Case report

Lepromatous leprosy in erythema nodosum leprosum

reaction mimicking Sweets syndrome
Yee-Kiat Heng, MRCP, Yuun-Tirng Lynn Chiam, MRCP, Yoke-Chin Giam, MMED(PAEDS),
and Wei-Sheng Chong, FRCP

National Skin Center, Singapore

Correspondence
Yee-Kiat Heng, MRCP
National Skin Centre
1, Mandalay Road
Singapore 308205
E-mail: ykheng@nsc.gov.sg

A 52-year-old Malay man presented with an acute one

week history of multiple erythematous papules and
nodules on the limbs, trunk, and face. On physical examination, he had multiple erythematous nontender, nonpruritic nodules and plaques on the trunk, limbs, and
forehead (Fig. 1). A few nodules showed pustulation and
ulceration (Fig. 2). He was febrile at 38 C. Systemic
review and general examination were normal. There were
no thickened nerves, neurological deficits, or deformities
of the hands or feet. Eyes and oral mucosa were normal.
The initial clinical impression was that of Sweets syndrome in view of the acute onset of typical lesions and
fever. Complete blood count revealed marked leukocytosis
(24.1 103/ll) with predominant neutrophilia (87.5%).
Erythrocyte sedimentation rate (ESR) was markedly raised.
Differential diagnoses included cutaneous lymphoma and
infective causes, such as cutaneous tuberculosis, multibacillary leprosy, and nontuberculous mycobacterial or
subcutaneous fungal infection.

Figure 2 Erythematous plaque with pustule and healing ulcer

Skin biopsy confirmed the diagnosis of lepromatous

leprosy with erythema nodosum leprosum reaction. The
histology showed a Grenz zone with granulomas throughout the dermis, consisting of foamy macrophages with
globi of bacilli within. Focal areas showed thrombosed
vessels with necrosis of vessel walls and numerous neutrophils. Fite stain was positive for leprae bacilli (Fig. 3).
Direct immunofluorescence showed granular deposits of
IgA and C3 in the upper and lower dermal blood vessel
walls, consistent with vasculitis.
Slit skin smear confirmed the presence of Mycobacterium leprae with a Bacteriological Index of 5+ and a
Morphological Index of <1%.
Discussion

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Figure 1 Erythematous plaques and nodules on the trunk

International Journal of Dermatology 2011, 50, 11241125

Leprosy has been controlled in Singapore for many years.

This has been achieved by a good program for control of
2011 The International Society of Dermatology

Heng et al.

Figure 3 Histology showed granulomas with Mycobacterium

leprae bacilli-laden macrophages (Fite stain, original
magnification 400)

leprosy. The latest reported incidence of leprosy is low at

0.1 per 100,000 resident population.1 Only 10 cases were
reported in 2008, mostly among foreign workers.
Most patients with multiple nodules would have been
biopsied and diagnosed early. This patient recalled having
multiple asymptomatic skin lesions for two years but did
not seek specialist opinion. Erythema nodosum leprosum
reactions may occur spontaneously in longstanding lepromatous leprosy,2 but for it to simulate Sweets syndrome
is much less common, though it was reported as early as
1987 by Kuo and Chan.3 In the patient described, the
clinical and histopathological features were consistent
with both lepromatous leprosy and Sweets syndrome. It
was suggested that leprosy in a reactional state should be
considered as a differential diagnosis for Sweets syndrome. In our patient, the histology showed numerous
neutrophils but was otherwise not consistent with Sweets
syndrome. Clinically, the lesions mimicked Sweets syndrome sufficiently for it to be considered as one of the
likely differential diagnoses. Fever, neutrophilia, and
rapid response to oral corticosteroids were observed, as
are expected in Sweets syndrome.4
In this patient, the diagnosis was clinched on histopathological examination. Reactional states of leprosy are
known to present in many ways. The skin manifestations

2011 The International Society of Dermatology

Lepromatous leprosy

Case report

of a type 2 lepra reaction typically appear in normal skin

between previously recognized plaques of borderline or
lepromatous leprosy.5 In this case, the reactional lesions
were the presenting symptom. As he did not have a preceding diagnosis of leprosy, this was an unexpected finding. Most reported cases mimicking Sweets syndrome
have prior diagnosis of borderline or lepromatous
leprosy.6
This patient was treated with WHO-recommended
drugs for lepromatous leprosy. The erythema nodosum
leprosum reaction was controlled with oral corticosteroids and clofazimine. Corticosteroid dose was gradually
reduced, with no recurrence in 12 months of follow-up.
This locally uncommon infection presented in an unusual manner, making this a case with valuable learning
points. Delayed diagnosis of leprosy leads to significant
morbidity7 and has important public health consequences.
With this case, we highlight the unusual Sweets syndrome-like presentation of lepromatous leprosy in erythema nodosum leprosum reaction and the need to
exclude infectious diseases, even in a nonendemic area.
References
1 Communicable Diseases Surveillance in Singapore 2007;
109110. http://www.moh.gov.sg/mohcorp/publication
sreports.aspx?id=20288 [Accessed September 1, 2010].
2 Rea TH, Levan NE. Erythema nodosum leprosum in a
general hospital. Arch Dermatol 1975; 111: 15751580.
3 Kuo TT, Chan HL. Severe reactional state in lepromatous
leprosy simulating Sweets syndrome. Int J Dermatol
1987; 26: 518520.
4 Cohen PR. Sweets syndrome a comprehensive review of
an acute febrile neutrophilic dermatosis. Orphanet J Rare
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5 Rea TH, Modlin RL. Leprosy. In: Freedberg IM, Eisen AZ,
Wolff K, Austen KF, Goldsmith LA, Katz SI, ed.
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6 Aires NB, Refkalefsky LW, Villela MA, et al. Sweets
syndrome type leprosy reaction. J Eur Acad Dermatol
Venereol 2009; 23: 467469.
7 Lockwood DN, Reid AJ. The diagnosis of leprosy is
delayed in the United Kingdom. QJM 2001; 94:
207212.

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