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Erythroderma: A unique presentation of acute lupus
i n d i a n j o u r n a l o f r h e u m a t o l o g y x x x ( 2 0 1 5 ) 1 e2
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Letter to the Editor
Erythroderma: A unique presentation of acute lupus
A 38-year-old lady with no known co morbidities presented with complaints of red scaly non itchy lesions over body of 16 days duration. Lesions started from face and increased rapidly to involve rest of the body over three days. There was associated history of photosensitivity, however there was no history of joint pains, oral ulcers, loss of hair, fever, dyspnea on exertion or chest pain. There was no history of similar lesions in past, preceding drug intake or history of atopy in self and family. General physical examination revealed pallor and pedal edema. Dermatological examination revealed generalised involvement of body with erythema and scaling, Nikolsky sign was negative and there was no oral, ocular or genital mucosal involvement (Fig. 1). Rest of the systemic examination was within normal limits. Investigations revealed Hemoglobin e 5.6 gm/dL, Total Leukocyte Count e 3800/mm3, Platelet Count e 1.8 lakh/mm3, Blood Urea e 96 mg/dL, Serum Creatinine e 2.6 mg/dL, ANA e 27 IU/mL, Anti dsDNA e Positive. Anti Ro/La, liver function test including SGOT, SGPT were within normal limits. Hepatitis B surface antigen, Antibodies to HCV and HIV were negative. Histopathological examination of skin biopsy revealed normal epidermis, degeneration of basal layer and interface dermatitis (Fig. 2). In view of erythroderma initially drug hypersensitivity and pemphigus foliaceus were considered, however in view of ANA and Anti dsDNA positivity, kidney involvement, anemia, thrombocytopenia and compatible histopathological features she was diagnosed as a case of acute lupus erythematosus. She was treated with Prednisolone 1 mg/Kg apart from supportive therapy. Three days after admission she had severe metabolic acidosis and needed hemodialysis and mechanical ventilation. Her condition did not improve and she died on day 5 of admission.
1.
histopathological features, specific histopathological feature being presence of interface dermatitis. Lupus specific cutaneous lesions have been further subdivided into acute, subacute and chronic cutaneous lupus depending on clinical characteristic and association with systemic disease.1 The risk of systemic involvement is highest with Acute Cutaneous Lupus Erythematosus (ACLE), lowest with Chronic Cutaneous Lupus Erythematosus (CCLE) with risk being intermediate in Subacute Cutaneous Lupus Erythematosus (SCLE).2 ACLE presents as transient localized (malar rash) or generalised (morbilliform) eruption, it can also present with Toxic Epidermal Necrolysis (TEN) like presentation rarely.3 ACLE is reported in 20e60% of lupus patient in various studies out of which generalised ACLE is seen in approximately 10% cases. Generalised ACLE presents as maculopaular exanthema with predilection for involvement of
Discussion
Systemic Lupus Erythematosus (SLE) is a multisystem autoimmune disease of unknown etiology. Skin is one of the target organ which is involved in SLE and involvement of skin not only leads to morbidity in SLE but also helps in early diagnosis of the disease. Gilliam classified cutaneous lupus into lupus specific and non specific lesion depending on
Fig. 1 e Generalised involvement of body with erythema and scaling.
Please cite this article in press as: Neema S, et al., Erythroderma: A unique presentation of acute lupus, (2015), http://dx.doi.org/ 10.1016/j.injr.2015.05.006
2
i n d i a n j o u r n a l o f r h e u m a t o l o g y x x x ( 2 0 1 5 ) 1 e2
references
Fig. 2 e Histopathological examination shows normal epidermis, degeneration of basal layer and interface dermatitis.
photoexposed and extensor aspects of the body.4 ACLE is a good clinical marker for underlying systemic disease and identification of varying manifestation of ACLE can lead one to suspect and diagnose Lupus. Diagnosis of generalised ACLE requires high degree of suspicion as it can be mistaken for various other conditions like drug rash, viral exanthema or dermatomyositis. We report erythroderma as a presentation of acute lupus which has never been reported earlier in literature. It is important to be aware of erythrodermic presentation of acute lupus as it was associated with fulminant course and fatal outcome.
1. Gilliam JN, Sontheimer RD. Distinctive cutaneous subsets in the spectrum of lupus erythematosus. J Am Acad Dermatol. 1981;4:471e475. 2. Watanabe T, Tsuchida T. Classification of lupus erythematosus based upon cutaneous manifestations. Dermatological, systemic and laboratory findings in 191 patients. Dermatology. 1995;190:277e283. 3. Ting W, Stone MS, Racila D, et al. Toxic epidermal necrolysislike acute cutaneous lupus erythematosus and the spectrum of the acute syndrome of apoptotic pan-epidermolysis (ASAP): a case report, concept review and proposal for new classification of lupus erythematosus vesiculobullous skin lesions. Lupus. 2004;13:941e950. 4. Cervera R, Khamashta MA, Font J, et al. Systemic lupus erythematosus:clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The European Working Party on Systemic Lupus Erythematosus. Medicine (Baltimore). 1993;72:113e124.
Shekhar Neema* Department of Dermatology, Command Hospital (EC), Alipore Road, Kolkata 700027, India Manas Chatterjee Department of Dermatology, Command Hospital (EC), Alipore Road, Kolkata 700027, India Atoshi Basu Department of Pathology, Command Hospital (EC), Alipore Road, Kolkata 700027, India *Corresponding author. Tel.: þ91 9830032821 (moblie). E-mail address: [email protected] Available online xxx 0973-3698/$ e see front matter Copyright © 2015, Indian Rheumatology Association. All rights reserved. http://dx.doi.org/10.1016/j.injr.2015.05.006
Please cite this article in press as: Neema S, et al., Erythroderma: A unique presentation of acute lupus, (2015), http://dx.doi.org/ 10.1016/j.injr.2015.05.006
ScienceDirect Available online at www.indianjrheumatol.com and www.sciencedirect.com
Letter to the Editor
Erythroderma: A unique presentation of acute lupus
A 38-year-old lady with no known co morbidities presented with complaints of red scaly non itchy lesions over body of 16 days duration. Lesions started from face and increased rapidly to involve rest of the body over three days. There was associated history of photosensitivity, however there was no history of joint pains, oral ulcers, loss of hair, fever, dyspnea on exertion or chest pain. There was no history of similar lesions in past, preceding drug intake or history of atopy in self and family. General physical examination revealed pallor and pedal edema. Dermatological examination revealed generalised involvement of body with erythema and scaling, Nikolsky sign was negative and there was no oral, ocular or genital mucosal involvement (Fig. 1). Rest of the systemic examination was within normal limits. Investigations revealed Hemoglobin e 5.6 gm/dL, Total Leukocyte Count e 3800/mm3, Platelet Count e 1.8 lakh/mm3, Blood Urea e 96 mg/dL, Serum Creatinine e 2.6 mg/dL, ANA e 27 IU/mL, Anti dsDNA e Positive. Anti Ro/La, liver function test including SGOT, SGPT were within normal limits. Hepatitis B surface antigen, Antibodies to HCV and HIV were negative. Histopathological examination of skin biopsy revealed normal epidermis, degeneration of basal layer and interface dermatitis (Fig. 2). In view of erythroderma initially drug hypersensitivity and pemphigus foliaceus were considered, however in view of ANA and Anti dsDNA positivity, kidney involvement, anemia, thrombocytopenia and compatible histopathological features she was diagnosed as a case of acute lupus erythematosus. She was treated with Prednisolone 1 mg/Kg apart from supportive therapy. Three days after admission she had severe metabolic acidosis and needed hemodialysis and mechanical ventilation. Her condition did not improve and she died on day 5 of admission.
1.
histopathological features, specific histopathological feature being presence of interface dermatitis. Lupus specific cutaneous lesions have been further subdivided into acute, subacute and chronic cutaneous lupus depending on clinical characteristic and association with systemic disease.1 The risk of systemic involvement is highest with Acute Cutaneous Lupus Erythematosus (ACLE), lowest with Chronic Cutaneous Lupus Erythematosus (CCLE) with risk being intermediate in Subacute Cutaneous Lupus Erythematosus (SCLE).2 ACLE presents as transient localized (malar rash) or generalised (morbilliform) eruption, it can also present with Toxic Epidermal Necrolysis (TEN) like presentation rarely.3 ACLE is reported in 20e60% of lupus patient in various studies out of which generalised ACLE is seen in approximately 10% cases. Generalised ACLE presents as maculopaular exanthema with predilection for involvement of
Discussion
Systemic Lupus Erythematosus (SLE) is a multisystem autoimmune disease of unknown etiology. Skin is one of the target organ which is involved in SLE and involvement of skin not only leads to morbidity in SLE but also helps in early diagnosis of the disease. Gilliam classified cutaneous lupus into lupus specific and non specific lesion depending on
Fig. 1 e Generalised involvement of body with erythema and scaling.
Please cite this article in press as: Neema S, et al., Erythroderma: A unique presentation of acute lupus, (2015), http://dx.doi.org/ 10.1016/j.injr.2015.05.006
2
i n d i a n j o u r n a l o f r h e u m a t o l o g y x x x ( 2 0 1 5 ) 1 e2
references
Fig. 2 e Histopathological examination shows normal epidermis, degeneration of basal layer and interface dermatitis.
photoexposed and extensor aspects of the body.4 ACLE is a good clinical marker for underlying systemic disease and identification of varying manifestation of ACLE can lead one to suspect and diagnose Lupus. Diagnosis of generalised ACLE requires high degree of suspicion as it can be mistaken for various other conditions like drug rash, viral exanthema or dermatomyositis. We report erythroderma as a presentation of acute lupus which has never been reported earlier in literature. It is important to be aware of erythrodermic presentation of acute lupus as it was associated with fulminant course and fatal outcome.
1. Gilliam JN, Sontheimer RD. Distinctive cutaneous subsets in the spectrum of lupus erythematosus. J Am Acad Dermatol. 1981;4:471e475. 2. Watanabe T, Tsuchida T. Classification of lupus erythematosus based upon cutaneous manifestations. Dermatological, systemic and laboratory findings in 191 patients. Dermatology. 1995;190:277e283. 3. Ting W, Stone MS, Racila D, et al. Toxic epidermal necrolysislike acute cutaneous lupus erythematosus and the spectrum of the acute syndrome of apoptotic pan-epidermolysis (ASAP): a case report, concept review and proposal for new classification of lupus erythematosus vesiculobullous skin lesions. Lupus. 2004;13:941e950. 4. Cervera R, Khamashta MA, Font J, et al. Systemic lupus erythematosus:clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The European Working Party on Systemic Lupus Erythematosus. Medicine (Baltimore). 1993;72:113e124.
Shekhar Neema* Department of Dermatology, Command Hospital (EC), Alipore Road, Kolkata 700027, India Manas Chatterjee Department of Dermatology, Command Hospital (EC), Alipore Road, Kolkata 700027, India Atoshi Basu Department of Pathology, Command Hospital (EC), Alipore Road, Kolkata 700027, India *Corresponding author. Tel.: þ91 9830032821 (moblie). E-mail address: [email protected] Available online xxx 0973-3698/$ e see front matter Copyright © 2015, Indian Rheumatology Association. All rights reserved. http://dx.doi.org/10.1016/j.injr.2015.05.006
Please cite this article in press as: Neema S, et al., Erythroderma: A unique presentation of acute lupus, (2015), http://dx.doi.org/ 10.1016/j.injr.2015.05.006