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Table of Contents 
Year : 2013  |  Volume : 58  |  Issue : 3  |  Page : 227-231
Alopecia mucinosa responding to antileprosy treatment: Are we missing something?

1 Department of Dermatology, P. D. Hinduja Hospital, Mahim, Mumbai, India
2 Thane Skin Centre, Thane, Maharashtra, India

Date of Web Publication20-Apr-2013

Correspondence Address:
Rajiv Joshi
14 Jay Mahal, A Road, Churchgate, Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.110834

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Three cases with single lesion of Alopecia mucinosa (follicular mucinosis) were treated with antileprosy treatment and showed rapid and complete resolution of the lesions with no recurrence on extended follow-up. Two children, a boy aged 14 years and a girl aged 12 years presented themselves, each, with a single hypopigmented, hypoesthetic patch on the face. Clinically leprosy was suspected, however, skin biopsy from both patients revealed follicular mucinosis as the only pathological finding, without any granulomas. Based on clinical suspicion both were started on multi drug therapy (MDT) for leprosy with complete resolution of the lesions. The third case, male, aged 22 years presented with a single erythematous, hypoesthetic plaque on the forehead.This lesion had been diagnosed as follicular mucinosis with folliculo-tropic mycosis fungoides, in the USA. He too responded completely within 3 months with rifampicin, ofloxacin, minocycline (ROM) treatment, which was given once monthly for a total of 6 months and remains free of disease since the past 1 year. Follicular mucinosis as the only pathology may be seen in facial lesions of clinically suspected leprosy in children and young adults. Based on histological findings these cannot be diagnosed as leprosy and will be considered as Alopecia mucinosa. These lesions, however, are always single and show rapid and complete response to antileprosy treatment. The authors suggest that in regions endemic for leprosy, such as India, single lesion Alopecia mucinosa on the face in children and young adults should be given antileprosy treatment.

Keywords: Alopecia mucinosa, follicular mucinosis, leprosy

How to cite this article:
Joshi R, Gopalani V. Alopecia mucinosa responding to antileprosy treatment: Are we missing something? . Indian J Dermatol 2013;58:227-31

How to cite this URL:
Joshi R, Gopalani V. Alopecia mucinosa responding to antileprosy treatment: Are we missing something? . Indian J Dermatol [serial online] 2013 [cited 2020 Aug 9];58:227-31. Available from:

What was known? Benign or idiopathic type of Alopecia mucinosa occurs in children and young adults and has a variable natural course and does not have any specific effective therapy.

   Introduction Top

Follicular mucinosis is a histological reaction pattern wherein variable amounts of epidermally derived mucin accumulate within follicular infundibula and the outer root sheath epithelium. [1] The numerous disease associations with production of follicular mucin suggests that this is a nonspecific reaction pattern. [2]

Alopecia mucinosa is a clinic-pathological entity which presents with several erythematous papules and plaques mainly over the head and face regions with associated loss of hair and histopathological findings of follicular mucinosis affecting most of the follicles.

Alopecia mucinosa has been divided into the primary or idiopathic variety and the secondary type associated with cutaneous T cell lymphoma.

Primary or idiopathic Alopecia mucinosa was first described in 1957 by Pinkus. [3] It is considered a benign condition, occurs mainly in children and young adults and presents with several reddish papules or plaques on the head and face with associated alopecia. It may persist for months or years and no specific therapy has been found to be effective for patients with idiopathic alopecia mucinosa. [4]

Secondary alopecia mucinosa, which many now consider to be mycosis fungoides, presents in older individuals, with or without other features of mycosis fungoides.

Follicular mucinosis as a histological finding by itself, does not denote a specific clinico-pathological entity and has been described as an incidental finding in several unrelated conditions like angiolymphoid hyperplasia, [5],[6] familial reticuloendotheliosis, [7] spongiotic dermatitis, [1] and in diffuse papular and eczematous eruptions. [8],[9]

Follicular mucinosis as the only histological finding without granulomas has been recently described in children with suspected leprosy who had complete clearing of the lesions with standard multi drug therapy (MDT) for leprosy. [10]

In this article we describe three cases, two children with a single facial lesion clinically suspected to be leprosy in whom biopsy showed follicular mucinosis without granulomas. Both cases had complete clearing of the skin lesions with standard MDT for leprosy. The third case was a 22-year-old male who had a single erythematous, hypoesthetic lesion on the forehead, diagnosed as folliculo-tropic mycosis fungoides in the USA, who also responded completely with rifampicin, ofloxacin, minocycline (ROM) treatment with no recurrence over the past 1 year.

   Case Reports Top

Case 1

A 14-year-old boy presented in September 2007, with a hypopigmented and hypoaesthetic shiny patch on the left cheek of few months duration. A clinical diagnosis of leprosy was considered and a 3 mm punch biopsy sample revealed features of follicular mucinosis with most follicles in the sections showing spaces between keratinocytes containing wisps of bluish mucin. Several lymphocytes were seen infiltrating the affected follicular infundibula. The upper and mid dermis had a moderately dense perivascular lymphocytic infiltrate with few eosinophils. No granulomas were seen.

Although a definite histological diagnosis of leprosy could not be rendered, based on the clinical suspicion the patient was started on MDT, with Rifampicin 450 mg once a month and dapsone 100 mg daily according to the standard World Health Organization (WHO) protocol. He was followed-up at monthly intervals and at the end of 6 months, the lesion had cleared significantly, although not completely and the treatment was stopped. Subsequently the lesion was seen to have completely cleared at follow up at the end of 1 and 2 years.

Case 2

A 12-year-old girl presented in March 2009, with hair loss over left eyebrow and dry hypopigmented skin of adjacent forehead. The skin lesion and hair loss had been present for about 3 months and was gradually progressive. Sensations were mildly impaired over the site but no thickened nerve was palpable. A clinical diagnosis of leprosy was considered and she was asked to apply a moisturizing cream and kept under observation as a biopsy was refused. A month later the patch had enlarged minimally, the surface was dry and showed follicular prominences, however, no erythema was seen [Figure 1]a. She agreed to a biopsy and a 3 mm punch biopsy was performed from the patch just above the left eyebrow. The biopsy showed a moderately dense superficial and mid perivascular infiltrate of lymphocytes with occasional eosinophils without any granulomas in the sections. Few of the follicular infundibula showed features of follicular mucinosis with mild spongiosis, separation of the keratinocytes from one another, and clear spaces between the keratinocytes, which contained wisps of light blue-gray mucin. Staining with Alcian blue revealed small quantities of bluish mucin [Figure 2],[Figure 3],[Figure 4].
Figure 1: (a) Case 2 at presentation with hypopigmented patch on left eyebrow region, (b) Case 2 after 6 months MDT, complete clearing of skin patch, with complete regrowth of eyebrow hair and visible scar of biopsy

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Figure 2: Photomicrograph of follicular mucinosis (case 2) (H and E, ×25)

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Figure 3: Photomicrograph of follicular mucinosis showing clear spaces between follicular infundibular keratinocytes with wisps of bluish mucin (H and E, ×400)

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Figure 4: Alcian blue stain for mucin (×250)

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Based on clinical suspicion of leprosy and because of increase in the size of the patch she was prescribed MDT, Rifampicin 300 mg once a month, and dapsone 50 mg daily as her weight was 30.5 kg. The treatment was, however, started a month later as she was in her village for the summer holidays. On follow-up 1 month after starting MDT, that is, in June 2009, the hypopigmented patch was still present, but had stopped increasing in size, the follicular prominences had flattened and some regrowth of hair was seen which continued through the next month. Her hemoglobin had fallen to below 9 gm/dl (G6PD was normal) so dapsone was reduced to 100 mg once a week and clofazimine 50 mg daily was introduced. Follow up in September 09, 3.5 months after starting of MDT showed faint hypopigmentation with indistinct margins. She completed 6 months of MDT in December 2009, at which time the lesion had cleared completely with regrowth of all hair [Figure 1]b.

Case 3

A 22-year-old Indian male, studying in the USA, presented in December 2010, with a 6 month history of a single gradually enlarging erythematous plaque on the left forehead of 4 × 3 cm size with mild hypoesthesia [Figure 5]. No thickened nerves were palpable. Three months back he had a skin biopsy in the USA and diagnosed as follicular mucinosis with folliculotropic mycosis fungoides. The biopsy sections showed accumulation of mucin in all follicles in the sections and a fairly dense lymphocytic infiltrate with several eosinophils. Lymphocytes were seen infiltrating the follicular epithelium but no atypical lymphocytes were seen. No granulomas were seen. Immunohistochemistry showed the infiltrating lymphocytes to be CD3 and CD4 positive. The T cell receptor (TCR) gene rearrangement studies done in USA had been reported as indeterminate. He had been advised topical Tretinoin and Deoximetasone creams, which he used without any improvement over 3 months.
Figure 5: Case 3 at presentation with erythematous plaque on left forehead

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He gave a history of childhood atopic dermatitis with mild asthma but was off all medications since several years. His baseline blood investigations repeated in India were normal except for eosinophilia of 21%. With the good response to MDT in the previous 2 cases it was decided to start him also on MDT, however, as he was shortly returning to the USA, he was started on ROM therapy (rifampicin 600 mg, ofloxacin 400 mg, minocycline 100 mg) to be taken once a month for 6 months. The skin lesion had completely healed after 3-4 months of starting the treatment. The patient completed all six doses of ROM, and has not had recurrence in the ensuing 1 year.

   Discussion Top

The objective of this paper is to draw attention to an unusual finding, that is, rapid and complete response of Alopecia mucinosa to antileprosy treatment with no subsequent relapse over fairly long periods of posttreatment observation. We report three cases to add to four similar cases reported earlier by one of the authors. [10]

The following points need further discussion:

  1. Clinical presentation: All our cases presented with single lesions which demonstrated hypoesthesia. This is in contrast to cases of Alopecia mucinosa reported in literature including the first report of six cases by Pinkus [3] in which multiple erythematous papules and plaques occur over the head and neck region.
  2. Treatment of alopecia mucinosa

    1. The lesions of Alopecia mucinosa tend to persist for months or years and may show spontaneous clearing, however, in general the therapy for alopecia mucinosa remains nonspecific as no single agent has been shown to consistently improve this condition. [4] In contrast all our cases showed a remarkable consistency in rapid and complete response to antileprosy treatment. These lesions had persisted for months, had even grown in size in case 2, and not responded to topical steroids and tretinoin in case 3. Starting antileprosy therapy resulted in immediate clinical response. Therefore it is too much of coincidence to consider that the response in all patients was due to natural remission, and it is most likely that it was the oral treatment that brought about healing of the lesions.
    2. Dapsone has been reported to clear Follicular mucinosis and the presence of dapsone in the MDT may be the agent responsible for healing in cases 1 and 2. Careful perusal of literature reveals that only two papers [8],[9] have reported response to dapsone. Important to note that both these cases were not of Alopecia mucinosa, but had a clinical presentation of diffuse acute dermatitis with an incidental finding of follicular mucinosis. We have not been able to find any reported case of Alopecia mucinosa treated with dapsone alone.
    3. Minocycline has been used in treatment of Alopecia Mucinosa, and minocycline in ROM may be the responsible for healing in case 3: Some papers [4],[11] have shown good response of follicular mucinosis to minocycline, however, contrary results with very minimal or no improvement has also been reported in patients with Alopecia mucinosa who were treated with minocycline for more than 3 months. [12] Thus minocycline does not seem to give consistently good results in all cases of Alopecia mucinosa.

Although clinically cases 1 and 2 (and the four cases described earlier [10] ) were clinically diagnosed as leprosy, the biopsy did not show any granulomas or features diagnostic of leprosy, but instead showed in all cases follicular mucinosis. Therefore the final clinic-pathologic diagnosis was Alopecia mucinosa.

At this stage it is pertinent to reflect on the vagaries and irony of the practice of medicine.

It is common practice that in regions endemic for leprosy, such as India, children and young adults who present with single hypoesthetic lesions on the face are not biopsied but treated on clinical suspicion alone and because they respond to MDT would be labeled as leprosy.

Our cases too, would have been accepted as leprosy if a biopsy had not been performed as there was clinical suspicion of leprosy and all of them did rapidly and completely respond to antileprosy treatment. With the results of the biopsy, however, these cases would be diagnosed as Alopecia mucinosa!

Without suggesting that these cases should be considered as leprosy, it is interesting to speculate on whether leprosy may present with histological findings of follicular mucinosis?

Cell mediated immune mechanisms play a role in the pathogenesis of follicular mucinosis and T lymphocytes that infiltrate follicular epithelium may lead to destructive changes in the follicular keratinocytes to produce mucin. [13],[14],[15]

Follicular mucinosis as a finding has been recorded as an additional histological marker in type 1 reactional state in borderline leprosy. [16] A recent paper [17] has reported that folliculotropism of lymphocytes was seen in 55% of patients with reactions in leprosy.

In patches of early leprosy granulomas may be absent and lymphocytes may be seen infiltrating the epidermis and follicular epithelium and arrector pilorum muscles. It is possible that these activated T lymphocytes may cause mucin accumulation in the affected follicles, and it is conceivable that follicular mucinosis may occur in lesions of leprosy without granulomas.

Considering the rapid and complete response to antileprosy treatment in 7 cases of Alopecia mucinosa (three cases described in this paper and the four similar cases described earlier [10] ), it is most unlikely that the response was a natural remission of the disease. While response to dapsone in the first six cases and minocycline in the last case is possible, the most consistent common factor in all cases seems to be antileprosy treatment.

In sum, we present three cases of Alopecia mucinosa, who presented with single lesions on the face, had follicular mucinosis on biopsy and who healed rapidly and completely with initiation of antileprosy treatment.

Thus we recommend that in regions endemic for leprosy, such as India, children and young adults who present with single lesions on the face that show follicular mucinosis as the only pathology, be treated with standard MDT.

   References Top

1.Hempstead RW, Ackerman AB. Follicular mucinosis: A reaction pattern in follicular epithelium. Am J Dermatopathol 1985;7:245-57.  Back to cited text no. 1
2.Elder DE, Elenitsas R, Johnson BL Jr, Murphy GF, Xu X, editors. Follicular Mucinosis and Alopecia Mucinosa. Inflammatory diseases of hair follicles, sweat glands and cartilage. In Lever's Histopathology of the skin, 10 th ed. Philadelphia: Wolters Kluwer /Lippincott Williams & Wilkins; 2009. p. 467-8.  Back to cited text no. 2
3.Pinkus H. Alopecia mucinosa: Inflammatory plaques with alopecia characterized by root sheath mucinosis. AMA Arch Derm 1957;76:419-26.  Back to cited text no. 3
4.Parker SR, Murad E. Follicular mucinosis: Clinical, histological and immunological remission with minocycline. J Am Acad Dermatol 2010;62:139-41.  Back to cited text no. 4
5.Wolff HH, Kinney J, Ackerman AB. Angiolymphoid hyperplasia with follicular mucinosis. Arch Dermatol 1978;114:229-32.  Back to cited text no. 5
6.Joshi R. Angiolymphoid hyperplasia with follicular mucinosis. Indian J Dermatol Venereol Leprol 2007;73:346-7.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
7.Freeman RG. Familial reticuloendotheliosis with eosinophilia and follicular mucinosis. Arch Dermatol 1972;105:737-8.  Back to cited text no. 7
8.Kubba RK, Sewart TW. Follicular mucinosis responding to dapsone. Br J Dermatol 1974;91:217-20.  Back to cited text no. 8
9.Rustin MH, Bunker CB, Levene GM. Follicular mucinosis presenting as acute dermatitis with response to dapsone. Clin Exp Dermatol 1989;14:382-4.  Back to cited text no. 9
10.Joshi RS. Follicular mucinosis in suspected Leprosy. Indian J Lepr 2002;74:341-7.  Back to cited text no. 10
11.Yotsumoto S, Uchimiya H, Kanzaki T. A case of follicular mucinosis treated successfully with minocycline. Br J Dermatol 2000;142:841-2.  Back to cited text no. 11
12.Wittenberg GP, Gibson LE, Pittelkow MR, el-Azhary RA. Follicular mucinosis presenting as an acneiform eruption: Report of four cases not responsive to minocycline. J Am Acad Dermatol 1998;38:849-51.  Back to cited text no. 12
13.Lancer HA, Bronstein BR, Nakagawa H, Bhan AK, Mihm MC Jr. Follicular mucinosis: A detailed morphologic and immunopathologic study. J Am Acad Dermatol 1984;10:760-8.  Back to cited text no. 13
14.Reed RJ. The T-lymphocyte, the mucinosis epithelial interstitium and immunostimulation. Am J Dermatopathol 1981;3:207-14.  Back to cited text no. 14
15.Ishibashi A. Histogenesis of mucin in follicular mucinosis. An electron microscopic study. Acta Derm Venereol 1976;56:163-71.  Back to cited text no. 15
16.Lazaro-Medina A, Tianco EA, Avila JM. Additional markers for the type 1 reactional states in borderline leprosy. Am J Dermatopathol 1990;12:417-21.  Back to cited text no. 16
17.Adhe V, Dongre A, Khopkar U. A retrospective analysis of histopathology of 64 cases of lepra reactions. Indian J Dermatol 2012;57:114-7.  Back to cited text no. 17
[PUBMED]  Medknow Journal  

What is new? Single lesion Alopecia mucinosa in children and young adults responds rapidly and completely with antileprosy treatment.


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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