Generalized papular and sclerodermoid eruption: Scleromyxedema.

Scleromyxedema (SM) is a rare chronic progressive and highly intractable cutaneous disease with unknown etiology, affecting both genders equally between 30 and 50 years. The disease is characterized with mucin deposits in the skin and/or other organs. In fact it is a clinicopathological subset of lichen myxedematosus (LM) according to a new classification. Sclerodermiform plaques and lichenoid papules are characteristic cutaneous lesions. An elevation of IgG λ (lambda) chain exists in most cases and extracutaneous involvement occurring with variable systemic findings is also detected. Generalized form is quite difficult to treat and may even be fatal. Herein, we present a male patient with typical features of generalized papular and sclerodermoid LM variety and with benign outcome by isotretinoin.

A Case of Acral Persistent Papular Mucinosis in Patient with HIV Infection / 대한피부과학회지

Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichen myxedematosus, which involves only the extensor surfaces of the hands, wrists and the distal part of forearms. Some subtypes of lichen myxedematosus including APPM have been reported with increased frequency in a patient with human immunodeficiency virus (HIV) infection. We describe a 31 year-old male patient with an HIV infection who presented with discrete, flesh-colored papules on the extensor surfaces of the wrists and the distal part of the forearms. Histopathologic findings revealed focal deposition of mucin in papillary and mid dermis that spared a small grenz zone. The lesions showed spontaneous regression after starting highly active antiretroviral therapy (HAART). We report a rare case of APPM in a patient with HIV infection which showed spontaneous regression with HAART, suggesting that APPM may represent one of the cutaneous markers of HIV infection.

A Case of Discrete Lichen Myxedematosus / 대한피부과학회지

Lichen myxedematosus (synonym, papular mucinosis) is a disorder characterized by lichenoid papules, nodules and plaques due to dermal mucin deposition, and a variable degree of fibrosis without thyroid dysfunction. Discrete lichen myxedematosus is a subtype of the localized lichen myxedematosus. Clinically, firm, smooth, waxy or flesh-colored papules measuring 2 to 5 mm in diameter, numbering just a few to hundreds, and involving limbs and trunk. The lesions progress slowly without systemic symptoms and rarely resolve spontaneously. Histologically, the upper and mid dermis shows edema and diffuse or focal mucinous deposit under normal epidermis. Fibroblast proliferation is variable, but there is neither collagen deposition nor sclerosis. On experiencing a case of discrete grouped papules on the arm and thigh of a sixty three year-old women, we present it as discrete lichen myxedematosus.

A Case of Acral Persistent Papular Mucinosis

Acral persistent papular mucinosis has been thought to be a distinct for of cutaneous mucinosis not associated with systemic diseases. It was recently classified as one of five subtypes of localized lichen mixedematosus. A 64-year-old man presented with a 15-year history of flesh-colored to translucent papules and a few round hypopigmented patches on his wrists, back of the hands, and distal forearms. Biopsy from the papular lesions revealed focal mucin accumulation in the upper reticular dermis. The clinical and histopathological features were consistent with acral persistent papular mucinosis.

A Case of Lichen Myxedematosus in a Patient with HIV Infection / 대한피부과학회지

Lichen myxedematosus(LM), also known as papular mucinosis, is a rare, idiopathic disorder caused by the focal dermal deposition of mucin and occurs both as an isolated entity and manifestations of various systemic disorders. Cutaneous disease is common in patients infected with human immunodeficiency virus(HIV) and cutaneous mucinosis has been reported with increased frequency in HIV patients. A male patient with HIV infection was consulted to our clinic for evaluation of asymptomatic diffusely-scattered papules on his upper extremities and trunk. Histopathological finding was the deposition of mucin in the dermis and lichen myxedematosus type 2 was diagnosed. We present of a case of lichen myxedematosus in a HIV-infected male patient.

A Case of Discrete Papular Mucinosis / 대한피부과학회지

Papular mucinosis, or lichen myxedematosus is one of the rare cutaneous diseases characterized by papular-lichenoid eruptions, in which mucin deposits in the dermis without thyroid dysfunction. A 31-year-old male presented with 3-5mm sized waxy, flesh-colored, multiple firm papules on the upper and lower extremities and trunk for 5 months. He did not have any familial medical history. None of the laboratory findings, including serum protein electrophoresis, was significant. Skin biopsies were performed from papules of his trunk and arm. Histopathological findings showed that the collagen fibers in the upper dermis were loosely arranged and separated by amorphous materials. These materials were all positive for alcian blue, colloidal iron, mucicarmine, and Giemsa staining. This case is compatible with discrete papular mucinosis which is the rare subtype of localized papular mucinosis.

A Case of Acral Persistent Papular Mucinosis

Acral persistent papular mucinosis is a subtype of the localized papular mucinosis. Clinically, ivory to flesh-colored papules develop exclusively on the back of the hands, the extensor surface of the wrists, and occasionally the distal forearms. Histologically, mucin accumulates in the upper reticular dermis, typically sparing a subepidermal zone and fibroblasts are not increased in number. On experiencing a case of numerous papules on both hands and forearms of a fifty year-old woman, we present it as acral persistent papular mucinosis.

Two Cases of Acral Persistent Papular Mucinosis / 대한피부과학회지

We describe two cases of acral persistent papular mucinosis. The characteristic findings include discrete papules on the extensor surfaces of the hands and wrists, mucous deposits within the dermis, persistence or slow progression for several years. APPM may be considered as the disease entity in the same spectrum with lichen myxedematosus.

Two Cases of Papular Mucinosis / 대한피부과학회지

Scleromyxedema is a rare fibromucinous disorder that is often difficult to treat and is associated with significant morbidity and mortality. Clinically, it shows white or flesh colored, dome-shaped waxy papules on the hands, arms, face, neck and upper trunk. There are proliferation of fibroblasts and mucin deposition in dermis and presence of serum paraproteins. It should be differentiated from scleroderma because of sclerotic skin changes. Patients were a 70 year old male and a 45 year old female. They showed multiple confluent or linearly arranged waxy papules on the head, neck and extremities. Sclerotic plaques were also seen. Abnormal findings were not detected in laboratory tests and bone marrow biopsy. Histologically, mucin deposition and marked proliferation of fibroblasts were seen in dermis. Therapy was performed with 200 mg/day of cyclophosphamide. Both patients showed good response and had no recurrence. The latter one had shown improvement of skin lesions but subsequently she developed leukocytopenia. Cyclophosphamide was stopped temporarily until the abnormal leukocytes count recovered. During therapy, mild hemiparesis developed. We could not find the cause of the symptom through physical examination and radiologic studies.

A Case of Papular Mucinosis / 대한피부과학회지

Papular mucinosis, or lichen myxedematosus, is a rare cutaneous disease characterized by papular-lichenoid eruptions, mucin deposition, paraproteinemia, no abnormalities of thyroid function and IgG or IgM deposition in the reticular dermis or just below the epidermis in about 35% cases on direct immunofluorescence study. We report a case of papular mucinosis in a 48-year-old male, who noticed the skin colored or yellowish waxy papules on his upper arm 1 year ago. Since then, similar lesions developed on his upper extremities, upper trunk, and neck during last year. Histopathological findings of the lichenoid papules showed that the collagen fibers in the upper dermis are loosly arranged and separated by amorphous materials. These amorphous materials were positve with colloidal iron stain. Serum protein electrophoresis showed no abnormal bands and an abnormal paraprotein was not detected. Direct immunofluorescenc study of a skin biopsy specimen showed a granular IgM deposit in the papillary dermis. This is the sixth case of papular mucinosis reported in Korea, but is the first case showing IgM deposit in the skin by immunofluorescence study.

A Case of Papular Mucinosis / 대한피부과학회지

Papular mucinosis, also known as lichen myxedematosus or scleromyxedema, is a chronic cutaneous disorder characterizecl by infiltration of mucin in the dermis without abnormalities of thyroid function. It often combines serum monoclonal paraproteins. Various drugs have been used for the treatment without consistent results. We report a case of papular mucinosis in a 51-year-old man, who started the disease on his scalp 10 years before the first examination. Subsequent development of the characteristic glistening papular lesions on his entire scalp, eye brows, ears and upper back continued for 5 years thereafter until complete resolution. Treatment with corticosteroids and melphalan showed only limited effects during the period.