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Case Reports
. 2021 Sep-Oct;66(5):574.
doi: 10.4103/ijd.IJD_595_20.

Case of Olmsted Syndrome with Essential Thrombocytosis Misdiagnosed as Acrodermatitis Enteropathica

Filiz Topaloğlu Demir  1 , Ceyda Çaytemel  2 , Nazlı Caf  2 , Zafer Türkoğlu  2 , Mesut Ayer  3 , Nesimi Büyükbabani  4
Affiliations
Case Reports

Case of Olmsted Syndrome with Essential Thrombocytosis Misdiagnosed as Acrodermatitis Enteropathica

Filiz Topaloğlu Demir et al. Indian J Dermatol. 2021 Sep-Oct.

Abstract

Olmsted syndrome is a rare genodermatosis. Palmoplantar keratoderma and periorificial keratodermic plaques are the most important clinical findings. Additional findings associated with a large number of systems may accompany such as teeth, nail deformities, alopecia, mental retardation, and bone-joint anomalies. Therefore, it is difficult to make a differential diagnosis from other palmoplantar keratodermas. It also needs to be differentiated from acrodermatitis enteropathica because of periorificial plaques. The absence of regression in lesions with zinc treatment excludes this disease. We present here an Olmsted syndrome case with essential thrombocytosis for the first time.

Keywords: Acrodermatitis enteropathica; Olmsted syndrome; essential thrombocytosis; mutilating keratoderma; palmoplantar keratoderma.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Hyperkeratotic verrucous plaques around the eyes, nose, and mouth and loss of hair follicles in scalp, eyebrows, and eyelashes
Figure 2
Figure 2
Sharp-limited hyperkeratotic plaques around the anus, scrotum, and penis
Figure 3
Figure 3
Bilateral palmar keratoderma extending from palmar surfaces to dorsum of the hands with flexion deformities of the digits
Figure 4
Figure 4
Significant hyperkeratosis, acanthosis, and focal parakeratosis on the biopsy taken from dorsal aspect of the hand (HEX400)
Figure 5
Figure 5
Intracorneal pustule formation, hyperkeratosis, acanthosis, and dermal intense inflammation on the perioral biopsy (HEX400)
See this image and copyright information in PMC

References

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