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A forgotten old disease

Published:February 24, 2017DOI:https://doi.org/10.1016/j.ejim.2017.02.018

      Keywords

      1. Indication

      An 81-year-old diabetic woman, presented to the out-patient clinic with a 10-year history of glove-like anesthesia in both hands. This neurologic deficit was reported to be accompanied by the spontaneous appearance of blisters on both palms that soon turned into ulcers and then healed only to appear again weeks later. She also disclosed incomplete anesthesia of the face, earlobes, and retroauricular region along with xerodermia, hypohidrosis, and difficulty on closing the eyelids. She reported no other skin lesions on any other part of her body. On physical examination both hands showed claw deformity with palm flattening due to severe thenar and hypothenar atrophy, together with finger shortening and distal phalangeal reabsorption on several of them (Fig. 1, Panel A and B). The sensory deficit compromised the palmar side of both hands but respected the territory of the radial nerve. Cubital nerve thickening was evident on palpation of the left wrist, and, Froment and bottle signs were positive on both hands. On facial examination, gleaming skin, ptosis, lagophthalmos, ectropion, partial madarosis of the lower eyelashes, diffuse skin thickening, and deep facial expression lines were noted (Panel C). Previously, the neurologic deficit on upper extremities and the skin thickening were adjudged to diabetic neuropathy, but glicosilated hemoglobin was normal.
      Fig. 1
      Fig. 1Panel A: Palm flattening due to severe thenar and hypothenar atrophy, associated with blisters and ulcers on both palms.
      Panel B: Claw deformity, together with finger shortening and distal phalangeal reabsorption on several of them.
      Panel C: Gleaming skin, lagophthalmos, ectropion, partial madarosis of the lower eyelashes, diffuse skin thickening, and deep facial expression lines.
      Panel D: Ziehl-Nielsen stain 1000×, showing abundant Hansen bacilli grouped on globi.

      2. What is the diagnosis?

      2.1 Diagnosis

      Skin and lymph smears were obtained from hands, elbows, knees, and earlobes, where Ziehl-Nielsen stains showed abundant Hansen bacilli grouped on globi (Panel D), with a bacillary index of +5,5. The patient was treated with a 1-year course of Dapsone, Rifampin and Clofazimine, following the WHO recommendations for multibacillary leprosy, presenting clinical improvement of the skin lesions but not of the neurologic deficit.
      Leprosy is a chronic, infectious disease caused by Mycobacterium leprae. It mainly affects the peripheral nervous system, skin, and certain other tissues such as the reticulo-endothelial system, bones and joints, mucous membranes, eyes, testes, muscles, and adrenals [
      • Talhari C.
      • Talhari S.
      • Penna G.O.
      Clinical aspects of leprosy.
      ]. Leprosy clinical presentation varies from few to widespread lesions. Cutaneous and peripheral nerve trunks are frequently invaded by M. leprae, the consequences of this invasion will depend on the affected nerves, individual immunological response, type of leprosy and reactions [
      • Scollard D.M.
      • Dacso M.M.
      • Abad-Venida M.L.
      Tuberculosis and leprosy: classical granulomatous diseases in the twenty-first century.
      ]. Nerve damage is usually characterized by impairment or complete sensory loss in the areas related to the peripheral nerves [
      • de Freitas M.R.
      • Said G.
      Leprous neuropathy.
      ].

      Authorship

      All authors participated equally in patient management, literature review and manuscript writing.

      Funding

      Nothing to declare.

      Conflict of interest statement

      All authors declare no conflicts of interest.

      References

        • Talhari C.
        • Talhari S.
        • Penna G.O.
        Clinical aspects of leprosy.
        Clin Dermatol. 2015; 33: 26-37
        • Scollard D.M.
        • Dacso M.M.
        • Abad-Venida M.L.
        Tuberculosis and leprosy: classical granulomatous diseases in the twenty-first century.
        Dermatol Clin. 2015; 33: 541-562
        • de Freitas M.R.
        • Said G.
        Leprous neuropathy.
        Handb Clin Neurol. 2013; 115: 499-514