The Kleeblattschädel Phenomenon: Sign or Syndrome?

Abstract

Syndrome" may be misleading to pediatricians not familiar with the dermatological nomenclature of epidermal nevi. The authors should have referred to three fairly recent articles which would have clarified the diagnosis of the patients presented.2-4From the pictures presented in the article by Lansky et al, it would appear that the patients had extensive epidermal nevi, but not necessarily of the type referred to as "sebaceous" or nevus sebaceus of Jadassohn, which has a characteristic histologic pattern and usually occurs on the head and neck as single lesions. More extensive presentations of sebaceous nevi as well as linear varieties have been reported but are uncommon. No histologic evidence for this type of nevus is presented in this paper.It would seem that the authors are describing children who fall into the type of neurocutaneous disease outlined by Solomon as epidermal nevus syndrome. Therefore, the title linear sebaceous nevus syndrome may be misleading. SHARON LANTIS, MD Madison, Wis References 1. Lansky LL, et al: Linear sebaceous nevus syndrome: A hamartoma variant. Am J Dis Child 123:587-590, 1972. 2. Solomon LM, Fretzin DF, Dewald RL: The epidermal nevus syndrome. Arch Dermatol 97:273-285, 1968. 3. Lantis SJ, et al: Nevus sebaceus of Jadassohn: Part of a new neurocutaneous syndrome. Arch Dermatol 98:117-123,1968. 4. Mehregan AH, Pinkus H: Life history of organoid nevi. Arch Dermatol 91:574\x=req-\ 588, 1965.We attempted to clarify existing nomenclature utilized in describing linear epidermal nevi and Dr. Lantis' letter illustrates the lack of uniformity in classification of the dermatological entities. The references quoted by Dr. Lantis were reviewed by us and only because of space limitations, were not included in the reference section. Biopsies were performed in all three cases and histologic findings of hyperkeratosis and parakeratosis were common to all.We wish to emphasize that the linear sebaceous nevus syndrome is representative of one of several types of linear epidermal nevi. Classification of neurocutaneous syndromes into groups and subgroups is far less important than attempting to delineate the pathogenesis of these entities. Furthermore, we do not believe that the nomenclature referred to in the article will mislead or create confusion, particularly among pediatricians. Dr. Lantis appears to be engaged in a battle of semantics and has completely overlooked the authors' intent to correlate the development of hamartomas of multiple or¬ gans occurring in association with sebaceous nevi and central nervous system dysfunction. It might be well to remember that "confusion exists in the eves of the beholder."

Short communications In Utero diagnosis of Kleeblattschädel (cloverleaf skull)

“…Although originally described as a syndrome (Holtermueller and Wiedemann, 1960), there have been proposals that the Kleeblattschadel deformity is more likely an anomaly which may be seen in association with severe mental retardation, multiple bony abnormalities and a variety of other syndromes (Cohen, 1972;Young ef al.. 1973;Angle et al, 1967;Feingold et al, 1969, Feingold ef al., 1973. Several reports indicate an association of KleebiattschHdel with Crouzon disease (Hall er a/., 1972; Feingold er a]., 1969).…”

Letters to the Editor

“…Several authors (Hall et al 1972, Cohen 1972, Feingold et al 1973) believe that cloverleaf skull syndrome/anomaly does not constitute a specific syndrome, but that the anomaly is associated with a variety of syndromes. The chromosomal abnormality in this particular patient could be a coincidence, but it is possible that the cloverleaf skull deformity could be associated with a partial trisomy of the long arm of chromosome 15.…”