Mancheno-Valencia A, Flores-Peña LG, Toussaint-Caire S, Díaz-Lozano M, Rosas-Manzano A, Fonte-Ávalos V, Vega-Memije ME

Language: English
References: 13
Page: 73-76
PDF size: 258.72 Kb.

ABSTRACT

Cutaneous syndromes associated to malformations or pathologies of other organs are relatively common, but diverse with regard to their manifestations. In cases with uncommon features the diagnostic process may be difficult, and the histopathological analysis could be the cardinal step in order to determinate the correct diagnosis. We report the case of a man with a disseminated dermatosis affecting the right side of the body, characterized by brown papules linearly arranged in association to extracutaneous defects (leiomyomatosis peritonealis disseminata, bilateral brachydactylia, shortening of the right pelvic member, genu varum, hypoacusia). A histopathological study was performed showing findings compatible with the diagnosis of basaloid follicular hamartoma. Posteriorly he was diagnosed of symptomatic epilepsy. Basaloid follicular hamartoma is a rare follicular malformation that has been described as an isolated entity or in association with systemic alterations. Previous reports have described cases with similar characteristics to the one we are subjecting, and there is a proposition to group these cases as part of a spectrum of a distinct phenotype.

REFERENCES

1. Adams D, Athalye L, Schwimer C, Bender B. A profound case of linear epidermal nevus in a patient with epidermal nevus syndrome. J Dermatol Case Rep. 2011; 5: 30-33. PMID: 21894253.

2. Gon-Ados S, Minelli L, Franzon PG. Case for diagnosis. Inflammatory linear verrucous epidermal nevus. An Bras Dermatol. 2010; 85: 729-731. PMID: 21152805.

3. Vujevich JJ, Mancini AJ. The epidermal nevus syndromes: multisystem disorders. J Am Acad Dermatol. 2004; 50: 957-961. PMID: 15153903.

4. Mills O, Thomas LB. Basaloid follicular hamartoma. Arch Pathol Lab Med. 2010; 134: 1215-1219. PMID: 20670146.

5. Lee MW, Choi JH, Moon KC, Koh JK. Linear basaloid follicular hamartoma on the Blaschko’s line of the face. Clin Exp Dermatol. 2005; 30: 30-34. PMID: 15663498.

6. Díaz-Martínez B, Izquierdo-Estirado M, Alramadan M, Menéndez-Ramos F, Buedo I, Arrieta-Peña E. Hamartoma folicular basaloide lineal unilateral. Med Cutan Iber Lat Am. 2009; 37: 251-256.

7. Brown AC, Crounse RG, Winkelmann RK. Generalized hair-follicle hamartoma, associated with alopecia, aminoacidura, and myasthenia gravis. Arch Dermatol. 1969; 99: 478-493. PMID: 4238829.

8. Delacretaz J, Balsiger F. Multiple familial follicular hamartoma (author’s transl). Dermatologica. 1979; 159: 316-324. PMID: 478069.

9. Kato N, Ueno H, Nakamura J. Localized basaloid follicular hamartoma. J Dermatol. 1992; 19: 614-617. PMID: 1491090.

10. Mehregan AH, Baker S. Basaloid follicular hamartoma: three cases with localized and systematized unilateral lesions. J Cutan Pathol. 1985; 12: 55-65. PMID: 3973186.

11. Jimenez-Acosta FJ, Redondo E, Baez O, Hernandez B. Linear unilateral basaloid follicular hamartoma. J Am Acad Dermatol. 1992; 27: 316-319. PMID: 1517495.

12. Happle R, Tinschert S. Segmentally arranged basaloid follicular hamartomas with osseous, dental and cerebral anomalies: a distinct syndrome. Acta Derm Venereol. 2008; 88: 382-387. PMID: 18709310.

13. Itin PH. Happle-Tinschert syndrome. Segmentally arranged basaloid follicular hamartomas, linear atrophoderma with hypo- and hyperpigmentation, enamel defects, ipsilateral hypertrichosis, and skeletal and cerebral anomalies. Dermatology. 2009; 218: 221-225. PMID: 19005246.