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E. M. C. Passaro, M. T. Silveira and N. Y. S. Valente Department of Dermatology, Hospital do Servidor Pu´blico do Estado de Sa˜o Paulo, Brazil Follicular mucinosis is a rare chronic inflammatory disease of unknown aetiology, presenting as mucin deposits around the follicles and sebaceous glands. It can progressto alopecia of the scalp and other hairy areas. Follicular mucinosis may be a benignprimary idiopathic disorder or secondary to malignant lymphoproliferative disorders. Itcan present with shiny papules or sharply marginated infiltrated erythematous scalingplaques, with follicular accentuation on the scalp, neck, trunk and limbs. There aremany local and systemic treatments. This paper discusses the case of an adult with anuncommon acneiform follicular mucinosis controlled with systemic corticosteroids.
lymphoproliferative processes such as cutaneous T- and B-cell lymphomas, Hodgkin’s disease, chronic and acute lymphocytic leukaemia.1 Two categories of dis- mucinosis in 1957 under the term alopecia mucinosis, ease have been identified in patients with the benign which was given in reference to a disease process of primary form. The first occurs in younger patients: there follicular degeneration resulting in alopecia. Histologi- are few lesions limited to the head and neck or upper cally the process manifests as deposition of mucin in the arms and which disappear spontaneously between epithelium of the follicular outer root sheath and 2 months and 2 years after onset. The second is a sebaceous glands.1 The condition was later renamed disorder of adult patients: the lesions are larger and follicular mucinosis by Jablonska, Chorzelski and more widespread and can take a longer period to Lancucki in 1959 because alopecia is not always improve. In the second type, which is linked to malignant lymphoproliferative conditions, the patients Follicular mucinosis is a chronic inflammatory disease are older and have widespread and infiltrated lesions of unknown aetiology. It is more commonly observed as that can progress to mycosis fungoides (MF).1,2 shiny pink or pale-coloured follicular papules or as The origin of the mucin deposits in the follicles is erythematous scaling infiltrated plaques. The involved unknown, but immunopathological studies have shown follicles may show conspicuous horny plugs, presenting that cytokines released from perifollicular T lympho- with alopecia when the scalp or other hairy regions are cytes might stimulate the follicular epithelium to secrete involved. The lesions may be localized or widely mucin.3,4 Immunohistochemical studies have shown disseminated. There are various distinctive clinical expansion of clonal T cells in many cases of primary presentations: alopecia areata-like, scarring alopecia, ‘benign’ mucinosis. The clonality is not always synony- nodules, cysts, chronic eczema and acneiform eruption.1 mous with malignancy, but its presence is a reason for primary disorder or be associated with malignant Various therapies have been tried for follicular muci- nosis: indomethacin, topical and systemic corticosteroids,dapsone, topical tretinoin, oral isotretinoin, minocycline, Correspondence: R. M. C. Passaro, R. Joa˜o de Souza Dias, 509 ⁄ 122, CampoBelo-Sa˜o Paulo, Brazil, 04618 tetracycline and psoralen with ultraviolet A.3,6 When the Tel.: +55 11 5088 8293. Fax: +55 11 5088 8293 condition has been associated with lymphoma, radio- therapy, topical nitrogen mustard, electron beam radi- ation and immunotherapy have been tried.7 Ó 2004 Blackwell Publishing Ltd • Clinical and Experimental Dermatology, 29, 396–398 Acneiform follicular mucinosis • E. M. C. Passaro et al.
Figure 2 Histopathology shows, in the centre of the field, a folliclesurrounded by a mixed inflammatory infiltrate. Mucin depositscan be seen in the outer sheath of the follicle (highlighted in thefigure inset). (Haematoxylin & eosin).
Figure 1 (a) Multiple erythematous follicular papules and noduleson the forehead and temples before treatment. The papules arenot coalescent and present an acne-like aspect. (b) Completeregression of the cutaneous lesions following treatment with oralprednisone 40 mg ⁄ day.
surrounding the hair follicles. A few eosinophils werepresent. Special stains for fungi and acid-fast bacilliwere negative. Demodex folliculorum was not isolated.
We report the case of a black 36-year-old male Syphilis and HIV serology were negative. Rosacea was patient, who complained of nodules on his face with the most likely diagnosis and so tetracycline, 1.5 g per slight pruritus that increased after sun exposure. The day, was given for 30 days but the patient did not lesions first became apparent 1 year before presentation.
Physical examination showed multiple red or pale A second biopsy revealed an inflammatory infiltrate isolated papules and nodules on his forehead (Fig. 1a), around the follicles composed of lymphocytes, histio- temples and behind his ear, without lymphadenopathy.
cytes and moderate number of eosinophils. Follicular The patient had no history of acne. At that time the epithelium showed cystic holes provoked by intracellu- diagnostic possibilities included: granulomatous rosa- lar and intercellular oedema (Fig. 2). Deposition of cea, demodicidosis, cutaneous tuberculosis, sarcoidosis, mucin in the follicular epithelium was demonstrated by Jessner–Kanof lymphocytic infiltrate, lupus erythema- Alcian blue stain. These findings confirmed the diagno- tosus, lymphocytoma cutis, lymphoma and syphilis.
sis of acneiform follicular mucinosis. Laboratory exam- Histopathological examination showed deep and ination revealed a normal full blood count, renal and superficial dermatitis with an inflammatory infiltrate liver function tests and chest X-ray.
Ó 2004 Blackwell Publishing Ltd • Clinical and Experimental Dermatology, 29, 396–398 Acneiform follicular mucinosis • E. M. C. Passaro et al.
We decided to treat the patient with oral predni- sone, 40 mg ⁄ day for 20 days. The patient improvedquickly and the lesions disappeared entirely (Fig. 1b).
The authors thank Prof Dr Cidia Vasconcellos, Dr Paulo Prednisone was gradually decreased by 10 mg weekly Ricardo Criado and Dr Mario Cesar Pires, Department of and was discontinued after 48 days. The patient has been followed up carefully for 7 months without Sa˜o Paulo, for their helpful comments.
Deposits of mucin in the follicles have been described in various dermatoses: alopecia areata,1,5 lichen planus,5melanocytic nevus,1,8 lentigo maligna,8 sarcoidosis,5 1 Bonta MD, Tannous ZS, Demierre MF et al Rapidly squamous cell carcinoma,9 lupus erythematosus,1,5,7,10 progressing mycosis fungoides presenting as follicular arthropod bites,1,4,5 pseudolymphoma5 and Hodgkin’s mucinosis. J Am Acad Dermatol 2000; 43: 635–40.
disease.5,11 Some authors have described follicular 2 Pereyo NG, Requena L, Galloway J et al Follicular mycosis fungoides: a clinicohistopathologic study. J Am Acad mucinosis with an acneiform clinical presentation1,3 but this raises the question of whether the deposition of 3 Wittenberg GP, Gibson LE, Pittelkow MR et al Follicular mucin around the follicles is primary or secondary.
mucinosis presenting as an acneiform eruption: report of We consider that further studies are necessary to clarify four cases. J Am Acad Dermatol 1998; 38: 849–51.
4 Abajo P, Martı´n R, Daude´n E. Follicular mucinosis associ- When the infiltrated lesions are generalized with a ated with cutaneous leishmaniasis. Acta Derm Venereol chronic course in patients over 30 years old the risk of association with MF is high. Histopathological features 5 Jackow CM, Papadopoulos E, Nelson B et al Follicular that draw attention to this include the presence of mucinosis associated with scarring alopecia, oligoclonal atypical lymphocytes, epidermotropism and a paucity of T-cell receptor Va expansion, and Staphylococcus aureus: eosinophils. However, there are reports of follicular when does follicular mucinosis become mycosis fungoides?J Am Acad Dermatol 1997; 37: 828–31.
mucinosis that progressed to MF in children or in 6 Yotsumoto S, Uchimiya H, Kanzaki T. A case of follicular patients with only a few isolated lesions on the face and mucinosis treated successfully with minocycline. Br J neck.1 Some authors have observed that MF associated with, or preceded by, follicular mucinosis is more 7 Klemke CD, Dippel E, Assaf C et al Follicular mycosis aggressive with lymph node involvement; histopatho- fungoides. Br J Dermatol 1999; 141: 137–40.
logical examination shows folliculotropism without 8 Summer WT, Grichnik JM, Shea CR et al Follicular muci- epidermotropism, i.e. follicular MF.2 The folliculotropic nosis as a presenting sign of acute myeloblastic leukemia.
variant of MF may require aggressive and distinct J Am Acad Dermatol 1998; 38: 803–5.
modalities of treatment because 7% of cases progress 9 Walchner M, Messer G, Rust A et al Follicular mucinosis in rapidly to lymph node involvement as compared with association with squamous cell carcinoma of the tongue.
less than 3% of classic MF cases.1,7 At present the J Am Acad Dermatol 1998; 38: 622–4.
10 Dawn G, Handa S, Kumar B. Follicular mucinosis and treatment options are electron beam radiation, immu- systemic lupus erythematosus. Dermatology 1997; 195: Patients with follicular mucinosis, whether children 11 von Kobyletzki G, Kreuter JA, Nordmeier R et al Treatment or adults, presenting with uncommon clinical variants of idiopathic mucinosis follicularis with UVA1 cold light must be followed up clinically and histologically to phototherapy. Dermatology 2000; 201: 76–7.
detect any evolution into cutaneous lymphoma.
Ó 2004 Blackwell Publishing Ltd • Clinical and Experimental Dermatology, 29, 396–398

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