Case for diagnosis. Infiltrated areas on the trunk^☆,☆☆

Abstract

Leprosy is an infectious disease with chronic evolution, caused by Mycobacterium leprae, an acid-fast bacillus that mainly affects the skin and peripheral nervous tissue. Many of the clinical manifestations of leprosy can mimic connective tissue diseases. The authors present the case of a 49-year-old woman who had been treated for four years for systemic lupus erythematosus in a rheumatological service. Skin biopsy of a plaque on the inguinal region was compatible with borderline lepromatous leprosy associated with a type 1 lepra reaction. The patient is undergoing treatment with multibacillary multidrug therapy, showing clinical improvement.

KEYWORDS
Leprosy; Leprosy, multibacillary; Panniculitis

Case report

A 49-year-old woman reported pain in the interphalangeal joints and lower limbs, a skin rash, and alopecia; all symptoms persisted for four years. She was being treated for systemic lupus erythematosus with hydroxychloroquine, azathioprine, methotrexate, and prednisone. On physical examination, she presented generalized erythematous plaques, and infiltrated areas on the trunk, on the ears, and fingers, as well as diffuse non-healing alopecia (Figs. 1 and 2).

Figure 1
Erythematous plaques on the trunk.

Figure 2
Erythematous plaques on the back of the hand and infiltration of the fingers.

She presented 24-hour proteinuria within normal limits, positive ANA (1:320, centromeric dotted nuclear pattern), positive anti-cardiolipin IgM antibodies, and anti-SM and anti-double helix DNA, both negative.

What is your diagnosis?

• a) Subacute lupus

• b) Dermatomyositis

• c) Lepromatous leprosy

• d) Diffuse eosinophilic fasciitis

She also presented paresthesia of the lower limbs, and thickening and pain on palpation of the left ulnar and left external sciatic popliteal nerves.

The bacilloscopy of the ear and two trunk lesions was positive (smear index: 3.33). Histopathology of the plaque in the inguinal region showed an epidermis with hyperkeratosis; in the dermis, edema and a perivascular, periadnexal, and interstitial mild inflammatory infiltrate composed of lymphocytes, histiocytes, plasmocytes, epithelioid cells, and sparse giant Langhans cells, involving the subcutaneous tissue, adipocytes and septa. Wade staining showed a large number of intracellular acid-fast bacilli, grouped in globi, fragmented and granular (Fig. 3A and 3B). PAS staining did not show thickening of the basal membrane, and no mucin was observed in the Alcian blue staining. In light of the histopathological alterations, a diagnosis of borderline lepromatous leprosy was made in association with type 1 lepra reaction. The patient denied having undergone previous treatment for leprosy and reported that a son-in-law had received anti-leprosy treatment. Multibacillary multidrug therapy and prednisone 60 mg/day were initiated for treating the symptoms and neural lesions. After 30 days of treatment, the patient presented significant improvement (Fig. 4).

Figure 3
(A), Perivascular and periadnexal inflammatory infiltrate composed of lymphocytes, histiocytes, plasma cells, and epithelioid cells (Hematoxylin & eosin, ×40); (B), Fragmented, granular, isolated acid-fast bacilli, also grouped in globi (Wade, ×40).

Figure 4
Residual hyperchromic patches on the trunk and upper limbs after 30 days of treatment with multidrug therapy and prednisone.

Discussion

Leprosy is a chronic contagious infectious disease, caused by Mycobacterium leprae, an acid-fast bacillus, with tropism for Schwann cells and the reticuloendothelial system. The bacillus mainly affects the skin and peripheral nervous tissue.¹ The ability of M. leprae to regulate cytokine production and induce Th1 or Th2 responses contributes to the variety of clinical presentations of the disease.²

Rheumatological manifestations are observed in 64% to 77% of patients with multibacillary leprosy, especially when presenting lepra reactions.³ Rheumatological symptons were observed in 41.8% of the patients with lepromatous leprosy; in 23.6% of those with tuberculoid borderline; in 18.2% of those with borderline forms; and in 16.4% of those with borderline lepromatous type.⁴

Positive ANA can be observed in 3% to 34% of leprosy patients. Titers are generally low, and the speckled and homogeneous patterns are the most common.⁵ Rheumatoid factor, anti-CCP, ANCA, anti-cardiolipin, and antiphospholipids have been observed in varying percentages in patients with the different forms of leprosy.^6-8 Non-scarring alopecia, especially in patients with the lepromatous leprosy, has already been described.⁹

The different clinical manifestations of leprosy pose a diagnostic challenge, since they can mimic other diseases, including autoimmune disorders. The difficulty of diagnosing leprosy early can increase the chance of transmission and cause deformities secondary to peripheral nerve involvement.¹⁰

• ☆
  How to cite this article: Góes LDM, Scrignoli JA, Morais P, Talhari C. Case for diagnosis. Infiltrated areas on the trunk. An Bras Dermatol. 2020. https://doi.org/10.1016/j.abd.2019.12.007
• ☆☆
  Study conducted at the Fundação de Dermatologia Tropical e Venereologia Alfredo da Matta, Manaus, AM, Brasil.
• Financial support
  None declared.

References

• ¹
  Talhari S, Penna GO, Gonçalves HS, Oliveira MLW. Hanseníase. 5th ed. Manaus: Editora DiLivros; 2014.
• ²
  Cruz RCDS, Bührer-Sékula S, Penna MLF, Penna GO, Talhari S. Leprosy: current situation, clinical and laboratory aspects, treatment history and perspective of the uniform multidrug therapy for all patients. An Bras Dermatol. 2017;92:761-73.
• ³
  Albert DA, Weisman MH, Kaplan R. The rheumatic manifestations of leprosy (Hansen disease). Medicine (Baltimore). 1980;59:442-8.
• ⁴
  Pereira HL, Ribeiro SL, Pennini SN, Sato EI. Leprosy-related joint involvement. Clin Rheumatol. 2009;28:79-84.
• ⁵
  Pradhan V, Badakere SS, Shankar Kumar U. Increased incidence of cytoplasmic ANCA (cANCA) and other autoantibodies in leprosy patients from western India. Lepr Rev. 2004;75:50-6.
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• ⁷
  Elbeialy A, Strassburger-Lorna K, Atsumi T, Bertolaccini ML, Amengual O, Hanafi M, et al. Antiphospholipid antibodies in leprotic patients: a correlation with disease manifestations. Clin Exp Rheumatol. 2000;18:492-4.
• ⁸
  Horta-Baas G, Hernández-Cabrera MF, Barile-Fabris LA, Romero-Figueroa del MS, Arenas-Guzmán R. Multibacillary leprosy mimicking systemic lupus erythematosus: case report and literature review. Lupus. 2015;24:1095-102.
• ⁹
  Abraham S, Ebenezer GJ, Jesudasan K. Diffuse alopecia of the scalp in borderline lepromatosus leprosy in an Indian patient. Lepr Rev. 1997;68:336-40.
• ¹⁰
  Teixeira G, Silva C, Magalhães V. Application of the diagnostic criteria for systemic lupus erythematosus to patients with multibacillary leprosy. Rev Soc Bras Med Trop. 2011;44:85-90.

Publication Dates

• Publication in this collection
  16 Nov 2020
• Date of issue
  Sep-Oct 2020

History

• Received
  30 July 2019
• Accepted
  8 Dec 2019