S Jayanthi

Giant shagreen patch associated with spina bifida occulta in tuberous sclerosis.

To the Editor: Although tuberous sclerosis was described over a century ago, it is only in the last decade that the full spectrum of the condition has been appreciated, and it is now frequently designated as the tuberous sclerosis complex (1). It is an autosomal dominant human genetic disease characterized by widespread hamartomas, usually occurring in the brain, eyes, skin, kidneys, liver, heart, and lungs (1,2). Its systemic nature was first recognized in the clinical triad of seizures, mental retardation, and facial lesions termed adenoma sebaceum (a misnomer) (3). Subsequent studies have led to recognition of the full clinical spectrum of abnormalities in the disease (2). We report an 11-year-old girl with tuberous sclerosis who had a giant shagreen patch in association with spina bifida occulta, rare association. To the best of our knowledge, such a large shagreen patch has not been described in the literature. An 11-year-old girl presented to our dermatologic clinic with an asymptomatic, gradually progressive area of pigmented, thickened skin over the lower back since birth. At the age of 8 years she noted multiple, asymptomatic, raised lesions on her face. She had generalized seizures since early childhood, for which she took antiepileptic medication. Her father was the only family member having a similar disorder. On physical examination, a healthy-looking girl had a large, yellowish, shiny plaque on her lumbosacral region measuring 19 cm · 13.5 cm (Fig. 1). The surface of the plaque was unevenly indurated and the underlying spine had kyphoscoliosis. In addition, she had adenoma sebaceum lesions over her cheeks (Fig. 2), a forehead plaque, and an ash-leaf macule over the abdomen. The oral cavity was normal except for enamel pits in the incisor teeth. Her intelligence was average and she showed good progress in school. Systemic examination was unremarkable. Routine hematologic and biochemical investigations were within normal limits. Histopathologic examination of a biopsy specimen from a representative skin lesion (back) showed a near-normal epidermis with the dermis having dense, broad, collagenous bundles in its lower part. These features were consistent with the clinical lesionof shagreenpatch.On skeletal survey, the skull film revealed a well-defined calcific density in the left parietal region and sclerosis of the skull vault. A radiograph of the lumbosacral region revealed kyphoscoliosis and spina bifida at S1 (Fig. 3). Computerized tomography

Chondroid syringoma of the ear lobe

1 Koga T, Kubota Y, Nakayama J. Syringocystadenoma papilliferum without an antecedent naevus sebaceous. Acta Derm Venereol 1999; 79: 237. 2 Patterson JW, Straka BF, Wick MR. Linear syringocystadenoma papilliferum of the thigh. J Am Acad Dermatol 2001; 45: 139–141. 3 Laube S, Suarez V, Hardwick N. A cluster of papules on the buttock of a teenager. Clin Exp Dermatol 2005; 30: 197– 198. 4 Saricaoglu H, Baskan EB, Ozuysal S, Tunali S. A case of syringocystadenoma papilliferum: an unusual localization on postoperative scar. J Eur Acad Dermatol Venereol 2002; 16: 534–536.

Tumor d'emblee responding to methotrexate and prednisolone

cm x 10 cm in size, ulcerated in the center and covered with greenish slough were seen over the medial aspect of both arms [Figure 1], and left forearm, left thenar eminence, and medial aspect of the left flank. The edges of the ulcers were raised and overhanging. The surrounding skin was violaceous and indurated. Two unulcerated nodules were also seen over the back. There was no significant regional lymphadenopathy. Systemic examination was unremarkable and no organomegaly was noted.