Acral persistent papular mucinosis: A case report and summary of 24 Japanese cases.

Acral persistent papular mucinosis is a rare subtype of localized lichen myxedematosus. We report a case of a 41-year-old woman with acral persistent papular mucinosis. She had a 5-year history of multiple, scattered, whitish papules, on the back of both hands, of 2-5 mm in diameter. Histopathological examination revealed a focal, fairly circumscribed area with sparse collagen fibers in the upper and middle dermis. The circumscribed area was positively stained with Alcian blue. The papules were clinically and histologically diagnosed as acral persistent papular mucinosis. We also summarized 24 Japanese acral persistent papular mucinosis cases, including ours. Although acral persistent papular mucinosis occurs predominantly in women according to overseas reports, the male : female ratio is 5:7 in Japan. It has been reported that tranilast (N-[3,4-dimethoxycinnamoyl]-anthranilic acid) was effective in two Japanese cases. In our case, the patient had been treated with tranilast for 2 months without improvement. Further studies are required to confirm the efficacy of tranilast for acral persistent papular mucinosis.

[Reticular erythematous mucinosis-A special subtype of cutaneous lupus erythematosus?] / Retikuläre erythematöse Muzinose ­ Sonderform eines kutanen Lupus erythematodes?

Reticular erythematous mucinosis (REM syndrome) is a rare skin disease that predominantly affects women. It is clinically characterized by flat, partly reticular, irregularly configurated pale erythema located in the region of the central chest and upper back. The skin alterations are usually asymptomatic or associated with slight pruritus or burning. Extracutaneous diseases and involvement of internal organs do not occur in REM syndrome. Histopathological features include perivascular and periadnexal lymphocytic inflammatory infiltrates as well as increased deposition of mucin in the dermis. Due to the similar histology to lupus erythematosus (LE) tumidus, it is discussed whether REM syndrome can be assigned to the spectrum of cutaneous LE or can be assessed as a lupus-like disease. Moreover, both conditions respond well to treatment with hydroxychloroquine.

Atypical Cutaneous Mucinosis Treated with Intravenous Immunoglobulin and Corticosteroid.

FA 25-year-old man presented with a 12-month history of progressive skin lesions involving the face, upper areas of the trunk, arms, and natal cleft. There was moderate pruritus, disfiguring lesions of the face, and mild arthralgia. No therapy had been applied before the referral to our clinic. On physical examination, there were skin-colored nodules on the face (Figure 1A), along with multiple firm subcutaneous nodules and tumors with a fairly symmetrical distribution on the extensor surface of the hands and forearms (Figure 2A). Dense papular lesions were found on the upper part of the back (Figure 3A). There was also a waxy infiltrated plaque in the natal cleft area (Figure 4A).

Primary cutaneous dermal mucinosis on herpes zoster scars.

The term isotopic response refers to the appearance of a new skin disease at the site of another unrelated and already healed skin disorder. Often, the first disease is herpes zoster (HZ). Several cutaneous reactions have been described in a dermatome recently affected by HZ. We present the case of a 33-year-old man who developed whitish papules with a zosteriform distribution on HZ scars. Histopathologic study with hematoxylin and eosin and Alcian blue (pH 2.5) staining demonstrated abundant deposits of mucin interstitially arranged between collagen bundles of the papillary dermis. Cutaneous dermal mucinosis as a postherpetic isotopic response is rare, but it should be added to the list of cutaneous reactions arising in HZ scars.

Adult Variant of Self-healing Cutaneous Mucinosis in a Patient with Epilepsy.

A 52-year-old woman was admitted with a 3 weeks history of periorbital edema and lips swelling. She developed several subcutaneous firm erythematous papules and nodules on the face, scalp and two indurated plaques on the upper back and left forearm. These lesions grew rapidly. The patient had a positive history of epileptic seizures since childhood. General examination was normal. There was a mild pitting edema on her hands and feet. Laboratory data were within normal limits. Histopathological examination revealed a well circumscribed accumulation of mucin in the dermis. Alcian blue stain was positive. Clinical and histopathological findings followed by spontaneous resolution of the lesions within a period of 4 months was compatible with diagnosis of self-healing cutaneous mucinosis. Herein we report the first case of self-healing cutaneous mucinosis associated with epilepsy.

Papulonodular mucinosis in a suspected systemic lupus erythematosus patient.

We present a 45-years-old suspected systemic lupus erythematosus (SLE) woman who had papulonodular mucinosis (PNM), without other cutaneous LE lesion. The lesions of PNM distributed on both legs which were an unusual location. In addition, the renal involvement was found and suspected from lupus nephritis. The patient was treated with prednisolone, mycofenolate mofetil and chloroquine. After 2 months of follow-up, the renal involvement was improved along with the disappearance ofskin lesions.

Reticular erythematous mucinosis on the midline of the back.

We report the case of a 51-year-old man who presented with reticular macular erythema and erythematous plaques on the midline of the back of 10 years' duration that seemed to worsen on exposure to sunlight. Based on the clinical and histopathologic findings, a diagnosis of reticular erythematous mucinosis (REM) was made. The patient was treated with hydroxychloroquine 400 mg daily and the lesions resolved by the end of the second week of therapy.

Papulonodular mucinosis in a patient with systemic lupus erythematosus and antiphospholipid syndrome.

The skin is one of the target organs most commonly affected in lupus erythematosus (LE) and a wide range of cutaneous changes have been described in LE patients. Papulonodular mucinosis (PNM) in particular is an uncommon cutaneous manifestation of LE. We discuss the case of a 26-year-old Senegalese woman with systemic LE and antiphospholipid syndrome (APS) who presented with pruritic papules on her back and extremities that appeared when she was on vacation in Africa and non-compliant with medications. Histopathologic examination was consistent with PNM. The patient was treated with mycophenolate mofetil and hydroxychloroquine, with subjective relief in pruritis at 6-week follow-up. To our knowledge, this is the first case of PNM presenting in a patient with both SLE and APS. Whether APS contributes to the pathogenesis of PNM is currently unknown.

Reticular erythematous mucinosis: a review of patients' characteristics, associated conditions, therapy and outcome in 25 cases.

BACKGROUND: Reticular erythematous mucinosis (REM) is an uncommon disease, the nosology and specific characteristics of which are controversial because most reports deal with single cases or small series. OBJECTIVES: To describe the characteristics of patients with REM regarding demographics, clinical and pathological features, comorbidities, treatment and course. METHODS: A retrospective and prospective study was conducted on 25 patients diagnosed with REM in the setting of university-affiliated dermatology departments and dermatopathology centres. RESULTS: Of the 25 patients with REM, 16 were women (sex ratio 2 : 1) and the mean age was 46 years. The roles of sun exposure and oral contraceptives were ambiguous. Associated diseases included hypertension (n = 4), malignancies (n = 3), autoimmune diseases (n = 3) and Borrelia infection (n = 1). Immunological studies (including serology and direct immunofluorescence) were noncontributory. The response to antimalarial treatment was good in > 80% of cases. Worsening or recurrence of the lesion after treatment discontinuation, or in the course of the disease, occurred in 31% of patients. CONCLUSIONS: We present the largest REM case series to date. The reticular pattern with involvement of the midline of the chest and back, the predilection for middle-aged women, the controversial relationship with photosensitivity and the possible association with other conditions such as malignancies and thyroid dysfunctions are the main characteristics that makes REM a recognizable disease.

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)-1ß. 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.