Disseminate and recurrent infundibulo-folliculitis in an Indian patient: A case report with review of literature.

A 17-year-old male patient presented with multiple discrete and confluent monomorphic skin-colored pinhead-sized follicular papules, with occasional pustules distributed on the neck, upper chest, upper posterior trunk, and proximal extremities of 4 months duration. The lesions were asymptomatic, and there was no prior history of topical application or history of atopic dermatitis. Routine investigations were normal. Histopathology of the papules showed a mononuclear infiltrate at the infundibulum of the hair follicle. We made a final diagnosis of disseminate and recurrent infundibulo-folliculitis. The patient was started on NB-UVB and topical tacrolimus. We are reporting an interesting case in an Indian patient.

Introduction

Disseminate and recurrent infundibulo-folliculitis (DRIF) is a rare noninfectious folliculitis occurring predominantly in the black race, first described by Hitch and Lund in 1968, and hence known as the Hitch and Lund disease.[12] This entity has occasionally been reported in other races also. It presents with asymptomatic skin-colored, monomorphic, follicular papules usually distributed on the trunk and extremities. Pustules and hair protruding from the papules may also be seen. The histopathological changes are confined to the infundibulum of the hair follicle, and hence the name DRIF.[3] Cases of DRIF in literature are undocumented, more so in India.[3]

Case Report

A 17-year-old male presented with asymptomatic papules on the neck, chest, trunk, and upper extremities of 4 months duration. The papules first started on the upper lateral neck bilaterally 4 months ago, and during the course of a few months extended to the lower neck, upper chest, shoulder, posterior upper trunk, and proximal extremities in that order. The papules were asymptomatic and persistent. The patient denied any history of fever prior to the onset of the lesions, past history of atopy, or any prior topical application. On examination, the patient had skin-colored multiple discrete and confluent, pin-head sized, monomorphic follicular papules distributed bilaterally on the neck and extending below the supraclavicular margin to involve the anterior upper chest, the posterior upper trunk upto the level of the suprascapular margin, and the proximal upper extremities upto the insertion of the deltoid [Figure 1a]. On close examination, some of the papules were arranged in linear rows across the neck creases and a few pustules were seen interspersed along with the follicular papules [Figure 1b]. The other areas of the body, scalp, palms and soles, mucous membranes, hair, and nails were normal. Systemic examination was unremarkable. We had a differential diagnosis of the following: bacterial folliculitis, pityrosporum folliculitis, follicular eczema, keratosis pilaris, lichen spinulosis, justa-clavicular beaded lines, and follicular lichen planus.

Figure 1

(a) Closely set monomorphic follicular papules on anterior and lateral part of neck, (b) Close view showing linear row of papules with a pustule (arrow)

The patient's blood hemogram, biochemistry, liver, and renal function tests were normal, and serological tests for syphilis and HIV were negative. A gram stain and pus culture and sensitivity from a pustule did not demonstrate any organism. A skin biopsy from a representative papule and pustule, stained with H and E, showed similar findings of a mononuclear infiltrate arranged at the infundibulum of the hair follicle in the longitudinal section [Figure 2a] and a perifollicular infiltrate in the crosssection [Figure 2b], diagnostic of DRIF. The other areas of the epidermis and dermis were normal. We made a final diagnosis of DRIF.

Figure 2

(a) Longitudinal section showing mononuclear infiltrate at the infundibulum of hair follicle (arrow), (b) Cross-section showing perifollicular mononuclear infiltrate, (H and E × 100)

Discussion

The classical presentation with follicular monomorphic skin-colored papules and a few pustules distributed on the neck, upper chest, and trunk and proximal extremities, along with a skin biopsy finding of a mononuclear infiltrate around the infundibulum of the hair follicle enabled us to make a diagnosis of DRIF. Studies have shown direct immunofluorescence to be negative.[3] The follicular papules in DRIF can be chronic or recurrent. The lesions in our patient were chronic and persistent, rather than recurrent, and hence disseminate and persistent infundibulo-folliculitis would be a more appropriate term.[4] There have been observations suggesting that DRIF may be a manifestation of atopic dermatitis in black patients. However, subsequent reports and in the present case also there were no evidence of past or present evidence of atopic dermatitis. It is proposed that DRIF could be a nonspecific skin reaction pattern to an unknown antigen. The search for an infective agent has so far been elusive. There have also been observations that DRIF could be an overt expression of normal follicular prominence because clinically they mimic “goose bumps.” However, the presence of occasional pustules and a mononuclear infiltrate around the infundibulum negates this view. A close differential diagnosis may be follicular eczema, but the latter is usually seasonal, pruritic and histopathology shows the features of spongiotic dermatitis and there is prompt response to topical steroids. Another close differential is justa-clavicular beaded lines.[5] This entity presents with skin-colored follicular papules distributed in the upper trunk, and histopathology shows features of hyperplastic pilo-sebaceous units along with spongiosis and exocytosis. The clinical presentation of DRIF is clinically classical, but other causes of follicular papules such as bacterial folliculitis, pityrosporum folliculitis, keratosis pilaris, lichen spinulosis, and follicular lichen planus have to be ruled out, even though these conditions have characteristic clinical and histopathological findings. Their differentiating features are given in Table 1.

Table 1

Differential diagnosis of DRIF

There are no definite treatment modalities for DRIF. The treatment modalities available are anecdotal and in most cases ineffective. Topical treatment modalities are potent corticosteroids,[6] tretinoin, 12% lactic acid and 20 − 40% urea. The systemic therapies mentioned are high dose oral vitamin A (100,000 IU/day),[4] isotretinoin,[78] and PUVA.[9] We started NB-UVB in our patient along with topical tacrolimus (0.1%) for 8 weeks, which was never tried before, but the response was moderate.

The present case was reported because of its rarity in Indian patients, and persistent course.

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Conflicts of interest

There are no conflicts of interest.