Case Report Open Access
Erythema Gyratum Repens with Esophageal Carcinoma
Langner D1, Wollschlager S2, Schonlebe J3, Wollina U1*
1Department of Dermatology and Allergology
2Department of Internal Medicine III
3Institute for Pathology "Georg Schmorl" Academic Teaching Hospital Dresden-Friedrichstadt, Städtisches Klinikum, Dresden, Germany
*Corresponding author: Prof. Dr. U Wollina, Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067 Dresden, Germany. E-mail: @
Received: July 11, 2016; Accepted: July 17, 2016; Published: July 22, 2016
Citation: Langner D, Wollschlager S, Schonlebe J, Wollina U (2016) Erythema Gyratum Repens with Esophageal Carcinoma. Clin Res Dermatol Open Access 3(5): 1-3.
Abstract
Erythema gyratum repens represents a rare facultative paraneoplasia. Quite often the dermatosis precedes tumor diagnosis. We report a 76-year-old male patient who initially presented with non-characteristic erythematous papules. After the diagnosis of an adenocarcinoma of the lower third of the esophagus the morphology of the dermatoses transformed into typically figurated erythemas with pronounced margins. Our case might support the hypothesis of cutaneous inflammation induced by crystallization of glutamine compounds in the skin. Tumor therapy was palliative by radiation. The erythema responded to a combined approach using topical corticosteroids and PUVA.

Keywords: Erythema Gyratum Repens; Paraneoplasia; Esophageal Carcinoma
Introduction
The first description of Erythema gyratum repens was made by Gammel who observed this rare skin disease in 1952 in a 55-year-old female with metastatic adenocarcinoma of the breast. Two days after mastectomie and axillary lymph node dissection erythema gradually disappeared. Six weeks later a complete remission was achieved [1].

In the majority of cases erythema gyratum repens is associated with a malignant tumor and represents a paraneoplastic dermatosis. The most common malignancy is lung cancer (32%), followed by esophageal cancer (8%) and breast cancer (6%) [2,3].

The current concept of pathophysiology assumes a tumorinduced humoral and/ or cellular immune response resulting in a cross-reactivity to skin or deposition of tumor antigen-antibody immunocomplexes along the basement membrane [2].

The typical clinical presentation is that of annular, garland shaped or spiral shaped erythemas with elevated borders, slight infiltration and scaling also known as wood grain pattern. The speed of propoagation reaches up to 10 cm per day [1].

Clinical presentation, however, may vary. About one third of patients do no suffer from malignant tumors, what fullfills the criterion of a facultative paraneoplastic dermatosis.
Case Report
Medical history
A 76-year-old male patient presented with pruritic and prgressive cutaneous lesions localized mainly of his trunk. The differential diagnoses at this time included eosinophilic dermatosis or systemic mastocytosis due to peripheral blood hypereosinophilia. He reported that 6 years and 6 months before similar lesions developed, that responded to psoralen Plus UltraViolett (UV)-Irradiation (PUVA) and topical corticosteroids.

During the last weeks, he expienrence an acute relapse associated with fatigue, loss of appetite, weightloss and pruitus.

Clinical investigations of internal organs were unremarkable. His skin, however, demonstrated multiple erythematous papules and plaques on trunk and proximal extremities. Darier sign was negative. During his stay in the hospital he developed target-like slightly elevated erythematous lesions (Fig. 1a, b).

A skin biopsy revealed an orthokeratotic epidermis with inclusions of serum and leukocytes and a psoriasisform pattern. In the central parts of the lesions spongiosis and parakeratosis was noted. There was a partly perivascular, partly interstitial inflammatory infiltrate composed of lymphocytes and monocytes, some neutrophilic and eosinophilic granulocytes, and occasional mast cells.Tere were no signs of a mycotic infection (Fig. 2).

Laboratory investigations: Hemoglobin 8.20 mmol/l (↓), hematokrit 0.398 l/l (↓), neutrophils 8.90 Gpt/l (↑), eosinophils 0.70 Gpt/l (↑), mast cell tryptase 22.1 μg/l (↑). The other parameters were in the normal range.

Imaging: X-ray and computerized tomography demonstrated some mediastinal calcifiyed lymph nodes. Abdominal sonography and coloscopy were unremarkable. Gastroscopy demonstrated erosions and inflammatory lesions in the distal third of the esophagus and erosions on the antrum (Fig. 3). Under the suspicion of a Barrett syndrome (Differential diagnosis: erosive antrum gastritis) a biopsy was taken. Histologic investigations revealed a moderate differentiated adenocarcinoma of the esophagus on Barrett syndrome (Fig. 4a,b).
Figure 1: Clinical presentation of erythema gyratum repens. (a) Periumbilical figurated erythema. (b) On the legs erythematous plques with slightly elevated borders.
Figure 2: Histopathology of erythema gyratum repens with an inflammatory infiltrate in upper dermis and around skin appendages (HE x 10).
Figure 3: Endoscopy of distal esophagus with Barrett syndrome but without a primary tumor suspicion.
Figure 4: Histology of esophageal adenocarcinoma. (a) HE x 10; (b) PAS-stain (x10).
Diagnosis
Erythema gyratum repens associated with esophageal adenocarcinoma and Barrett syndrome (G2, category 4.3 of the modified Vienna classification [4].
Therapy and course
Topical treatment was realized using fluocinolone acetonid ointment twice daily in combination with bath-PUVA (8 sessions). Antipruritic oral treatment was realized by fexofenadin and ranitidin. This resulted in significant improvement of cutaneous lesions and complaints.

The cancer was treated by radiotherapy. Cutaneous lesions changed into wood grain pattern erythema but disappeared step by step thereafter.
Discussion
In case of paraneoplastic erythema gyratum repens, skin lesions often develop several months before detection of cancer [5]. Differential diagnosis include erythema gyratum repens-like mycosis fungoides [6], paraneoplastic bullous uemphigoid [7] or erythema gyratum-like bullous lupus erythematosus [8]. Other figurated erythemas also need consideration (Table 1).

Our case is remarkable because of initially unspecific cutaneous lesions. After the diagnosis of esophageal adenocarcinoma the lesions shape-shifted into the characteristic wood grain pattern. The case illustrates that erythema gyratum repends may develop from nonspecific cutaneous erythemas.

The etiology of erythema gyratum repens remains unclear. Recently, Forrester discussed a possible relationsship to L-glutamine cristallization in living tissues. Glutamine is released
Table 1: Functional characterization of expanded CD8+ Mart-1 antigen specific cells after initial stimulation and re-stimulation with appropriate antigen

Disease

Remarks

Bullous pemphigoid

rare manifestation, BP-antibodies

Dermatitis herpetiformis Duhring

 

antibodies to endomysium and transglutaminase

 

Erythema annulare centrifugum

 

association to allergy, infections, tumors

 

Eosinophilic annular erythema

subtype of Wells syndrome

Erythema chronicum migrans

 

Borelliosis

 

Erythema gyratum repens

facultative paraneoplasia

Erythema papulosa semicircularis  recidivans

 

 

acute phases during hot summer time,

unknown etiology

 

Erythrokeratodermia variabilis

genodermatosis, disorder of  keratinization

Granuloma annulare

histopathology with dermalen granulomas

IgA-lineäre Dermatose

rarely figurate erythemas, akantholysis

Lupus gyratum repens

rare Lupus erythematosus variant

 

Neutrophilic figurated erythema

 

may be associated with leukemia/lymphoma of childhood

 

 

Psoriasis

 

rarely with figurated erythemas

 

 

Sweet-Syndrom

 

figurated erythemas possible, neutrophilic

dermatosis

 

Urticaria

may show with elevated borders

Summary and Conclusions
Erythema gyratum repens is a rare facultative cutaneous paraneoplasia. Initial symptoms can be uncharacteristic, wood grain pattern lesions develop only in mature stages. In about 1/3 of cases the disease is not tumor-associated. Nevertheless, a diagnosis of erythema gyratum repens warrants an in depth search for possible underlying malignancies.
References
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