Tropical medicine rounds
Novel presentation of lepromatous leprosy in an erythema gyratum repens-like pattern Saritha Mohanan1, MD, Abarna S. Devi1, MD, Rashmi Kumari1, MD, Devinder Mohan Thappa1, MD, and Rajesh N. Ganesh2, MD
1 Department of Skin and Sexually Transmitted Diseases, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India, and 2 Depratment of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
Abstract Objectives
Leprosy
can
have
diverse
cutaneous
and
occasionally
perplexing
presentations. We report an unusual case of lepromatous leprosy (LL) with annular lesions resembling erythema gyratum repens. Report A 55-year-old man presented with a symmetrical, hypopigmented, and erythematous rash of bizarre appearance over the lateral aspect of the upper arm, and anterior and posterior aspects of the trunk of two months’ duration. He gave a history of
Correspondence Rashmi Kumari, MD Department of Skin and Sexually Transmitted Diseases Jawaharlal Institute of Postgraduate Medical Education and Research Dhanvantri Nagar, Gorimedu, Pondicherry Puducherry 605006, India E-mail: [email protected]
self-resolving episodes of bilateral pedal edema, and numbness and pricking sensations in both the hands and feet, which had occurred intermittently over the previous six years. An ulcer measuring 2 cm in size was present over the adjacent surface of the right first and second toes. The bilateral ulnar and radial cutaneous nerves were symmetrically thickened. Results Slit-skin smears revealed numerous acid-fast bacilli. Skin biopsy from the trunk showed collections of histiocytes, lymphocytes, and plasma cells in the dermis and around the blood vessels. The patient was diagnosed with LL and started on multibacillary multidrug therapy.
Conflicts of interest: None.
Conclusions Lepromatous leprosy can have varied clinical manifestations and is often a great imitator. However, the skin smear positivity, even in normal skin, symmetrical cutaneous and peripheral nerve involvement, and histopathology in the present patient were indicative of LL. This report highlights a rare presentation of leprosy. Clinicians should be aware of these rare manifestations as lepromatous cases still occur in certain regions.
Introduction The scourge of leprosy has been eliminated from 32 states of India as of March 2011.1 However, it is far from being eradicated as some states, such as Bihar and Chattisgarh, which still show high prevalences of the disease.1 Leprosy can have diverse cutaneous presentations and occasionally perplexes the clinician, who may not be aware of unusual presentations. We report an unusual case of lepromatous leprosy (LL) with annular lesions resembling erythema gyratum repens (EGR). Case report
210
A 55-year-old man presented at the dermatology outpatient department with a symmetrical, hypopigmented, and erythematous rash of bizarre appearance over the lateral aspect of the upper arm, and anterior and posterior aspects of the trunk of two months’ duration (Figs. 1 and 2), which he attributed to an insect bite. On probing, the patient also gave a history of self-resolving episodes of International Journal of Dermatology 2014, 53, 210–212
bilateral pedal edema, and numbness and pricking sensations in both the hands and feet, which had occurred intermittently over the previous six years. He also reported a history of occasional episodes of blistering of the bilateral palms and soles, probably caused by hypoesthesia. On examination, multiple, well-defined, erythematous and hypopigmented, non-scaly macules forming concentric rings resembling the grain of wood were seen to be arranged symmetrically over the anterior and posterior aspects of the trunk, the lateral aspect of the upper arm, and thighs, simulating EGR. An ulcer measuring 2 cm in size was present over the adjacent surface of the right first and second toes. The bilateral ulnar and radial cutaneous nerves were symmetrically thickened. There was no evidence of neuritis. Slit-skin smears from the hypopigmented patches, eyebrows, ear lobes, and normal skin revealed numerous acid-fast bacilli. A skin biopsy from the trunk showed collections of histiocytes, lymphocytes, and plasma cells in the dermis and around the blood vessels (Fig. 3a). Fite’s stain was strongly positive (Fig. 3b). The patient was diagnosed with LL and started ª 2013 The International Society of Dermatology
Mohanan et al.
Novel presentation of lepromatous leprosy
Tropical medicine rounds
(b)
(a)
Figure 1 Hypopigmented areas arranged in concentric circles are apparent on the chest in a 55-year-old man subsequently diagnosed with lepromatous leprosy
Figure 2 Clinical examination of the patient’s back demonstrates a rash arranged in rings
on a World Health Organization (WHO) multibacillary multi-drug therapy (MB-MDT) regimen. One year later, the patient is continuing MB-MDT as he defaulted therapy twice. His family members and neighbors did not show features of leprosy. The subject was counseled about the nature of the disease and the need for regular follow-up. As the numbness is persisting, the patient was advised of appropriate hand and foot care to avoid blistering and ulceration. Discussion Lepromatous leprosy can demonstrate varied clinical manifestations and is often a great imitator. Generally, skin lesions are widespread and appear as symmetrically ª 2013 The International Society of Dermatology
Figure 3 (a) Histopathology shows a histiolymphocytic infiltrate around a neurovascular bundle in the dermis. (Hematoxylin and eosin stain; original magnification 9 100.) (b) Fite–Faraco staining for acid-fast leprosy bacilli was strongly positive (original magnification 9 1000)
distributed hypopigmented macules, nodules, or plaques. The mid-borderline spectrum of Hansen’s disease can manifest in bizarre and localized patches; however, the skin smear positivity even in normal skin, symmetrical cutaneous and peripheral nerve involvement, and histopathology in our patient were indicative of LL. Certain unusual presentations have been reported in Hansen’s disease, such as an appearance reminiscent of facial keloids,2 an appearance apparently indicative of physical abuse,3 a pre-auricular cyst-like presentation of type 2 reaction,3 a systemic lupus erythematosus (SLE)-like presentation,4 and pustular erythema nodosum leprosum.5 The present patient, whose disease belonged within the spectrum of LL, demonstrated patches arranged in concentric rings and circles resembling EGR, except for the absence of scaling. Other possible differential diagnoses include tinea imbricate and erythema annulare centrifugum.6 Erythema gyratum repens was first described by Gammel in 1952 in a woman with adenocarcinoma of the breast.7 Gyratum means coiled or winding around a central point, and repens (Latin) means to crawl or creep; the name itself describes the classic eruption of concentric erythematous rings that develop trailing scale at their edges and advance at a rapid rate (1 cm/day).7 In 80% of patients, an underlying neoplasia can be detected.8 Tumor antigens are said to cross-react with endogenous skin antigens, making them susceptible to immune complex deposition.9 The disease usually presents around nine months before the diagnosis of malignancy.10 Erythema gyratum repens-like eruptions have also been described as occurring in the wake of benign conditions such as pityriasis rubra pilaris,10 bullous pemphigoid,11,12 International Journal of Dermatology 2014, 53, 210–212
211
212
Novel presentation of lepromatous leprosy
Mohanan et al.
SLE and lupus vasculitis,13 and even when the underlying problem is unknown.14 The history of sensory disturbances in the extremities in our patient led us to suspect Hansen’s disease. However, the patient could have been misdiagnosed as having a peripheral neuropathy in association with a malignancy, leading to an unnecessary workup for malignancy. This report is intended to highlight a rare presentation of leprosy in this era of leprosy elimination. Clinicians should be aware of these rare manifestations of leprosy as lepromatous cases still abound in certain regions of the world.
5 Dave S, Thappa DM, Nori AV, et al. A rare variant of erythema nodosum leprosum: a case report. Dermatol Online J 2003; 9: 11. 6 Cotterman C, Eckert L, Ackerman L. Syphilis mimicking tinea imbricata and erythema annulare centrifugum in an immunocompromised patient. J Am Acad Dermatol 2009; 61: 165–167. 7 De La Torre-Lugo EM, S anchez JL. Erythema gyratum repens. J Am Acad Dermatol 2011; 64: e89–e90. 8 M€ uller CS, Lorenz MT, Tilgen W, et al. Primary manifestation of erythema gyratum repens as a transient erythroderma in a patient with bronchial carcinoma. Int J Dermatol 2010; 49: 676–678. 9 Abreu Velez AM, Howard MS. Diagnosis and treatment of cutaneous paraneoplastic disorders. Dermatol Ther 2010; 23: 662–675. 10 Demonchy E, Lacour JP, Ortonne JP, et al. Erythema gyratum repens, not always a bad omen for patients. J Eur Acad Dermatol Venereol 2010; 24: 738–739. 11 Breathnach SM, Wilkinson JD, Black MM. Erythema gyratum repens-like figurate eruption in bullous pemphigoid. Clin Exp Dermatol 1982; 7: 401–406. 12 Hauschild A, Swensson O, Christophers E. Paraneoplastic bullous pemphigoid resembling erythema gyratum repens. Br J Dermatol 1999; 140: 550–552. 13 Pique E, Palacios S, Santana Z. Leukocytoclastic vasculitis presenting as an erythema gyratum repens-like eruption on a patient with systemic lupus erythematosus. J Am Acad Dermatol 2002; 47(Suppl.): 254–256. 14 Campbell L, Freedman JR, ODonoghue M, et al. Erythema gyratum repens without associated malignancy. J Am Acad Dermatol 2011; 65: e22–e23.
Tropical medicine rounds
References 1 National Leprosy Eradication Program. Progress report as on 31st March 2011. http://nlep.nic.in/. [Accessed April 21, 2012]. 2 Gorin RJ, Chaplik I, Deitz M. Hansens disease masquerading as facial keloid. Poster session presented at: American Academy of Dermatology 62nd annual meeting, Feb 6–11, 2004, Washington DC. J Am Acad Dermatol 2004; 50: P1–P198. 3 Wong SM, Ho V. Leprosy mistaken for physical abuse. Poster session presented at American Academy of Dermatology 65th annual meeting, Feb 2–6, 2007, Washington DC. J Am Acad Dermatol 2007; 56: AB122. 4 Alberti JR, Cabrera A, Martiniuk F, et al. Leprosy masquerading as lupus. J Am Acad Dermatol 2005; 52: 702–703.
International Journal of Dermatology 2014, 53, 210–212
ª 2013 The International Society of Dermatology
Novel presentation of lepromatous leprosy in an erythema gyratum repens-like pattern Saritha Mohanan1, MD, Abarna S. Devi1, MD, Rashmi Kumari1, MD, Devinder Mohan Thappa1, MD, and Rajesh N. Ganesh2, MD
1 Department of Skin and Sexually Transmitted Diseases, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India, and 2 Depratment of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
Abstract Objectives
Leprosy
can
have
diverse
cutaneous
and
occasionally
perplexing
presentations. We report an unusual case of lepromatous leprosy (LL) with annular lesions resembling erythema gyratum repens. Report A 55-year-old man presented with a symmetrical, hypopigmented, and erythematous rash of bizarre appearance over the lateral aspect of the upper arm, and anterior and posterior aspects of the trunk of two months’ duration. He gave a history of
Correspondence Rashmi Kumari, MD Department of Skin and Sexually Transmitted Diseases Jawaharlal Institute of Postgraduate Medical Education and Research Dhanvantri Nagar, Gorimedu, Pondicherry Puducherry 605006, India E-mail: [email protected]
self-resolving episodes of bilateral pedal edema, and numbness and pricking sensations in both the hands and feet, which had occurred intermittently over the previous six years. An ulcer measuring 2 cm in size was present over the adjacent surface of the right first and second toes. The bilateral ulnar and radial cutaneous nerves were symmetrically thickened. Results Slit-skin smears revealed numerous acid-fast bacilli. Skin biopsy from the trunk showed collections of histiocytes, lymphocytes, and plasma cells in the dermis and around the blood vessels. The patient was diagnosed with LL and started on multibacillary multidrug therapy.
Conflicts of interest: None.
Conclusions Lepromatous leprosy can have varied clinical manifestations and is often a great imitator. However, the skin smear positivity, even in normal skin, symmetrical cutaneous and peripheral nerve involvement, and histopathology in the present patient were indicative of LL. This report highlights a rare presentation of leprosy. Clinicians should be aware of these rare manifestations as lepromatous cases still occur in certain regions.
Introduction The scourge of leprosy has been eliminated from 32 states of India as of March 2011.1 However, it is far from being eradicated as some states, such as Bihar and Chattisgarh, which still show high prevalences of the disease.1 Leprosy can have diverse cutaneous presentations and occasionally perplexes the clinician, who may not be aware of unusual presentations. We report an unusual case of lepromatous leprosy (LL) with annular lesions resembling erythema gyratum repens (EGR). Case report
210
A 55-year-old man presented at the dermatology outpatient department with a symmetrical, hypopigmented, and erythematous rash of bizarre appearance over the lateral aspect of the upper arm, and anterior and posterior aspects of the trunk of two months’ duration (Figs. 1 and 2), which he attributed to an insect bite. On probing, the patient also gave a history of self-resolving episodes of International Journal of Dermatology 2014, 53, 210–212
bilateral pedal edema, and numbness and pricking sensations in both the hands and feet, which had occurred intermittently over the previous six years. He also reported a history of occasional episodes of blistering of the bilateral palms and soles, probably caused by hypoesthesia. On examination, multiple, well-defined, erythematous and hypopigmented, non-scaly macules forming concentric rings resembling the grain of wood were seen to be arranged symmetrically over the anterior and posterior aspects of the trunk, the lateral aspect of the upper arm, and thighs, simulating EGR. An ulcer measuring 2 cm in size was present over the adjacent surface of the right first and second toes. The bilateral ulnar and radial cutaneous nerves were symmetrically thickened. There was no evidence of neuritis. Slit-skin smears from the hypopigmented patches, eyebrows, ear lobes, and normal skin revealed numerous acid-fast bacilli. A skin biopsy from the trunk showed collections of histiocytes, lymphocytes, and plasma cells in the dermis and around the blood vessels (Fig. 3a). Fite’s stain was strongly positive (Fig. 3b). The patient was diagnosed with LL and started ª 2013 The International Society of Dermatology
Mohanan et al.
Novel presentation of lepromatous leprosy
Tropical medicine rounds
(b)
(a)
Figure 1 Hypopigmented areas arranged in concentric circles are apparent on the chest in a 55-year-old man subsequently diagnosed with lepromatous leprosy
Figure 2 Clinical examination of the patient’s back demonstrates a rash arranged in rings
on a World Health Organization (WHO) multibacillary multi-drug therapy (MB-MDT) regimen. One year later, the patient is continuing MB-MDT as he defaulted therapy twice. His family members and neighbors did not show features of leprosy. The subject was counseled about the nature of the disease and the need for regular follow-up. As the numbness is persisting, the patient was advised of appropriate hand and foot care to avoid blistering and ulceration. Discussion Lepromatous leprosy can demonstrate varied clinical manifestations and is often a great imitator. Generally, skin lesions are widespread and appear as symmetrically ª 2013 The International Society of Dermatology
Figure 3 (a) Histopathology shows a histiolymphocytic infiltrate around a neurovascular bundle in the dermis. (Hematoxylin and eosin stain; original magnification 9 100.) (b) Fite–Faraco staining for acid-fast leprosy bacilli was strongly positive (original magnification 9 1000)
distributed hypopigmented macules, nodules, or plaques. The mid-borderline spectrum of Hansen’s disease can manifest in bizarre and localized patches; however, the skin smear positivity even in normal skin, symmetrical cutaneous and peripheral nerve involvement, and histopathology in our patient were indicative of LL. Certain unusual presentations have been reported in Hansen’s disease, such as an appearance reminiscent of facial keloids,2 an appearance apparently indicative of physical abuse,3 a pre-auricular cyst-like presentation of type 2 reaction,3 a systemic lupus erythematosus (SLE)-like presentation,4 and pustular erythema nodosum leprosum.5 The present patient, whose disease belonged within the spectrum of LL, demonstrated patches arranged in concentric rings and circles resembling EGR, except for the absence of scaling. Other possible differential diagnoses include tinea imbricate and erythema annulare centrifugum.6 Erythema gyratum repens was first described by Gammel in 1952 in a woman with adenocarcinoma of the breast.7 Gyratum means coiled or winding around a central point, and repens (Latin) means to crawl or creep; the name itself describes the classic eruption of concentric erythematous rings that develop trailing scale at their edges and advance at a rapid rate (1 cm/day).7 In 80% of patients, an underlying neoplasia can be detected.8 Tumor antigens are said to cross-react with endogenous skin antigens, making them susceptible to immune complex deposition.9 The disease usually presents around nine months before the diagnosis of malignancy.10 Erythema gyratum repens-like eruptions have also been described as occurring in the wake of benign conditions such as pityriasis rubra pilaris,10 bullous pemphigoid,11,12 International Journal of Dermatology 2014, 53, 210–212
211
212
Novel presentation of lepromatous leprosy
Mohanan et al.
SLE and lupus vasculitis,13 and even when the underlying problem is unknown.14 The history of sensory disturbances in the extremities in our patient led us to suspect Hansen’s disease. However, the patient could have been misdiagnosed as having a peripheral neuropathy in association with a malignancy, leading to an unnecessary workup for malignancy. This report is intended to highlight a rare presentation of leprosy in this era of leprosy elimination. Clinicians should be aware of these rare manifestations of leprosy as lepromatous cases still abound in certain regions of the world.
5 Dave S, Thappa DM, Nori AV, et al. A rare variant of erythema nodosum leprosum: a case report. Dermatol Online J 2003; 9: 11. 6 Cotterman C, Eckert L, Ackerman L. Syphilis mimicking tinea imbricata and erythema annulare centrifugum in an immunocompromised patient. J Am Acad Dermatol 2009; 61: 165–167. 7 De La Torre-Lugo EM, S anchez JL. Erythema gyratum repens. J Am Acad Dermatol 2011; 64: e89–e90. 8 M€ uller CS, Lorenz MT, Tilgen W, et al. Primary manifestation of erythema gyratum repens as a transient erythroderma in a patient with bronchial carcinoma. Int J Dermatol 2010; 49: 676–678. 9 Abreu Velez AM, Howard MS. Diagnosis and treatment of cutaneous paraneoplastic disorders. Dermatol Ther 2010; 23: 662–675. 10 Demonchy E, Lacour JP, Ortonne JP, et al. Erythema gyratum repens, not always a bad omen for patients. J Eur Acad Dermatol Venereol 2010; 24: 738–739. 11 Breathnach SM, Wilkinson JD, Black MM. Erythema gyratum repens-like figurate eruption in bullous pemphigoid. Clin Exp Dermatol 1982; 7: 401–406. 12 Hauschild A, Swensson O, Christophers E. Paraneoplastic bullous pemphigoid resembling erythema gyratum repens. Br J Dermatol 1999; 140: 550–552. 13 Pique E, Palacios S, Santana Z. Leukocytoclastic vasculitis presenting as an erythema gyratum repens-like eruption on a patient with systemic lupus erythematosus. J Am Acad Dermatol 2002; 47(Suppl.): 254–256. 14 Campbell L, Freedman JR, ODonoghue M, et al. Erythema gyratum repens without associated malignancy. J Am Acad Dermatol 2011; 65: e22–e23.
Tropical medicine rounds
References 1 National Leprosy Eradication Program. Progress report as on 31st March 2011. http://nlep.nic.in/. [Accessed April 21, 2012]. 2 Gorin RJ, Chaplik I, Deitz M. Hansens disease masquerading as facial keloid. Poster session presented at: American Academy of Dermatology 62nd annual meeting, Feb 6–11, 2004, Washington DC. J Am Acad Dermatol 2004; 50: P1–P198. 3 Wong SM, Ho V. Leprosy mistaken for physical abuse. Poster session presented at American Academy of Dermatology 65th annual meeting, Feb 2–6, 2007, Washington DC. J Am Acad Dermatol 2007; 56: AB122. 4 Alberti JR, Cabrera A, Martiniuk F, et al. Leprosy masquerading as lupus. J Am Acad Dermatol 2005; 52: 702–703.
International Journal of Dermatology 2014, 53, 210–212
ª 2013 The International Society of Dermatology
