Brief Report

Vol. 30, No. 6, 2018 733

Received June 27, 2017, Revised November 21, 2017, Accepted for publication December 31, 2017

Corresponding author: Jeonghyun Shin, Department of Dermatology, Inha University Hospital, 27 Inhang-ro, Jung-gu, Incheon 22332, Korea. Tel: 82-32-890-2238, Fax: 82-32-890-2236, E-mail: jshin@inha.ac.krORCID: https://orcid.org/0000-0002-4995-9533

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Copyright © The Korean Dermatological Association and The Korean Society for Investigative Dermatology

Fig. 1. Recurrent large purpuric erythematous annular patch on the left side of the chest. (A) Photo-graph taken in May 2015. Patient had had recurrent eruptions at the same location 6, 8, and 10 years previously. (B) January 2009 photo-graph shows a large erythematous patch with purpuric papules on the left chest, which regressed com-pletely without treatment. Black circles indicate the biopsy sites (A: margin of erythematous patch, B: purpuric papule). We received the patient’s consent form about publi-shing all photographic materials.

https://doi.org/10.5021/ad.2018.30.6.733

An Atypical Erythromelalgia on the Chest

Si Hyub Lee, Hee Seong Yoon, Seung Dohn Yeom, Ji Won Byun, Gwang Seong Choi, Jeonghyun Shin

Department of Dermatology, Inha University School of Medicine, Incheon, Korea

Dear Editor:Erythromelalgia is a rare clinical syndrome that is charac-terized by episodic intense burning pain and marked er-ythema usually on the distal extremities1. It is classified as a primary or secondary condition depending on whether there are underlying diseases, such as myeloproliferative disease, autoimmune disease, and neurological disorders. We report a case of atypical clinical presentation of erythromelalgia.A 74-year-old woman presented with a large annular er-ythematous to purpuric patch on her left chest that had persisted for 6 months (Fig. 1A). She reported that sensory functions on the left face, neck and chest had diminished after neurosurgery for cervical syringomyelia 30 years ago. She complained a burning and heating sensation on the lesion and flushing of the ipsilateral neck and ear, which

was somewhat relieved by cooling. This was the fourth time that the patch had occurred at the same location for 10 years. The previous episodes used to occur suddenly and regress spontaneously over several weeks. She denied any preceding psychologic events or other concomitant symptoms. She said the elevation of the body temperature seemed to worsen the symptom. She has not had any oth-er therapy like acupuncture. The patient had no other medical history except for osteoporosis and no remarkable family history. Laboratory tests, including complete blood count, blood chemistry, erythrocyte sedimentation rate, and antinuclear antibody levels were within normal limits or negative. Prothrombin time and activated partial throm-boplastin time were also in normal values. Histopathologi-cal findings were nonspecific except for dilated capillaries in the dermis (Fig. 2A, B). There was no vasculitis nor er-

Brief Report

734 Ann Dermatol

Fig. 2. Two skin biopsies collected in January 2009 and May 2015 yielded the same results. (A, B) Biopsy was taken at the margin of the patch in May 2015: dilated capillaries in upper dermis and otherwise unremarkable findings. (C, D) Biopsy was taken at the purpuric papule in January 2009: histopathology revealed thin epi-dermis with flattened dermoepi-dermal junction and telangiectasia in dermis. H&E, (A, C) ×40 and (B, D) ×200.

ythrocyte extravasation. These findings were the same as when she had previously visited our department for the same chief complaint 6 years prior in 2009 (Fig. 1B, 2C, 2D). The human herpes virus 8 stain was negative.Based on the clinical and histopathological findings, we diagnosed the patient with erythromelalgia on the chest. We assumed that it was secondary to syringomyelia be-cause the skin lesion was confined to the area of neuro-logic abnormality. The clinical progress of the eryth-romelalgia waxed and waned despite treatment with low-dose oral steroids, aspirin, and gabapentin. It sus-tained for 3 months more and regressed spontaneously without medication.Erythromelalgia is usually triggered by heat exposure or exercise, and it tends to be alleviated by cooling2. Although it commonly involves the feet and hands, eryth-romelalgia can also occur on the face or ears1,3. Histo-pathological findings are often nonspecific2. Because there is no confirmatory diagnostic test, clinical suspicion, along with a detailed history and physical examination during episodes, is required to establish a diagnosis. The differ-ential diagnosis of this case should be early stage of Kaposi sarcoma, and painful bruising syndrome. The hu-man herpes virus 8 stain was negative and the clinical course was not compatible with Kaposi sarcoma in that the lesion had occurred abruptly and regressed spon-taneously. Painful bruising syndrome (psychogenic pur-pura, autoerythrocyte sensitization syndrome) shows re-

current bruise or ecchymosis with psychologic events. However, the lesion of the present case was not exactly an ecchymosis in the clinical and histopathologic findings, but rather a well-defined erythematous to purpuric patch, and had lasted several weeks to months. The recurrent ep-isodes of the lesion had not been associated with emo-tional provocation.The exact pathological mechanism of erythromelalgia re-mains unclear. Primary erythromelalgia is classified as a channelopathy caused by autosomal dominant mutations in SCN9A, which encodes the sodium channel alpha sub-unit4. Secondary erythromelalgia may accompany a num-ber of diseases, including myeloproliferative disease with thrombocythemia, diabetes, hypertension, rheumatoid ar-thritis, systemic lupus erythematosus, infectious diseases, and musculoskeletal and neurological disorders1. Therefore, once a diagnosis is established, potential secondary caus-es must be excluded.We have found 11 cases of erythromelalgia associated with spinal cord pathology in the literature1,4, and one case report of erythromelalgia presenting on the face, ear and chest in a healthy young woman of European descent5.The natural clinical course of erythromelalgia varies. Although there are descriptions of many therapeutic regi-mens, such as aspirin, neuroleptics, and vasoactive agents, the optimal therapy remains unclear1,2. Our patient did not response to drug therapy, and her erythromelalgia re-gressed spontaneously.

Brief Report

Vol. 30, No. 6, 2018 735

Received August 16, 2017, Revised November 10, 2017, Accepted for publication December 31, 2017

Corresponding author: Jung-Im Na, Department of Dermatology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, 82 Gumi-ro 173beon-gil, Bundang-gu, Seongnam 13620, Korea. Tel: 82-31-787-7314, Fax: 82-31-787-4058, E-mail: vividna@gmail.comORCID: https://orcid.org/0000-0002-5717-2490

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Copyright © The Korean Dermatological Association and The Korean Society for Investigative Dermatology

ACKNOWLEDGMENT

This work was supported by Inha University Research grant.

CONFLICTS OF INTEREST

The authors have nothing to disclose.

REFERENCES

1. Cohen JS. Erythromelalgia: new theories and new therapies. J

Am Acad Dermatol 2000;43:841-847.2. Davis MD, O’Fallon WM, Rogers RS 3rd, Rooke TW.

Natural history of erythromelalgia: presentation and outcome in 168 patients. Arch Dermatol 2000;136:330-336.

3. Patel M, Femia AN, Eastham AB, Lin J, Canales AL, Vleugels

RA. Facial erythromelalgia: a rare entity to consider in the differential diagnosis of connective tissue diseases. J Am

Acad Dermatol 2014;71:e250-e251.

4. Mosel DD, Rosler D. Erythromelalgia presenting after neuro-surgical intervention in a patient with multiple malforma-

tions of the spinal cord. J Am Acad Dermatol 2011;65:

e120-e122.5. Gaur S, Koroscil T. Late-onset erythromelalgia in a pre-

viously healthy young woman: a case report and review of

the literature. J Med Case Rep 2009;3:106.

https://doi.org/10.5021/ad.2018.30.6.735

Two Cases of Pigmented Contact Dermatitis Caused by Pure Henna Hair Dyes

Jung-Won Shin, Ji Young Choi, Chang-Hun Huh, Jung-Im Na

Department of Dermatology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea

Dear Editor:Pigmented contact dermatitis (PCD) is a variant of contact dermatitis, in which brown-gray reticulated pigmentation is the main symptom rather than typical eczematous changes. We present two cases of PCD caused by pure henna.A 60-year-old woman presented with reticulated, grayish hyperpigmentation over her face, with mild erythema (Fig. 1A). We received the patient’s consent form about publish-ing all photographic materials. She used a natural hen-na-based hair dye (HennakingTM; NaturalHealthKorea, Seoul, Korea) 2 months prior, with lesions developing af-

ter the second use. Histopathological examination re-vealed vacuolar degeneration of the epidermis and numer-ous dermal melanophages (Fig. 1B, C). Patch testing with the Korean standard series (KOR-1000, ChemotechniqueⓇ; Chemotechinique Diagnostics, Vellinge, Sweden), as well as her cosmetics and her hair dye (red henna powder 20% aqua), was positive to potassium dichromate (+/+, D2 and D4) and red henna (±/+), and negative to other com-pounds, including ρ-paraphenylenediamine (PPDA). The henna-based dye was analyzed by Safety Monitor Corps Eco Friendly Lab Inc. (SEL Inc., Asan, Korea; http:// www.sel.re.kr) and no chromium or chrome salts were