A case of eccrine epithelioma is presented. A 62-year-old woman was seen for with a lesion on the left forearm present for 36 years, and complicated by multiple recurrences. Histopathologically, it was composed of numerous small nests of basaloid cells, cystic and syringoid epithelial structures within the reticular dermis and invading the contiguous fat and muscle tissues. Tubular structures and cystic spaces contained PAS-positive, diastate-resistant, and alcian blue-positive material. Although the tumor was very aggressive locally, no metastasis had occurred throughout its long course.
Two children were seen who had cutaneous lesions of varicella localized to sunburned and suntanned skin. Atypical features were observed in both cases. Based on certain hypotheses, we concluded that sun exposure may modify the clinical picture of varicella.
Congenital lower lip pits are a rare developmental malformation of the lower lip. Clinically they present as bilateral depressions in the vermilion zone of the lower lip. It is important to be aware of this disorder because lower lip pits have also been reported in a variety of other congenital disorders and are associated with other malformations. Ten cases of this entity are presented. Nine of the 10 patients are members of two related families. The clinical and pathologic picture as well as some of the genetic and therapeutic aspects of this peculiar condition are discussed.Congenital fistulas of the lower lip are congenital malformations that were first described by Demarquay in 1845 (1). This entity is also known under the eponym "Van der Woude syndrome" because the more recent extensive description by Van der Woude in 1954 (2). Their clinical relevance is based on their frequent association with cleft lip and cleft palate, and less commonly with other congenital anomalies. A series of 10 patients is presented, 9 of whom were members of two related families. The remaining patient had no affected relatives.
CASE REPORTS
Patient 1A 2-year-old boy had bilateral depressions on both sides of the midline of the lower lip present since birth. The lesions were asymptomatic. His parents reported no secretions from the pit. Physical examination revealed no other anomalies of the oral cavity, periauricular region, or rest of the body. Pregnancy and delivery had been uneventful. No first-or second-generation relatives exhibited similar lesions.
Patient 2A 20-year-old woman presented with dryness and fissures affecting the lower lip. The patient ascribed these lesions to sporadic watery secretions from the base of a pair of bilateral labial pits present since birth (Fig. 1). On examination, a stylet could be advanced several millimeters into the pits, and compression of the margins produced a saliva-like secretion. The lesions were excised, and the histologic study revealed an invagination that progressively thinned toward the base (Fig. 2). The lining consisted of stratified epithelium similar to the epithelium lining the lips. Intracellular edema and abundant clear and vacuolated cells were observed.
Patients 3-10A family study showed the presence of identical lesions in a younger male sibling of patient 2, their maternal aunt, and five of her living offspring. Three of these offspring moreover had a cleft palate, while one had both a cleft palate and cleft lip (Figs. 3 and 4).
We report a 26 year-old man with bilateral progressive palmoplantar keratoderma, automutilation of the 3rd, 4th and 5th toes of the left foot, and recurrent episodes of gingivitis in childhood. The patient had signs common to both Papillon-Lefevre and Vohwinckel syndromes. Acitretin was given (30 mg/day) with good results.
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