Erythema Gyratum Repens with Esophageal Carcinoma

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.


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.

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).
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).

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.
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

Diagnosis
Erythema gyratum repens associated with esophageal adenocarcinoma and Barrett syndrome (G2, category 4.3 of the modified Vienna classification [4].

Figure 2 :
Figure 2: Histopathology of erythema gyratum repens with an inflammatory infiltrate in upper dermis and around skin appendages (HE x 10).

Figure 3 :
Figure 3: Endoscopy of distal esophagus with Barrett syndrome but without a primary tumor suspicion.