On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.84 no.1 Rio de Janeiro Jan./Feb. 2009
Alesso Cervantes SartorelliI; Francisco Eduardo M. LeiteII; Isabel Violeta de Carvalho FriedmanIII; Horácio FriedmanIV
Laboratório Diagnose - Brasilia (DF), Brazil
IIDermatologist - Brasilia (DF), Brazil
IIIPathologist, Laboratório Diagnose - Brasilia (DF), Brazil
IVFull Professor, Department of Pathology, Medical School, Universidade de Brasilia - Brasilia (DF), Brazil
Tumor of the follicular infundibulum is an uncommon cutaneous lesion with different forms of clinical presentation, namely solitary and multiple/eruptive variants. The former shows predilection for head and neck and presents as a papulonodular scaly tumor. The latter is less frequent and occurs on facial, neck and upper chest areas as brown, reddish or more commonly hypopigmented macules leading to the differential diagnosis with vitiligo. The different clinical forms share the same histopathologic aspect. The present report describes a case of the multiple variant of tumor of the follicular infundibulum disclosing facial vitiligoid macules in a 35-year-old male patient.
Keywords: Hypopigmentation; Neoplasms, Adnexal and skin appendage; Skin neoplasms
Follicular infundibulum tumor (FIT) or infundibuloma, described in 1961 by Mehregan and Butler, is an uncommon tumor found in less than 10/100,000 skin biopsies, but it has different forms of clinical presentation: 1) solitary; 2) multiple or eruptive; 3) associated with Cowdens disease, sebaceous nevus, Schopf-Schulze-Passarge syndrome, and 4) associated with other skin tumors 1-7. Single lesion is common, preferably in middle-aged women and located on the head and neck, asymptomatic and papulonodular desquamative, measuring 1 to 2 cm simulating a basal cell carcinoma. The eruptive/ multiple form (infundibulomatosis), described by Kossard, is rare, with multiple lesions on the face, neck and upper portion of the trunk, symmetrical, with aspect of hypopigmented macules or papules, light brown or red 3, 8, 9. Sun exposure accentuates the multiple hypopigmented lesions that remain like that while the surrounding skin is tanned. The solitary and multiple variants have similar microscopic aspect, with proliferation in fenestrated plaque of pale keratinocytes organized in anastomosed trabeculae connected to the epidermis. The present case illustrates the multiple hypopigmented lesions: the uncommon vitiligoid form of the tumor.
Male 35-year-old patient, had facial hypopigmented macules for 3 years, symmetric and asymptomatic. Woods glass highlighted the lesions (Figure 1), one of which was submitted to biopsy. Microscopic analysis revealed fenestrated plaque connected to the epidermis basis and formed by anastomosed trabeculae of pale keratinocytes (Figure 2A), containing glycogen inside them (Figure 2B). Fontana-Masson staining showed absence of melanin pigmentation in the lesion (Figure 3A). Adjacent epidermis showed preserved pigmentation (Figure 3B). CO2 laser management repigmentation and improvement of lesion texture. Later, we received skin biopsy result showing very similar microscopic aspect, which we learned was from the patients brother. The dermatologist learned that other family members had clinically similar lesions. However, the patient and his family members refused to undergo treatment or follow-up.
The present discussion is focused on the multiple form of FIT. Clinical aspect of normally different, enabling diagnostic suspicion 4. However, some cases have small and few lesions, difficult to visualize, which leads to underdiagnosing 4. FIT has microscopic aspect which is normally typical, with well circumscribed lesion, formed by pale keratinocytes, containing glycogen and proliferating from the epidermal basis so as to form a fenestrated plaque of anastomosed trabeculae. The desmoplastic variant and sebaceous or eccrine differentiation have been rarely described 2, 10, 11. There are many elastic fibers on the adjacent dermis. Hair follicles present in the area are involved by the lesion but do not produce follicular structures. Both multiple and solitary cases are considered benign 3. Evident basal membrane, broad pale cytoplasm, and absence of mitosis, atypias and apoptosis normally facilitate the differential diagnosis with basal cell carcinoma. Immunohistochemical reaction to BerEp4 for basal cell carcinoma is negative for FIT .2, 6, 12, 13 However, the rare transformation of FIT into basal cell carcinoma has already been described 2, 3, 4, 14. In the present case, the clinical presentation directed the differential diagnosis to macular hypopigmented lesions, including vitiligo. The histopathological aspect was typical of FIT - hypopigmentation could be explained by lack of melanin in the lesion, as shown by Fontana-Masson staining, previously described by Cribier and Grosshans.4 To bring together clinical suspicion of vitiligoid lesions with histopathological diagnosis of tumor lesion is a rare situation, but characteristics of FIT. Even though the genetic basis has not been established, the presence of more than one family member with histologically identical lesion and occasional association with other genetic-known syndromes favor the hypothesis of a possible underlying genetic defect 2, 3, 15. It is recommendable to have periodical follow-up of patients, considering the impossibility of having complete treatment, because of the cosmetic implications and the remote possibility of transformation into basal cell carcinoma.
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Mailing Address: How to cite this article: Sartorelli AC, Leite
FEM, Friedman IVC, Friedman H. Máculas hipopigmentadas vitiligóides
e tumor do infundíbulo folicular. An Bras Dermatol. 2008;84(1):68-70.
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How to cite this article: Sartorelli AC, Leite FEM, Friedman IVC, Friedman H. Máculas hipopigmentadas vitiligóides e tumor do infundíbulo folicular. An Bras Dermatol. 2008;84(1):68-70.