Positive photobiological investigation in reticular erythematous mucinosis syndrome

BACKGROUND: Reticular erythematous mucinosis (REM) syndrome is a rare disorder. Its clinical course is cyclic with remissions and exacerbations. In this disease, photosensitivity has previously been noticed but rarely demonstrated. We report three new cases with positive photobiological investigation. CASE REPORTS: Three patients (two males, one female) with a mean age 47 years were seen with reticular erythematous papules on the upper chest and or back. After sun exposure, the lesions were exacerbated. Skin biopsies showed dermal lymphocytic perivascular infiltration with mucin deposition between collagen bundles. Direct immunofluorescence was negative. Antinuclear antibodies were absent. In cabin, ultraviolet (UV)A exposure reproduced clinically and histologically REM lesions in our cases. UVA and UVB provocating phototests were negative. In all patients treatment with oral antimalarials and external photoprotection was effective. CONCLUSIONS: In our patients, we confirm the photosensitive feature of REM syndrome by provocative irradiation in UVA cabin. The mechanism of triggering is actually unclear. It is supposed that UV radiation, heat, and perspiration are necessary to reveal this affection.     

Reticular erythematous mucinosis (REM)

All patients with reticular erythematous mucinosis (REM) published until August 1987 are analysed. REM and plaque-like form of cutaneous mucinosis are different diseases but belong to one spectrum. REM should not be called a syndrome, because a syndrome consists of several signs mostly in various organs. REM and plaque-like form of cutaneous mucinosis are classified as mid-line mucinosis, as suggested by Steigleder and Küchmeister (8). Increasing numbers of mid-line mucinoses are published over the years, apparently midline-mucinosis are not so rare as thought before.     

Reticular erythematous mucinosis syndrome. Report of with positive immunofluorescence

We report a case with the clinical and histological features of the reticular erythematous mucinosis syndrome (REM), in which there was moderate, continuous, fine, granular, IgM deposition along the basal layer. Similar direct immunofluorescence results have been reported in only two previous cases.     

RETICULAR ERYTHEMATOUS MUCINOSIS (REM SYNDROME) OF STEIGLEDER: ITS RELATIONSHIP TO OTHER MUCINOSES AND TO CHRONIC ERYTHEMATA*

The reticular erythematous mucinosis syndrome of Steigleder is a macular erythema-tous form of inflammatory mucinosis. Clinically and histopathologically it has many similarities with the plaque-like mucinosis of Perry. Transitional forms between the two conditions exist. The histopathological picture of the syndromes is not diagnostic and is indistringuishable from that of the lymphocytic infiltration of the skin described by Jessner and Kanof and from some of the chronic inflammatory dermatoses. The clinical picture in combination with the histopathological changes is, however, sufficiently characteristic to define the mucinosis of Steigleder and Perry as a clinical entity.     

Reticular erythematous mucinosis--a review

Reticular erythematous mucinosis (REM) is a rare cutaneous condition often referred to as plaque-like mucinosis and midline mucinosis. Although the exact etiology remains undefined, efforts to elucidate pathogenesis, disease associations, and prospective treatment modalities have been encouraging. Induction of the disease has been associated with viral processes, solar irradiation, specific cell lines, and cytokines such as Interleukin (IL)-1β. Clinically, patients typically develop erythematous macules and papules that coalesce into reticulated patterns on the midline of the chest or back. The lesions have a tendency to respond to systemic antimalarial therapy, but novel therapeutic approaches with ultraviolet A1 light (UVA1) and pulse dye laser (PDL) have been promising. Histologically, REM is associated with a mild, predominantly lymphocytic infiltrate with variable deep perivascular extension. Mucin may be seen in the upper and mid dermis and is prominent around the infiltrate and appendages. IgM deposits may be visualized under direct immunoflourescence along the basal layer. Because of the similarities between REM and tumid lupus, the two disease processes have often been grouped together. The remarkable overlap between the two diseases suggests that the two conditions may actually be the same disease.     

Plaque-like cutaneous mucinosis: case report and literature review

Midline mucinosis was observed in a 14-year-old man, which was confined to the midline of the back and appeared as asymptomatic, nonindurated, hyperpigmented plaques. Skin biopsies showed prominent interstitial mucinosis with perivascular lymphocytic infiltration. A literature review of plaque-like mucinosis revealed 14 previous cases with this distinct presentation that may overlap with reticular erythematous mucinosis and connective tissue disease. Midline mucinosis has been previously reported in prepubertal children but is rare.     

Cutaneous mucinosis

Clinically, the skin changes observed in a 34-year-old woman were suggestive of the reticular erythematosis mucinosis (REM) syndrome. On palpation, however, the changes suggested the plaque-type REM syndrome. Histologically, broad, well-delineated cell-rich bundles were found with abundant alcian-blue-positive material, together with a lymphocytic infiltrate. In such areas showing close contact with the lymphocytic infiltrate, stellate cells with delicate blue processes and protoplasmic granula predominated. Areas preferentially affected with deposits of mucopolysaccharides in the REM syndrome and its variants were not affected in the case presented. The histogenesis of this hitherto undescribed cutaneous mucinosis is discussed.     

Netzförmig angeordnete erythematöse Plaques

Reticular erythematous mucinosis (REM) syndrome primarily affects young women; the skin lesions usually appear on the chest and upper back. REM is diagnosed on the basis of the clinical picture and histological findings. REM syndrome is often associated with lupus erythematosus tumidus. Both diseases respond well to treatment with chloroquin. Topical tacrolimus and the use of a pulsed dye laser have fewer side effects and have also proved to be effective.     

Morphea-like cutaneous mucinosis

A whitish, indurated, oval plaque, lasting for 1.5 year, was observed in the right paraumbilical skin of a middle-aged man in otherwise good health. No erythema was present or referred. Histological examination revealed massive deposits of a globular, slightly basophilic, amorphous substance in the dermis, accompanied by a slight perivascular mononuclear cell infiltrate and scattered mast cells. Special staining with Alcian blue at pH 2.5 confirmed the intense deposition of mucin involving the entire dermis. sparing epidermis, adnexa and perivascular areas. No abnormal laboratory results indicating lupus erythematosus or thyroid dysfunction were present. Morphea was excluded on clinical and histological grounds. A plaque-like form of cutaneous mucinosis was diagnosed. Plaque-like mucinosis and reticular erythematous mucinosis (REM) syndrome are currently considered to be the same disease, although some authors noted a different response lo treatment. Clinical presentation of our case was unusual: no erythema preceded or accompanied the plaque growth, which had a whitish, indurated, morphea-like appearance from the beginning but with preservation of hair follicles, quite different from archetypical REM syndrome based on clinical grounds.     

Mucinosis eritematosa reticular

—Reticular erythematous mucinosis (REM) is a rare entity characterized by infiltrated erythematous plaques on exposed areas of the thorax and back. It has been related to sun exposure, hormonal alterations, rheumatological processes and breast cancer.A 28-year-old woman presented with an infiltrated erythematous plaque on the left breast, which had been developing for 4 years, and which had spread to both breasts over time. The histopathological study showed a perivascular and periadnexal lymphocytic infiltrate, which was accompanied by evident mucin deposits in the upper and mid-dermis. Treatment with colchicine and hydroxychloroquine was administered, with partial regression of the lesions.     

Reticular erythematous mucinosis – (REM syndrome) in twins

We present female twins with reticular erythematous mucinosis (REM syndrome). Remarkably, the lesions developed in both sisters almost at the same time in the same locations after UV exposure. Reports of familial manifestations of REM syndrome are very rare and an association to a distinct HLA constellation has not been proven. Our report clearly suggests a genetic predisposition.     

Reticular erythematous mucinosis syndrome in a patient with polyarthritis

A patient with seronegative oligoarthritis who developed the reticular erythematous mucinosis (REM) syndrome is described. This syndrome is considered to be a dermatological entity unrelated to systemic disorders. Aggravation of the rash by exposure to sunlight and a good response to anti-malarial agents suggest a relationship with rheumatological disorders, e.g. rheumatoid arthritis and systemic lupus erythematosus. Dermatologists consulted by a patient with the REM syndrome should be aware of the possibility of an associated rheumatological disease.     

网状红斑黏蛋白病1例

报告1例网状红斑黏蛋白病。患者男，49岁。头面部、胸背部红斑、丘疹伴瘙痒10年，日晒后皮损无明显改变。组织病理检查示真皮乳头层，网状层上部血管及毛囊周围淋巴细浸润，真皮胶原束之间有阿新蓝染色阳性的黏蛋白沉积。     

Morphological study of the reticular erythematous mucinosis syndrome

Histopathological studies were conducted on skin biopsies of 4 patients with the reticular erythematous mucinosis (REM) syndrome. The diagnosis was made on the basis of the clinical picture in 3 of the cases and from histological characteristics in the 4th. These morphological studies were done parallel with a study of skin biopsies with quite a similar histological picture but in which the clinical picture clearly indicated differing entities. The nosologic place of REM syndrome is questioned and the impression arises that there is a clear-cut histogenetical connection with several inflammatory skin diseases.     

毛囊黏蛋白病

报告1例毛囊黏蛋白病。患者男,33岁。全身泛发红斑伴瘙痒13年余,加重近半年,并出现眉毛脱落。皮损组织病理检查示：真皮毛囊周围炎性细胞浸润。阿新蓝染色阳性,毛囊内可见黏蛋白沉积。诊断为毛囊黏蛋白病。     

Reticular erythematous mucinosis occurring in a brother and sister

Reticular erythematous mucinosis (REM) is a rare, primary cutaneous mucinosis clinically characterized by a persistent reticular erythema on the mid chest and mid-upper back, and histologically by a mononuclear cell infiltrate and deposits of mucin in the dermis. To our knowledge, the present report of REM occurring in a Caucasian man and his sister is the first reported case of familial REM. Since a host-specific immune response to unknown antigens may be involved in the pathogenesis of this entity, human leukocyte antigen typing was determined and compared to those reported in autoimmune diseases.     

Favorable response of reticular erythematous mucinosis to ultraviolet B irradiation using a 308-nm excimer lamp

Reticular erythematous mucinosis (REM) is a rare chronic mucinosis. Histologically, the presence of mucin in the upper dermis is the most specific feature. A 73-year-old woman presented to our outpatient clinic with a 4-year history of netlike macular erythema with slight edema on her left arm. She was diagnosed as having REM on the basis of the clinical picture and histological findings. She was treated with ultraviolet B irradiation using the VTRAC Excimer Lamp system with favorable response. This is the first reported case that was treated with a 308-nm excimer lamp.     

Pimecrolimus 1 percent cream and pulsed dye laser in treatment of a patient with reticular erythematous mucinosis syndrome

We report on the efficacy of twice daily application of pimecrolimus 1 percent cream in a 48-year-old woman with reticular erythematous mucinosis (REM) syndrome and compare its results with pulsed dye laser (PDL) on the other side of her chest and back. The patient was previously treated by hydroxychloroquine but only a fair response was observed. After application of 5 months of pimecrolimus, the lesions completely resolved and the result was comparable with the other side of her body treated by pulsed dye laser PDL. Topical pimecrolimus and pulsed dye laser appear to be effective and safe treatments for REM.     

网状红斑黏蛋白病1例

患者男,26岁。面颈部、躯干、四肢皮疹1年。皮损组织病理示轻度角化过度,表皮水肿,真皮浅至中层毛细血管扩张充血,周围有较多单一核细胞浸润。阿新蓝染色见真皮乳头层及网状层大量黏蛋白样物质沉积。诊断:网状红斑黏蛋白病。