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A man with leonine facies
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  1. Mahmudur Rahman Siddiqui1,
  2. F M Siddiqui2
  1. 1Department of Internal Medicine, Dhaka Medical College Hospital, Dhaka, Bangladesh
  2. 2Department of Internal Medicine, Institute of Epidemiology Disease Control and Research (IEDCR), Dhaka, Bangladesh
  1. Correspondence to Dr Mahmudur Rahman Siddiqui, dr.mahmud99{at}yahoo.com

https://doi.org/10.1136/bcr.10.2011.5057

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    Description

    A 42-year-old man presented with gradually progressive thickening of his scalp, face and extremities. He also complained of hyperhidrosis of palms and sole. On examination, he had coarse ‘leonine like’ facies, with thickened folded skin of face, eyelids and scalp with mechanical partial ptosis (figure 1). Scalp examination revealed typical gyrate appearance (cutis verticis gyrate). He had ‘spade like’ hands and feet. There was moderate clubbing of the fingers and toes, but the hands had ‘claw like’ appearance due to a persistent flexion of the interphalangeal joints (figures 2–4). X-ray of the wrists and ankles revealed a faint radiolucent line beneath the new periosteal bone along the shaft of long bones at their distal ends. X-ray chest and skull were normal. Slit skin smear for leprosy was negative and growth hormone assay was normal. He is the only child of healthy consanguineous parents. Other affected members are not described in this family. All these features are typical of ‘pachydermoperiostosis’ (PDP). PDP, also known as Touraine–Solente–Gole’ syndrome, is a genetic disorder and has an autosomal recessive or dominant (variable expression) inheritance pattern.1 2 The condition usually begins insidiously at puberty and is characterised by pachydermia (thickened skin of face and extremities, thick and corrugated scalp), periostosis (excessive bone formation) and finger clubbing.2 Because of thickness and heaviness of eyelids, mechanical ptosis can occur. This is often known as idiopathic or primary hypertrophic osteoarthropathy to distinguish it from secondary or pulmonary hypertrophic osteoarthropathy.1 3 PDP is usually confused with acromegaly and leprosy.3

    Figure 1
    Figure 1

    A leonine like appearance of the face mimics lepromatous leprosy.

    Figure 2
    Figure 2

    Clubbing of the fingers.

    Figure 3
    Figure 3

    Claw like’ appearance of the fingers.

    Figure 4
    Figure 4

    Spade like hand with clawing.

    References

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      1. Rastogi R,
      2. Suma GN,
      3. Prakash R,
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      . Pachydermoperiostosis or primary hypertrophic osteoarthropathy: A rare clinicoradiologic case. Indian J Radiol Imaging 2009;19:123–6.
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    2. ↵
      1. Castori M,
      2. Sinibaldi L,
      3. Mingarelli R,
      4. et al
      . Pachydermoperiostosis: an update. Clin Genet 2005;68:477–86.
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    3. ↵
      1. Tolat SN,
      2. Gharpuray MB,
      3. Dhobale SM
      . Pachydermoperiostosis: the disease and its disfigurement. Indian J Dermatol Venereol Leprol 1994;60:369–71.
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    View Abstract

    Footnotes

    • Competing interests None.

    • Patient consent Obtained.