Kyrle’s disease (KD) and Flegel’s disease (FD) are rare variants
of primary perforating dermatoses, characterized by transepidermal elimination
of abnormal endogenous materials. In 1916, the Austrian dermatologist Josef
Kyrle described multiple hyperpigmented papules and nodules 5–10 mm in size
with a central keratotic plug in a 22-year-old diabetic woman. On the other hand,
Flegel disease is considered a genodermatosis with autosomal dominant
inheritance, manifesting in the third to fourth decade of life, although it has
been reported in patients as young as 13 years of age. We describe a 6-year-old
Yemeni girl with a 2-year history of generalized asymptomatic, small,
reddish-brown keratotic papules with a lenticular central keratotic plug. The
patient exhibited the clinical characteristics of FD; i.e., generalized small
lesions mainly over the face, trunk, and extensor surfaces of the extremities,
rather than the large lesions typically observed in KD. Although these features
are synonymous with FD, the histology of a punch biopsy was consistent with KD.
The patient was otherwise healthy, and no family members had a history of
similar diseases. The patient was diagnosed with KD-FD, owing to the
manifestation of features associated with both diseases. This case supports the
hypothesis that KD and FD are different manifestations or at least represent
different stages of the same disease.
Author
(s) Details
Mohammad
Ali Alshami
Dermatology Department, University Hospital, Sana’a University,
Sana’a, Yemen.
Mona
Jameel Mohana
Dermatology Department, University Hospital, Sana’a University,
Sana’a, Yemen.
Please see the book here:- https://doi.org/10.9734/bpi/dhrni/v5/2072
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