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Indian J Dermatol
Indian J Dermatol
IJD
Indian J Dermatol
Indian Journal of Dermatology
0019-5154
1998-3611
Wolters Kluwer - Medknow India

IJD-69-350
10.4103/ijd.ijd_281_24
Correspondences
A Gardner-Diamond Syndrome Accompanied by Complex Regional Pain Syndrome
Yokoyama Emi
Kawakami Yoshio
Okada Ayumi 1
Yashiro Masato 2
Tetsunaga Tomoko 3
Morizane Shin
From the Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan E-mail: emiyoko1203@smile.ocn.ne.jp
1 Department of Child Psychosomatic Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan
2 Department of Locomotive Pain Center, Division of Rheumatic Diseases Okayama University Hospital, Okayama, Japan
3 Department of Orthopedic Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan
Jul-Aug 2024
19 8 2024
69 4 350352
3 2024
3 2024
Copyright: © 2024 Indian Journal of Dermatology
2024
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pmcDear Editor,

Gardner-Diamond syndrome (GDS), also known as psychogenic purpura, is characterized by spontaneous, painful ecchymosis occurring after psychological stress, with a predilection for the extremities in young women.[1] Complex regional pain syndrome (CRPS) is a neuropathic disorder exhibiting sensory (hyperesthesia and allodynia), vasomotor (temperature asymmetry and skin color changes), sudomotor/oedema (oedema and sweating changes), and motor/trophic (motor impairment and trophic) symptoms.[2] Herein, we report a case of GDS accompanied by CRPS.

A 14-year-old girl presented with a two-week history of painful eruptions on the right thigh that had been preceded by two years of chronic pain in the same region. Five years earlier, she had developed an avoidant/restrictive food intake disorder and no attendance at school. Subsequently, she started treatment at a paediatric psychosomatic medicine department. On initial examination, a well-defined purpura with localized warmth, 10 × 4 cm in size, on the right thigh was observed [Figure 1a]. Laboratory examination results, including platelet count, blood coagulation test, and autoantibodies, were within normal ranges. Magnetic resonance imaging (MRI) on T2-weighted images of the lesion showed high-intensity signals in the subcutaneous fatty tissue and superficial fascia [Figure 1b]. Histopathology showed extravasation of red blood cells and scattered neutrophilic infiltration in the subcutis [Figure 1c and d]. The ecchymosis disappeared a month later, and two weeks later, a similar ecchymosis sprouted anew on the right knee. [Figure 1e], and its histopathology revealed subcutaneous haemorrhage without vasculitis [Figure 1f and g]. Subsequently, there were episodic appearances of ecchymoses on extremities after psychological stresses, such as trials of school attendance and family troubles. Two years later, painful ecchymosis accompanied by hyperesthesia and swelling developed on the right forearm [Figure 2a and b], and thermography showed increased temperature in the lesional skin compared to the unaffected regions [Figure 2c and d]. Oral prednisolone (30 mg/day) alleviated the swelling, whereas chronic lesional pain persisted. One month later, the patient developed motor impairment and contractures of the right fingers, accompanied by brownish verrucous plaques consistent with Akatsuki disease [Figure 2e]. The latest treatment included pregabalin, mirogabalin, several sedatives, and antihistamines. After the patient became aware of possible psychological triggers for GDS, there was a reduction in the frequency of purpura formations.

Figure 1 (a) A well-defined purpura, 10 × 4 cm in size, on the right thigh at the first visit. (b) Magnetic resonance imaging (MRI) on T2-weighted images of the right thigh showing high-intensity signals in the subcutaneous fatty tissue and superficial fascia. (c) Haematoxylin and eosin (H-E) staining, 40×. (d) H-E staining 400×. Histopathology of the purpura on the right thigh showing extravasation of red blood cells and scattered neutrophilic infiltration in the subcutis. (e) An ecchymosis on the inner aspect of the right knee. (f) H-E staining 40×. (g) H-E staining 400×. Histopathology of the ecchymosis on the right knee showing subcutaneous haemorrhage without vasculitis

Figure 2 (a and b) An ecchymosis and swelling on the right forearm. (c and d) Thermography of the ecchymosis on the right forearm: a whitish area corresponds to the ecchymosis (35.0°C), whereas a reddish area corresponds to unaffected regions (34.2°C). (e) Contractures of the right fingers one month after the onset of the ecchymosis on the right forearm, accompanied by brownish verrucous plaques consistent with Akatsuki disease

The clinical symptoms on the right forearm met the Budapest criteria for the diagnosis of CRPS.[2] Thermography can be a good tool to quantify thermal changes in the affected limb of CRPS.[3] There are several cases of CRPS in association with GDS.[4] GDS is believed to be caused by sensitization to phosphatidylserine in autologous erythrocyte membranes and is diagnosed by autologous erythrocyte sensitization skin tests,[1] which were not conducted in our case due to an inability to obtain consent. The pathophysiology of CRPS is multifactorial, including nervous and immune system dysfunctions and potentially autoimmune, genetic, or psychological factors.[5] Therefore, autoimmune and psychological factors are likely to affect the development of both GDS and CRPS in the present case. The patient was initially suspected of self-harm or abuse, which affected her psychological burden. These syndromes should be recognized as a differential for painful purpura on the extremities.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and that due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.
==== Refs
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3 Pérez-Concha T Tijero B Acera M Fernández T Gabilondo I Gómez-Esteban JC Usefulness of thermography in the diagnosis and classification of complex regional pain syndrome Neurologia (Engl Ed) 2020 38 342 9
4 Edinger LK Schwartzman RJ Gardner-Diamond syndrome associated with complex regional pain syndrome J Dermatol Case Rep 2013 7 10 4 23580908
5 Scarff JR Managing psychiatric symptoms in patients with complex regional pain syndrome Innov Clin Neurosci 2022 19 56 9 35382068
