-shaped dimples that become visible upon removal of the horn plugs. Hypovitaminosis A is often accompanied by dry and rough skin as well as symptoms like dark adaptation disorder or night blindness, in addition to follicular papules. Lichen nitidus tends to occur in the lower abdomen, penis, and buttocks. The notable histopathological feature is the elongation of epidermal processes on both sides, accompanied by infiltration of inflammatory cells in the surrounding dermal papilla. Currently, there is no specific treatment for DRIF. However, literature reports suggest that retinoids appear to be the most effective option, while the use of a large quantity of topical glucocorticoids is not recommended.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.