|Year : 1995 | Volume
| Issue : 3 | Page : 159-162
VL Aswani, RH Malkani
V L Aswani
Source of Support: None, Conflict of Interest: None
A case of follicular mucinosis is being reported. Clinically, the patient had discrete follicular and non-follicular papules on the face, neck and arms. Light microscopy confirmed the follicular outer root sheath, and sebaceous gland epithelial degenerative changes. The infiltrating inflammatory cells were morphologically benign. The response to oral dapsone therapy is highlighted.
Keywords: Follicular mucinosis, Dapsone
|How to cite this article:|
Aswani V L, Malkani R H. Follicular mucinosis. Indian J Dermatol Venereol Leprol 1995;61:159-62
| Introduction|| |
The first series of patients with alopecia mucinosa was presented by Pinkus in 1957. He described six cases of slowly enlarging inflammatory plaques with alopecia and root sheath mucinosis. A few years later Jablonska et al suggested the name follicular mucinosis, because alopecia is not always an apparent or prominent feature.
Clinically follicular mucinosis (FM) may start as grouped follicular papules or plaques that are hypopigmented, slightly erythematous, scaly and eczematous or there may be a solitary nodule. The plaques and nodules are generally firm, but may be soft. Sometimes mucin can be squeezed out of affected follicles. If a hair bearing area is involved non-scarring alopecia may result. The most commonly affected areas are the head and neck, but with multiple lesions the trunk and extremities may also be involved. Rarely, follicular papules may be generalised without obvious grouping. There is usually little or no pruritus. Rare cases of local dysesthesia have been reported. A coexisting lymphoma is associated with follicular mucinosis in about 15% of cases.
| Case Report|| |
A 62-year-old male presented with multiple, discrete skin coloured to reddish papules on the face, neck and lower arms that were associated with mild itching since 1 year. There was no hisotry of photosensitivity. He had diabetes which was under control with diet and exercise. There was no history of drug intake prior to the onset of the disease.
His vital parameters were within normal limits. Cutaneous examination revealed discrete, skin coloured to slightly erythematous, follicular and nonfollicular papules on the face, neck and lower arms. They ranged in size from 2-5 mm and were firm in consistency. Some of the hair follicles were accentuated but did not contain keratin plugs; some were without hair. Systemic examination revealed no abnormality.
A complete blood count was within normal limits. The fasting and post-prandial blood sugar levels were 100 mg and 130 mg respectively. Liver and renal function tests were normal.
A skin biopsy showed dilated hair follicles with mucinous changes and the formation of cystic cavities in the outer root sheath surrounded by a predominantly lymphocytic infiltrate and a few epithelioid cells. One section revealed a large cystic cavity formed due to mucinous degeneration of the outer root sheath containing degenerated outer root sheath cells, lymphocytes and eosinophils. A mucicarmine stain did not show mucin.
Patient was given dapsone, 100 mg orally daily. After one week of treatment he had no new lesions, after two weeks the lesions had reduced in size and by the end of the month they had resolved.
| Discussion|| |
Our patient presented with discrete, firm, follicular and nonfollicular papules, some with accentuated follicular prominences, on the sun exposed parts of the body, suggesting a differential diagnosis of papular polymorphic light eruption and papular discoid lupus erythematosus. It was only on skin biopsy that the diagnosis of follicular mucinosis was made.
No mucin could be demonstrated on staining with the mucicarmine stain as it can be removed during fixation on account of its solubility in water. Staining of a frozen section is required in such cases.
The disease has been known to assume one of three possible clinical courses:
- (a) Spontaneous resolution after 2 months to 2 years, without any recurrence,
- (b) A chronic course with the persistence of individual lesions or the appearance of new lesions for over 2 years, but with no sign of the development of reticulosis, or
- (c) An association with reticulosis.
In a study by Nickoloff, the following histologic criteria were found in benign juvenile idiopathic FM, in contrast to FM associated with mycosis fungoides:
- (1) The lymphocytic infiltrate was generally confined to the follicular, perifollicular or perivascular zones with no extension into the epidermis.
- (2) Within follicular epithelium there was a dense collection of lymphocytes with occasionally a typical appearing nuclei but never as Pautrier microabscess More Detailses.
- (3) There was the absence of a significant associated plasma cells or eosinophil containing inflammatory dermal infiltrate.
Our patient responded well to treatment with dapsone, but no mode of therapy is consistently effective. Other modalities include topical, intralesional, or systemic steroids; superficial X-ray therapy; and PUVA therapy. In patients with FM and a lymphoma, therapy should be directed at the malignancy.
| References|| |
|1.||Pinkus H. Alopecia mucinosa: Inflammatory plaques with alopecia characterized by root sheath mucinosis. Arch Dermatol 1957;76:419-26. [PUBMED] |
|2.||Jablonska S, et al. Mucinosis follicularis. Hautarzt 1959;10:27-33. [PUBMED] |
|3.||Arnold HL Jr. Dysesthesia in alopecia mucinosa: A possible diagnostic sign. Arch Dermatol 1962;85:409-10. [PUBMED] |
|4.||Emmerson RW. Follicular mucinosis: A study of 47 patients. Br J Dermatol 1969;81:395-413. [PUBMED] |
|5.||Kubba RK, Stewart TW. Follicualr mucinosis responding to dapsone. Br J Dermatol 1974;91:217-20. [PUBMED] |
|6.||Nickoloff BJ, et al. Benign idiopathic vs mycosis fungoides associated follicular mucinosis. Pediatr Dermatol 1985;2:201-6. [PUBMED] |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]