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Calderón Herschman, Perla Estrella

González Cuevas, Rubén Wladimir

Ortega Peña, Macarena

Peruilh Bagolini, Leonardo Andrés

Vargas Mora, Pablo Andrés

Ruben González‐Cuevas

Leonardo Peruilh‐Bagolini

Perla Calderón

Macarena Ortega‐Peña

Pablo Vargas‐Mora

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A single plaque on the chin

Repositorio Académico de la Universidad de Chile

Pediatric Dermatology. 2021;38:e3–e4. wileyonlinelibrary.com/journal/pde   |  e3PediatricDermatology© 2020 Wiley Periodicals LLC. DOI: 10.1111/pde.14441  P H O T O Q U I ZA single plaque on the chinRuben González-Cuevas MD1  |   Leonardo Peruilh-Bagolini MD2 |   Perla Calderón MD3 |   Macarena Ortega-Peña MD4 |   Pablo Vargas-Mora MD21Dermatology Service, Hospital del Salvador, Santiago, Chile2Department of Dermatology, Facultad de Medicina, Universidad de Chile, Santiago, Chile3Dermatology Service, Hospital Clinico Universidad de Chile, Santiago, Chile4Escuela de Medicina, Universidad Mayor, Santiago, ChileCorrespondence: Rubén González MD, Manuel Montt, Depto 905, Providencia, Santiago 7500599, Chile.Email: ruben.gonzalez.c@gmail.comKeywords: follicular, follicular mucinoses, lymphoma, mucinoses, mucinosis, mycosis fungoidesC A SE REPORTA 16-year-old girl, without significant past medical history was eval-uated for a painless skin lesion on her chin. The lesion had appeared 2 months prior and had shown no response to topical tacrolimus 0.1% ointment twice daily for 2 weeks. The patient did not recall any prior trauma.Physical examination revealed a solitary, pink plaque, measuring 2 cm in diameter with irregular borders, rough surface, and a raised center located on the right side of her chin (Figure 1). The rest of clinical examination was unremarkable. A skin biopsy was performed (Figures 2-4).WHAT IS THE DIAGNOSIS?The brightness and contrast of figures have been adjusted, following the ethical considerations of the journal. F I G U R E  1    F I G U R E  2    F I G U R E  3    F I G U R E  4    e4  |     PediatricDermatologyGONZÁLEZ-CUEVAS Et AL.Diagnosis: Primary follicular mucinosisDISCUSSIONHematoxylin and eosin stain showed the epidermis with mild spon-giosis and presence of dilated hair follicles with intrafollicular lym-phocytes and a peripheral infiltrate composed of lymphocytes and eosinophils (Figures 2 and 3). Alcian blue stain revealed prominent intrafollicular mucin deposition (Figure 4). Treatment with one intral-esional injection of betamethasone resulted in complete disappear-ance of the plaque.Follicular mucinosis (FM) is a rare inflammatory disorder of un-known cause, characterized by mucin deposition within the follicu-lar epithelium and sebaceous glands. Numerous classifications have been described for FM, but it is mainly categorized into two types. The benign idiopathic, or primary FM, typically occurs in patients from 11 to 35 years (mean 20.4 years).1 Primary FM may appear as acute or subacute plaques composed by clustered follicular papules, sometimes showing scaling. It usually favors the face and neck and is associated with alopecia. Primary FM is usually self-limiting, and most cases heal spontaneously after 2 to 24 months.2Secondary FM is associated with inflammatory conditions in-cluding lupus erythematosus, insect bites, eczema, alopecia areata, and hypertrophic lichen planus, or malignant diseases such as my-cosis fungoides, Sezary syndrome, leukemia cutis, cutaneous B-cell lymphoma, and Hodgkin's disease.3-5 In children, primary FM is more common than malignancy associated FM.6It is important to differentiate primary FM from the follicu-lar variant of mycosis fungoides (FMF).2,7 The definitive diagnosis should take into account a correlation between clinical findings (age, location and number of lesions, and response to treatment), histo-pathology, and immunohistochemistry. The diagnosis of primary FM is favored by an early age onset, single mildly infiltrated plaques of confluent follicular papules, located on the head and neck, with or without alopecia, and fast improvement with treatment. In con-trast, FMF is more frequent in adults, commonly presenting as multiple, large, hypo-, or hyperpigmented erythematous infiltrated plaques with overlying follicular papules and alopecia, on any loca-tion, without sustained improvement to conventional treatments.8 Histopathology provides additional information, as it reveals mixed inflammatory infiltrate and mucin within hair follicles, with mild or absent fibrosis in FM, while in FMF the infiltrate may reveal atyp-ical T lymphocytes, with epidermotropism and folliculotropism, sometimes forming intraepidermal clusters (Pautrier microabscess). Immunohistochemistry of the T lymphocytes in most FMF cases shows loss of CD5+ and CD7+ expression, and CD4+ cells outnum-bering CD8+ cells.2,7-9Other diagnoses in the differential of primary FM include kera-tosis pilaris, lichen spinulosus, alopecia areata, and allergic contact dermatitis and a skin biopsy is often required for the diagnosis.10 Despite being a benign condition, monitoring patients with FM for cutaneous T lymphoma is advised, especially in persistent cases, al-though the progression toward MF has not been observed in some studies.1There is no specific treatment for primary FM and treatment mo-dalities are variable, but initial treatment consists of mild-to-mod-erate potency topical corticosteroids. Systemic and intralesional corticosteroids, topical pimecrolimus, PUVA, dapsone, hydroxychlo-roquine, minocycline, isotretinoin, and imiquimod have also induced improvement of lesions.11ORCIDRuben González-Cuevas  https://orcid.org/0000-0003-1707-7373 R E FE R E N C E S 1. Khalil J, Kurban M, Abbas O. Follicular mucinosis: a review. Int J Dermatol. 2020. https://doi.org/10.1111/ijd.15165. [Epub ahead of print]. 2. Lewars M, Levin J, Purcell S. Follicular mucinosis. Indian Dermatol Online J. 2013;4(4):333-335. 3. Akinsanya AO, Tschen JA. Follicular mucinosis: a case report. Cureus. 2019;11(5):e4746. 4. Passos PC, Zuchi MF, Fabre AB, Martins LE. Follicular mucinosis - case report. An Bras Dermatol. 2014;89(2):337-339. 5. Rongioletti F, De Lucchi S, Meyes D, et al. Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and mo-lecular study comparing the primary benign form and the my-cosis fungoides-associated follicular mucinosis. J Cutan Pathol. 2010;37(1):15-19. 6. Bauer FJ, Almeida JRP, Sementilli A, Mattos E Dinato SL. Idiopathic follicular mucinosis in childhood. An Bras Dermatol. 2020;95(2):268-270. 7. Zvulunov A, Shkalim V, Ben-Amitai D, Feinmesser M. Clinical and histopathologic spectrum of alopecia mucinosa/follicular mu-cinosis and its natural history in children. J Am Acad Dermatol. 2012;67(6):1174-1181. 8. Emge DA, Lewis DJ, Aung PP, Duvic M. How to discern folliculo-tropic mycosis fungoides from follicular mucinosis using a pediatric case. J Cutan Med Surg. 2018;22(3):336-340. 9. Hempstead RW, Ackerman AB. Follicular mucinosis. A reaction pat-tern in follicular epithelium. Am J Dermatopathol. 1985;7(3):245-257. 10. Bella-Navarro R, Martí-Fajardo N, Martín-Hernández JM, Jordá-Cuevas E. Follicular mucinosis in childhood: a case report and re-view of the literature. Actas Dermosifiliogr. 2012;103(4):335-336. 11. Alonso de Celada RM, Feito Rodriguez M, Noguera Morel L, Beato Merino MJ, De Lucas LR. Treatment of primary follicular mucinosis with imiquimod 5% cream. Pediatr Dermatol. 2014;31(3):406-408.

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A single plaque on the chin

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