682 Correspondence ingested therefore equates to 25–30 mg of prednisolone, a from the second decade onwards.2–4 Other reported features ) reasonable adult dose and, for this child, 1 mg kg 1. There include associated hair shaft abnormalities (pili torti and trich- are no available data concerning the likely absorption of corti- orrhexis nodosa) admixed with hypotrichosis, prominent milia costeroid from a topical preparation such as Eumovate in a affecting the face, hypohidrosis, pinched nose with hypoplastic child of this age; however, it would be a coincidence if this nasal alae and prominent columella, atopic diathesis with event were not involved in the development of GPP in this comedones, keratosis pilaris, joint hypermobility, lingua plicata case. While unlikely to be a common occurrence in everyday and hyperpigmentation of the forehead.5,6 clinical practice it does appear that ingestion of topical steroid A 3-year-old girl presented at the age of 2 years with preparations may be a potential trigger for acute GPP. increasing numbers of multiple brown asymptomatic papules over the genital area and medial aspect of the thighs (Fig. 1a). Departments of Dermatology and *Paediatrics, S.D. ORPIN She had hypotrichia since birth (Fig. 1b), and had prominent Heartlands and Solihull NHS Trust (Teaching), J. HAFIJI facial milia and follicular atrophoderma of the cheeks Solihull Hospital, Lode Lane, Solihull, H.M. GOODYEAR* (Fig. 1c) consistent with a diagnosis of Bazex–Dupre´–Christol West Midlands B91 2JL, U.K. A. SALIM syndrome. Multiple members of her family showed similar E-mail: [email protected] features (the patient’s grandfather was originally reported by Gould and Barker in 19783) (Fig. 2). Her mother had features References of facial milia, follicular atrophoderma of the cheeks and dorsa of the hands, hypotrichia (since birth), hypohidrosis and axil- 1 Cassandra M, Conte E, Cortez B. Childhood pustular psoriasis elicited lary hidradenitis suppurativa. The patient’s newborn brother by the streptococcal antigen: a case report and review of the litera- was born with hypotrichia, mild erythema and ichthyosis and ture. Pediatr Dermatol 2003; 20:506–10. has subsequently developed multiple facial milia, suggesting 2 Zelickson B, Muller S. Generalised pustular psoriasis in childhood. that he also has Bazex–Dupre´–Christol syndrome. A biopsy J Am Acad Dermatol 1991; 24:186–94. 3 Beylot C, Puissant A, Bioulac P et al. Particular clinical features of from the labial area in our patient revealed multiple dermal psoriasis in infants and children. Acta Derm Venereol (Stockh) 1979; 59 nodules consisting of well-defined arrangements of rounded (Suppl. 87):95–7. nests of basaloid cells in a loose lobular (or ‘organoid’) pat- 4 Ryan T, Baker H. The prognosis of generalized pustular psoriasis. tern (Fig. 1d). A loose myxoid stroma without retraction arte- Br J Dermatol 1971; 85:407–11. fact was present surrounding the interconnecting strands of 5 Ryan T, Baker H. Systemic corticosteroids and folic acid antagonists basaloid cells (Fig. 1e). Well-formed papillary mesenchymal in the treatment of generalized pustular psoriasis. Evaluation and bodies, mitotic figures and necrosis were not seen. A diagnosis prognosis based on the study of 104 cases. Br J Dermatol 1969; 81:134–45. of multiple benign trichoepitheliomas occurring in Bazex– 6 Mengesha Y, Bennett M. Pustular skin disorders: diagnosis and treat- Dupre´–Christol syndrome was made. ment. Am J Clin Dermatol 2002; 3:389–400. Bazex–Dupre´–Christol syndrome is thought to be transmit- 7 Burden A. Management of psoriasis in childhood. Clin Exp Dermatol ted by an X-linked dominant pattern of inheritance.7 The 1999; 24:341–5. genetic defect has been reported to localize to Xq24-q27.8 8 Leman J, Burden A. Psoriasis in children: a guide to its diagnosis Neither trichoepitheliomas nor hidradenitis suppurativa have and management. Paediatr Drugs 2001; 3:673–80. been reported in association with Bazex–Dupre´–Christol 9 Demitsu T, Kosuge A, Yamada T et al. Acute generalised exanthema- tous pustulosis induced by dexamethasone injection. Dermatology syndrome. Most of the reported clinical features of Bazex– 1996; 193:56–8. Dupre´–Christol syndrome demonstrate abnormalities in the development of follicular structures, suggesting that the Conflicts of interest: none declared. defective gene codes for a protein intimately involved in folli- cular differentiation and development. Trichoepitheliomas are benign tumours which arise from cells derived from the hair follicle.9 Lesions may occur singly as a papule or nodule (up to 2 cm in diameter) or as multiple A case of Bazex–Dupre´–Christol syndrome 2–8 mm diameter skin-coloured papules on the face of chil- associated with multiple genital dren or young adults centred around the nasolabial folds and trichoepitheliomas preauricular regions. Trichoepitheliomas rarely affect the vul- val region.10 The major histological and clinical differential DOI: 10.1111/j.1365-2133.2005.06819.x diagnosis of trichoepithelioma includes basal cell carcinoma and trichoblastoma.9,11 There is considerable controversy SIR, Bazex–Dupre´–Christol syndrome was first described by regarding the histological definition of these follicular tumours Bazex et al. in 1964.1 It is characterized by a triad of congenital and differentiation between these entities can be difficult, hypotrichosis, follicular atrophoderma (affecting the dorsa of although various features are reported to be helpful in differ- the hands and feet, the face, and extensor surfaces of the entiating trichoepitheliomas from basal cell carcinomas.11,12 elbows or knees) and the development of basocellular Furthermore, it remains unclear whether basal cell carcinomas neoplasms (including basal cell naevi and basal cell carcinomas) may arise from trichoepitheliomas.9 Ó 2005 British Association of Dermatologists British Journal of Dermatology 2005 153, pp664–699 Correspondence 683 Fig 1. (a) Multiple trichoepitheliomas (multiple brown flat-topped papules) affecting the genital area and inner thighs. (b) Hypotrichia as a neonate. (c) Prominent facial milia with early follicular atrophoderma of the cheeks. (d) Scanning low power view of trichoepithelioma showing loosely organized lobules of basaloid cells (organoid pattern) with loose myxoid stroma but lacking retraction artefact around the lobules (haematoxylin and eosin; original magnification · 2). (e) High power view of basaloid cells showing unusual myxoid stroma without retraction artefact. Mesenchymal bodies, mitotic figures and necrosis are absent (haematoxylin and eosin; original magnification · 20). suggests that the genetic mutation responsible for Bazex–Dup- re´–Christol syndrome may also have pleiotropic effects respon- sible for the multiple trichoepitheliomas seen in our case. It is unclear whether our patient will go on to develop basal cell carcinomas (or cylindromas and spiradenomas). We report what we believe to be the first case of Bazex– Dupre´–Christol syndrome associated with trichoepitheliomas and hidradenitis suppurativa. Bazex–Dupre´–Christol syndrome is associated with the development of basal cell carcinomas; however, it is unknown whether they arise from previously undiagnosed trichoepitheliomas. Department of Dermatology, A. YUNG Leeds General Infirmary, J.A. NEWTON-BISHOP* Fig 2. Updated pedigree of family reported by Gould and Barker.3 Leeds LS1 3EX, U.K. BCC, basal cell carcinoma; HS, hidradenitis suppurativa; TE, multiple *Department of Dermatology, trichoepitheliomas. St James’s University Hospital, Leeds LS9 7TF, U.K. Familial multiple trichoepithelioma is inherited as a auto- E-mail: [email protected]; 13 somal dominant disorder. The association of multiple famil- [email protected] ial trichoepitheliomas with basal call carcinoma, cylindroma and occasionally eccrine spiradenoma is denoted Brooke– References Spiegler syndrome, a autosomal dominant disorder due to loss of heterozygosity at 9p21.14 In contrast, sporadic trichoepitheli- 1 Bazex A, Dupre´ A, Christol B. Ge´nodermatose complexe de type in- oma may be associated with loss of heterozygosity at 9p22.3, de´termine´ associant une hypotrichose, un e´tat atrophodermique a common site of genetic defects found in basal cell carcino- ge´ne´ralise´ et des de´ge´ne´rescences cutane´es multiples (epitheliomas- mas.15–17 Recent studies in Chinese families with multiple basocellulaires). Bull Soc Franc Derm. Syph. 1964; 71:206. 2 Goeteyn M, Geerts ML, Kint A et al. The Bazex–Dupre´–Christol syn- trichoepitheliomas (without cylindromatosis) have demonstra- drome. Arch Dermatol 1994; 130:337–42. ted genetic defects at 16q12-q13, the site of the recessive cyl- 3 Gould DJ, Barker DJ. Follicular atrophoderma with multiple basal indromatosis oncogene CYLD, the tumour suppressor gene cell carcinomas (Bazex). Br J Dermatol 1978; 99:431–5. responsible for the development of cylindromatosis. This 4 Kidd A, Carson L, Gregory DW et al. A Scottish family with Bazex– observation confirms that different genetic mutations may Dupre´–Christol syndrome: follicular atrophoderma, congenital manifest with identical phenotypic expression.18–20 This hypotrichosis, and basal cell carcinoma. J Med Genet 1996; 33:493–7. Ó 2005 British Association of Dermatologists British Journal of Dermatology 2005 153, pp664–699 684 Correspondence 5 Glaessl A, Hohenlautner U, Landthaler M et al. Sporadic Bazex–Dup- of the ulcer is clean and granulating, and borders are not re´–Christol–like syndrome: early onset basal cell carcinoma,