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Adult Still’s Disease Resembling Drug-related Scratch Dermatitis

73-year-old woman without any pertinent history was admitted to the hospital due to remittent fever with erythema. She showed itching and linearly arranged erythema on the chest, back, and abdomen [Figure 1a and b]. As she had been taking daily cefditoren pivoxil for the 4 days before her admission, she was diagnosed as having drug-related scratch dermatitis, and the antibiotic treatment was stopped. Her fever remained. Laboratory data showed elevated levels of white blood cells (14,800/μl, normal range 4000–7000) and liver enzymes such as aspartate aminotransferase (AST) 138 IU/L (normal range 5–40), alanine aminotransferase 97 IU/L (normal range 5–35), and ferritin (17469.5 ng/mL, normal range 5–152).

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Clinical Research in Hematology  •  Vol 1  •  Issue 2  •  2018 14
A
73-year-old woman without any pertinent history
was admitted to the hospital due to remittent fever
with erythema. She showed itching and linearly
arranged erythema on the chest, back, and abdomen [Figure
1a and b]. As she had been taking daily cefditoren pivoxil for
the 4 days before her admission, she was diagnosed as having
drug-related scratch dermatitis, and the antibiotic treatment
was stopped. Her fever remained. Laboratory data showed
elevated levels of white blood cells (14,800/μl, normal
range 4000–7000) and liver enzymes such as aspartate
aminotransferase (AST) 138 IU/L (normal range 5–40),
alanine aminotransferase 97 IU/L (normal range 5–35),
and ferritin (17469.5 ng/mL, normal range 5–152). A blood
culture was negative for bacteria. A computed tomography
examination revealed mild mediastinal lymphadenopathy
of the cervicum to the abdomen. Biopsies of mediastinal
lymph node, bone marrow, and skin were performed, and all
were negative for the infiltration of lymphoma cells or any
malignancy. As the patient’s fever and erythema remained, a
biopsy of the skin erythema was performed [Figure 1c and d].
The histopathological diagnosis was non-specific interface
dermatitis.As infection and malignancy were denied, she was
finally diagnosed as having adult Still’s disease (ASD) based
on the high ferritin level.[1-3]
Prednisolone (1 mg/kg/day) was
administered, and her fever and erythema disappeared within
1 week. The prednisolone was gradually tapered, and the
patient has maintained in complete remission.
ASD is an inflammatory disorder characterized by daily
fever, arthritis, and evanescent rash.[1,2,4,5]
The typical skin
rash of AST is a salmon-colored non-pruritic macular
eruption that disappears during afebrile periods. The rash
predominantly involves the trunk and extremities. However,
in our patient’s case, the erythema of ASD was itchy so that
it was indistinguishable from drug-related scratch dermatitis.
And also, the erythema sustained unrelated to fever. The
diagnosis of ASD is partly a diagnosis of exclusion and
should be distinguish from other disease which could show
similar symptoms and findings.[2]
Among the laboratory
abnormality seen in ASD, ferritin has been suggested to be
a good serologic marker not only for diagnosis but also for
monitoring the response to treatment.[3,6]
In this case, we
finally came to the diagnosis of ASD by excluding infection,
other autoimmune diseases, drug reaction, and malignancy
(mainly malignant lymphoma). It is important to realize that
a variety of dermatological symptoms could be observed
in ASD.[7,8]
And also, we should be aware that majority of
Adult Still’s Disease Resembling Drug-related
Scratch Dermatitis
Yutaka Shimazu, Yoichi Kato, Mariko Hara, Masaharu Nohgawa
Department of Hematology, Japanese Red Cross Wakayama Medical Center, Wakayama, Japan
Address for correspondence:
Yutaka Shimazu, Department of Hematology, Japanese Red Cross Wakayama Medical Center, 4-20, Komatsubara-dori,
Wakayama 640-8558, Wakayama, Japan. Phone: +81-73-422-4171. Fax: +81-73-426-1168. E-mail: yshimazu@kankyo.ne.jp
https://doi.org/10.33309/2639-8354.010204 www.asclepiusopen.com
© 2018 The Author(s). This open access article is distributed under a Creative Commons Attribution (CC-BY) 4.0 license.
EDITORIAL
Figure 1: Photos of skin lesion in this patient, a 73-year-old
woman. (a) Low-power field. (b) High-power field. (c and d)
Histological staining (hematoxylin and eosin staining) of the
biopsy specimen of the patient’s skin erythema. (c) Low-
power field, ×200. (d) High-power field, ×400
d
c
b
a
Editorial
 Clinical Research in Hematology  •  Vol 1  •  Issue 2  •  2018
the patients with atypical cutaneous lesion require more
aggressive treatment.[7,8]
In conclusion, recognition of the
clinical variant is crucial for the early diagnosis of ASD.
REFERENCES
1.	 Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R,
Mizushima Y, Kashiwagi H, et al. Preliminary criteria
for classification of adult still’s disease. J Rheumatol
1992;19:424-30.
2.	 Govoni M, Bortoluzzi A, Rossi D, Modena V. How I treat
patients with adult onset still’s disease in clinical practice.
Autoimmun Rev 2017;16:1016-23.
3.	 Schwarz-Eywill M, Heilig B, Bauer H, BreitbartA, Pezzutto A.
Evaluation of serum ferritin as a marker for adult still’s disease
activity. Ann Rheum Dis 1992;51:683-5.
4.	 Bywaters EG. Still’s disease in the adult. Ann Rheum Dis
1971;30:121-33.
5.	 Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sève P. Adult-onset
still’s disease. Autoimmun Rev 2014;13:708-22.
6.	 Van Reeth C, Le Moel G, Lasne Y, Revenant C, Agneray J.
Elevation of serum ferritin as a marker for adult still’s disease
activity. J Rhematol 1994;21:890.
7.	 Yamamoto T. Cutaneous manifestations associated with adult-
onset still’s disease: Important diagnostic values. Rheumatol
Int 2012;32:2233-7.
8.	 Narváez Garcia FJ, Pascual M, López de Recalde M, Juarez P,
Morales-Ivorra I, Notario J, et al. Adult-onset still’s disease
with atypical cutaneous manifestations. Medicine (Baltimore)
2017;96:e6318.
How to cite this article: Shimazu Y, Kato Y, Hara M,
Nohgawa M. Adult Still’s Disease Resembling
Drug-related Scratch Dermatitis. Clin Res Hematol
2018;1(2):14-15.

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Adult Still’s Disease Resembling Drug-related Scratch Dermatitis

  • 1. Clinical Research in Hematology  •  Vol 1  •  Issue 2  •  2018 14 A 73-year-old woman without any pertinent history was admitted to the hospital due to remittent fever with erythema. She showed itching and linearly arranged erythema on the chest, back, and abdomen [Figure 1a and b]. As she had been taking daily cefditoren pivoxil for the 4 days before her admission, she was diagnosed as having drug-related scratch dermatitis, and the antibiotic treatment was stopped. Her fever remained. Laboratory data showed elevated levels of white blood cells (14,800/μl, normal range 4000–7000) and liver enzymes such as aspartate aminotransferase (AST) 138 IU/L (normal range 5–40), alanine aminotransferase 97 IU/L (normal range 5–35), and ferritin (17469.5 ng/mL, normal range 5–152). A blood culture was negative for bacteria. A computed tomography examination revealed mild mediastinal lymphadenopathy of the cervicum to the abdomen. Biopsies of mediastinal lymph node, bone marrow, and skin were performed, and all were negative for the infiltration of lymphoma cells or any malignancy. As the patient’s fever and erythema remained, a biopsy of the skin erythema was performed [Figure 1c and d]. The histopathological diagnosis was non-specific interface dermatitis.As infection and malignancy were denied, she was finally diagnosed as having adult Still’s disease (ASD) based on the high ferritin level.[1-3] Prednisolone (1 mg/kg/day) was administered, and her fever and erythema disappeared within 1 week. The prednisolone was gradually tapered, and the patient has maintained in complete remission. ASD is an inflammatory disorder characterized by daily fever, arthritis, and evanescent rash.[1,2,4,5] The typical skin rash of AST is a salmon-colored non-pruritic macular eruption that disappears during afebrile periods. The rash predominantly involves the trunk and extremities. However, in our patient’s case, the erythema of ASD was itchy so that it was indistinguishable from drug-related scratch dermatitis. And also, the erythema sustained unrelated to fever. The diagnosis of ASD is partly a diagnosis of exclusion and should be distinguish from other disease which could show similar symptoms and findings.[2] Among the laboratory abnormality seen in ASD, ferritin has been suggested to be a good serologic marker not only for diagnosis but also for monitoring the response to treatment.[3,6] In this case, we finally came to the diagnosis of ASD by excluding infection, other autoimmune diseases, drug reaction, and malignancy (mainly malignant lymphoma). It is important to realize that a variety of dermatological symptoms could be observed in ASD.[7,8] And also, we should be aware that majority of Adult Still’s Disease Resembling Drug-related Scratch Dermatitis Yutaka Shimazu, Yoichi Kato, Mariko Hara, Masaharu Nohgawa Department of Hematology, Japanese Red Cross Wakayama Medical Center, Wakayama, Japan Address for correspondence: Yutaka Shimazu, Department of Hematology, Japanese Red Cross Wakayama Medical Center, 4-20, Komatsubara-dori, Wakayama 640-8558, Wakayama, Japan. Phone: +81-73-422-4171. Fax: +81-73-426-1168. E-mail: yshimazu@kankyo.ne.jp https://doi.org/10.33309/2639-8354.010204 www.asclepiusopen.com © 2018 The Author(s). This open access article is distributed under a Creative Commons Attribution (CC-BY) 4.0 license. EDITORIAL Figure 1: Photos of skin lesion in this patient, a 73-year-old woman. (a) Low-power field. (b) High-power field. (c and d) Histological staining (hematoxylin and eosin staining) of the biopsy specimen of the patient’s skin erythema. (c) Low- power field, ×200. (d) High-power field, ×400 d c b a
  • 2. Editorial Clinical Research in Hematology  •  Vol 1  •  Issue 2  •  2018 the patients with atypical cutaneous lesion require more aggressive treatment.[7,8] In conclusion, recognition of the clinical variant is crucial for the early diagnosis of ASD. REFERENCES 1. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult still’s disease. J Rheumatol 1992;19:424-30. 2. Govoni M, Bortoluzzi A, Rossi D, Modena V. How I treat patients with adult onset still’s disease in clinical practice. Autoimmun Rev 2017;16:1016-23. 3. Schwarz-Eywill M, Heilig B, Bauer H, BreitbartA, Pezzutto A. Evaluation of serum ferritin as a marker for adult still’s disease activity. Ann Rheum Dis 1992;51:683-5. 4. Bywaters EG. Still’s disease in the adult. Ann Rheum Dis 1971;30:121-33. 5. Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sève P. Adult-onset still’s disease. Autoimmun Rev 2014;13:708-22. 6. Van Reeth C, Le Moel G, Lasne Y, Revenant C, Agneray J. Elevation of serum ferritin as a marker for adult still’s disease activity. J Rhematol 1994;21:890. 7. Yamamoto T. Cutaneous manifestations associated with adult- onset still’s disease: Important diagnostic values. Rheumatol Int 2012;32:2233-7. 8. Narváez Garcia FJ, Pascual M, López de Recalde M, Juarez P, Morales-Ivorra I, Notario J, et al. Adult-onset still’s disease with atypical cutaneous manifestations. Medicine (Baltimore) 2017;96:e6318. How to cite this article: Shimazu Y, Kato Y, Hara M, Nohgawa M. Adult Still’s Disease Resembling Drug-related Scratch Dermatitis. Clin Res Hematol 2018;1(2):14-15.