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Case Reports
. 2022 Jul 18;10(7):10.1002/ccr3.6098.
doi: 10.1002/ccr3.6098. eCollection 2022 Jul.

Unusual facial lesions in H syndrome

Mariem Rekik  1 , Emna Bahloul  1 , Mohamed Ben Rejeb  2 , Khadija Sellami  1 , Slim Charfi  3 , Hamza Chouk  4 , Tahya Boudaouara  3 , Hamida Turki  1
Affiliations
Case Reports

Unusual facial lesions in H syndrome

Mariem Rekik et al. Clin Case Rep. .

Abstract

H Syndrome is a rare genodermatosis. It may include facial involvement such as: facial telangiectasia, both hypo- and hyperpigmented lesions, hirsutism, swollen cheeks due to subcutaneous infiltration and eczematous lesions. We describe a new facial phenotype with dermoscopic and histological features in the spectrum of non-Langerhans cell histiocytosis.

Keywords: H Syndrome; dermoscopy; facial lesions; non‐Langerhans cell histiocytosis.

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Conflict of interest statement

None.

Figures

FIGURE 1
FIGURE 1
(A): Finger flexion contracture (camptodactyly) (B): Deformation and disorganization of teeth; (C), (D): Symmetrical, hyperpigmented, and thickened patches with hypertrichosis in the inner thighs, pubic, and lumbar regions
FIGURE 2
FIGURE 2
Erythematous, annular, and figurate lesions slightly keratotic without atrophy in cheeks and nose
FIGURE 3
FIGURE 3
Dermoscopic image showing multiple telangiectasias drawing a reticulated network
FIGURE 4
FIGURE 4
Lymphocytic and perivascular histiocytic infiltrate of the dermis (HE ×400)
FIGURE 5
FIGURE 5
Anti‐CD68 immunostaining revealing the presence of numerous perivascular histiocytes (CD68 ×200)
See this image and copyright information in PMC

References

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