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A fatal case of Lucio phenomenon from India

  • Author(s): Kumari, Rashmi
  • Thappa, Devinder Mohan
  • Basu, Debdatta
  • et al.
Main Content

A fatal case of Lucio phenomenon from India
Rashmi Kumari MD1, Devinder Mohan Thappa MD1, Debdatta Basu MD2
Dermatology Online Journal 14 (2): 10

1. Department of Dermatology and Sexually Transmitted Diseases, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry-605006, India. dmthappa@gmail.com
2. Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry-605006, India


Abstract

A 65-year-old man presented with cutaneous ulcerations involving the legs, hands, abdomen, buttocks, and pinna, along with fever, arthralgia, and anorexia for the prior 10 days. On cutaneous examination, dark, irregular-shaped bizarre erythematous purpuric spots and angulated ulcers were seen over bilateral, upper extremities and trunk including dorsum of hands, finger tips and the pinnae of both ears. Most striking were the presence of multiple deep ulcers covered with a blackish eschar and in some areas yellow slough eroding the subcutaneous tissue with ragged margins. These ulcers were distributed symmetrically over the thighs, lower legs and gluteal region. Slit-skin smear examination revealed a bacterial index (BI) of 6+ with globi from earlobes, ulcers 3+, eyebrows 3+ and normal skin 2+ and morphologically showed mainly solid (20-30%), fragmented (60-70%) and granular (5-10%) acid-fast bacilli. Biopsy from the ulcer margin revealed an ulcerated epidermis and dermis. The dermis had infiltrate of foamy macrophages, and evidence of ischemic necrotizing vasculitis, with fibrinoid necrosis and new vessel formation. There was presence of clumps of acid-fast bacilli (AFB) within macrophages, periadnexally, perivascularly, and also within endothelial cells. These clinical and histopathological features helped us to arrive at the diagnosis of Lucio phenomenon in an untreated case of Lucio leprosy which is rarely reported from areas other than Mexico.


In 1852, Raphael Lucio and Ignácio Alvarado [1], in their report "Opúsculo sobre el mal de San Lazaro o elefantíase de los griecos," described a form of leprosy, they called spotted (manchada) or Lazarine leprosy. In this form, there are no localized skin infiltrations, i.e., non-nodular leprosy. Frequent necrotizing reactions take place with scarlet spots that later darken and may ulcerate, leaving atrophic and hypochromic scars with a thin hyperpigmented border. Latapi and Chevez Zamora [2], in 1948, added some characteristics to the original Lucio and Alvarado description, calling it as Lucio leprosy or pure, primitive, diffuse and non-nodular form of leprosy. It is more frequent in Mexico and is distinct from the diffuse forms that progress with nodular lesions, since in Lucio leprosy the Virchow cells do not form nodules. The skin presents with diffuse infiltration, resulting in a healthy appearance; afterward it becomes myxedematous, especially on the face and hands, and years later it becomes atrophic and ichthyosiform. Such is the clinical base for the onset of necrotizing spots that constitute the necrotizing erythema or Lucio phenomenon (LP). We herewith report such a case in a 65-year-old man for its rarity and unique features.


Clinical synopsis

A 65-year-old man from Tamilnadu was referred to us from the Department of Medicine with cutaneous ulcerations over the legs, hands, abdomen, buttocks and pinna, along with fever, arthralgia and anorexia for past 10 days. These ulcers had first appeared over bilateral lower legs as painless, scarlet spots of irregular bizarre shapes that later darken and ulcerate, leaving atrophic and hypochromic scars with a thin hyperpigmented border in some areas. Within 2 days, similar lesions appeared involving the trunk, upper limbs as well as pinna of both ears. He had been diagnosed as a case of leprosy 15 years before and advised to take anti-leprosy treatment but due to social stigma, he had refused to take any treatment in spite of being aware of his disease. He had experienced similar ulcerations mainly over his lower legs repeatedly for the last year which earlier used to heal spontaneously with antibiotics from a local doctor. However, this episode, being more severe, he was referred to our institute.

On cutaneous examination, dark, irregular-shaped, bizarre, erythematous, purpuric spots and angulated ulcers were seen over bilateral, upper extremities and trunk including dorsum of hands, finger tips and the pinnae of both ears (Fig. 1). Most striking were the presence of multiple deep ulcers covered with a blackish eschar (Figs. 2 & 3) and in some areas yellow slough eroding the subcutaneous tissue with ragged margins. These ulcers were distributed symmetrically over the thighs, lower legs (Fig. 4) and gluteal region. At the edge of some of the ulcerated lesions over the thighs, the skin had a shiny and succulent appearance. In between, superficial atrophic scars of old, healed lesions were present. The face was diffusely infiltrated without any evidence of nodulation or localized infiltrated plaques with ciliary and superciliary madarosis. Erosions were also seen over the buccal mucosa, lips, scrotum and glans. Glove-and-stocking anesthesia was present over the extremities. Bilaterally, the ulnar, radial, lateral popliteal, posterior tibial and great auricular nerves were moderately thickened. Systemic examination showed no other abnormality except for a low-grade fever.


Figure 1Figure 2
Figure 1. Vasculitic ulcerated lesions over the pinna
Figure 2. Superficial angulated ulcers seen over the thighs and scrotum

Figure 3Figure 4
Figure 3. Necrotizing lesions over the buttocks
Figure 4. Deep necrotic ulcers covered with yellow slough over lower legs

Slit-skin smear examination revealed a bacterial index (BI) of 6+ with globi from earlobes, ulcers 3+, eyebrows 3+ and normal skin 2+ and morphologically showed mainly solid (20-30%), fragmented (60-70%) and granular (5-10%) acid-fast bacilli. Biopsy from the ulcer margin revealed an ulcerated epidermis and dermis. The dermis had infiltrate of foamy macrophages (Fig. 5), and evidence of ischemic necrotizing vasculitis, with fibrinoid necrosis (Fig. 6) and new vessel formation. There was presence of clumps of acid-fast bacilli (AFB) within macrophages, periadnexally, perivascularly, and also within endothelial cells (Fig. 6).


Figure 5Figure 6
Figure 5. Hematoxylin and eosin-stained section showing intact epidermis with subepidermal grenz zone. The dermis had infiltrate of foamy macrophages (H & E X 100).
Figure 6. Fite-Faraco stained section showing fibrinoid necrosis with thickening of vessel wall and globi of acid fast bacilli including within endothelial cells (X 400).

Complete hemogram revealed hemoglobin of 7.5 g⁄dL, total leukocyte count of 20,000/µL with neutrophilia and shift to the left, and raised erythrocyte sedimentation rate (40 mm in the first hour). Liver and renal function tests, urinalysis, coagulation studies, blood sugar, antinuclear factor, rheumatoid arthritis factor, lupus erythematosus cell test, antiphospholipid antibodies, cryoglobulins, serology for syphilis, sputum for AFB, Mantoux test and chest X-ray were normal. Serology for HIV, syphilis and HBsAg was negative. Smears and cultures for fungus, and mycobacterium tuberculosis obtained from the ulcers were negative. Bacterial culture from the lower legs revealed a growth of Staphylococcus aureus.

On the basis of typical clinical presentation of diffuse non-nodular leprosy presenting with vasculitic ulcers without any other cause for this vasculonecrotic phenomenon the diagnosis of Lucio leprosy (Ridley-Jopling) with Lucio phenomenon was considered and confirmed by histopathology. WHO MDT (multibacillary) regimen, consisting of rifampicin 600 mg and clofazimine 300 mg once a month, dapsone 100 mg daily, and clofazimine 100 mg every other day, was initiated along with systemic antibiotics. Daily dressings of hydrogen peroxide and saline were given. No new ulcers appeared further and the ulcers started healing over the following 2 weeks but he developed MRSA (multidrug resistant Staphylococcus aureus) septicemia and died on the 16th day of hospitalization.


Discussion

Lucio phenomenon (LP) is described as a necrotizing reactional picture that is present in patients with the pure and primitive form of diffuse Lucio leprosy that never evolves with papules, plaques or leprotic nodules. The necrotizing lesions that developed over this diffuse lepromatosis were called Lucio phenomenon eponymically and erythema necroticans (ENe) descriptively [2]. It is a reactional state, different from erythema nodosum leprosum, affecting LL or BL patients and not only in those with the pure and primitive form of leprosy as it was first described earlier. It is endemic in Mexico (especially in the provinces of Jolisco and Sinaloa). However, this phenomenon is not restricted to Mexico. Lucio phenomenon has been reported in the USA, Spain, South and Central America, including Brazil, and East and West Asia [3].

Lucio phenomenon manifests three to four years after onset of the disease and is more common in untreated patients or in those receiving inadequate treatment [1, 2, 3]. This phenomenon tends to disappear 6-8 weeks after the beginning of the treatment; when the patients still present with reactions after treatment, they are of the erythema nodosum type. The skin lesions are characterized by erythematous spots that progress to superficial 0.5 to 1 cm lesions of irregular format, more frequently triangular, polygonal or angular, with a localized burning sensation. Sometimes, blisters also appear. The lesions coexist in several evolution stages in the same patient. Between two and four weeks, the healing process of ulceration begins and a superficial scar is formed.

Histopathology picture in our case was suggestive of Lucio phenomenon. Lucio phenomenon has to be differentiated from vasculonecrotic reactions seen in ENL or type-2 lepra reaction. Their differentiating features are given in Table 1.

Latapi, at the V International Leprosy Congress held in Havana, in 1948, already warned about the improper use of LP term for patients with other forms of multibacillary leprosy (BL and LL), who developed blisters and ulcerations, but did not present Lucio leprosy [2]. These cases actually represent type 2 lepra reaction with vasculonecrotic phenomenon presenting with extensive deep eschars that are often confused and mislabeled as LP [5, 6]. Thappa et al [5] also warned about the improper labeling of many cases of nodular lepromatous leprosy cases presenting with vasculonecrotic phenomena as Lucio leprosy with Lucio phenomenon. In our patient, the characteristic bizarre, geometric, erythematous lesions resulting in superficial ulceration and healing with scars in absence of any constitutional symptoms in a patient with untreated diffuse non-nodular form of leprosy suggested the diagnosis of Lucio phenomenon. Other causes of necrotizing skin diseases, such as factitia dermatitis, erythema multiforme bullosum, vasculitides, purpura fulminans, pyoderma gangrenosum, ecthyma, tuberculosis, late syphilis and idiopathic leukocytoclastic vasculitis were ruled out.

There is no consensus regarding the specific treatment of Lucio phenomenon which is mostly empirical, based on case reports of successful treatment. MDT for multibacillary leprosy (WHO recommended) reported to be effective in treating Lucio phenomenon, supports the theory that the trigger event of Lucio phenomenon is the massive replication of M. leprae, which must be prevented in order to stop this unique leprotic reaction. When MDT for leprosy is introduced at the beginning of the Lucio phenomenon outbreak, the prognosis is usually good. However, when patients present with extensive skin involvement, secondary infection and anemia, as in the case reported here, the prognosis is poor even with treatment. Therefore, we consider that the poor response to treatment observed in this case was due to the advanced stage at which Lucio phenomenon was diagnosed and treated. The role of corticosteroids is controversial. Thalidomide and clofazimine are less effective compared with their efficacy in ENL.

This report highlights the rare presentation of Lucio leprosy with Lucio phenomenon in India and its typical clinical presentation and histopathology.

References

1. Lucio R, Alvarado I. Opusculo Sobre el Mal de San Lazaro O, Elefan ciasis de los Griegos.Mexico: M. Murguia y Cia, 1852; 53.

2. Latapi F., Zamora A.C. The "spotted" leprosy of Lucio (la lepra manchada de Lucio): An introduction to its clinical and histological study. Int J Lepr 1948; 16: 421-30.

3. Costa IM, Kawano LB, Pereira CP, Nogueira LS. Lucio's phenomenon: a case report and review of the literature. Int J Dermatol. 2005 ;44(7):566-71.

4. Moschella SL. Leprosy. In: Moschella SL, Hurley HJ, eds. Dermatology, 2nd edn. Philadelpia: WB Saunders Company, 1985; 967-968.

5. Thappa DM, Karthikeyan K, Kumar BJ. Is it Lucio leprosy with Lucio phenomenon or something else? Indian J Lepr 2002; 74: 161-166.

6. Saoji V, Salodkar A. Lucio leprosy with Lucio phenomenon Indian J Lepr 2001; 73: 267-272.

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