Four Cases of the Rare Skin Manifestation: Monoclonal-Proteinemia / 罕见病研究

The skin manifestations of monoclonal(M)-proteinemia are rare and present in patients with monoclonal gammopathy of undetermined significance (MGUS), smoldering plasma cell myeloma (SMM) and multiple myeloma (MM). In this study, we reported 4 cases with M-proteinemia-related rare skin lesions, including pyoderma gangrenosum (PG), erythema elevatum diutinum (EED), cutis laxa (CL) and lichen myxedematosus(LM). These skin lesions are specific, where the potential mechanism was immune-mediated paraneoplastic syndrome rather than direct plasma cell infiltration. Anti-plasma cell treatment was effective in treating skin lesions. The clinical outcome of MM-related skin changes was correlated to tumor control, whereas the prognosis of MGUS or SMM related skin lesions was favorable. Skin involvement in M-proteinemia is extremely rare and less well-known, which greatly impairs quality of life. The diagnosis and treatment of these 4 cases support the need for futher study.

Scleromyxedema with Monoclonal Gammopathy / 대한피부과학회지

Scleromyxedema is a rare disorder characterized by generalized papular and sclerodermoid eruption, increased fibroblast proliferation, mucin deposition, and monoclonal gammopathy in the absence of thyroid disease. It is a generalized subtype of lichen myxedematosus. A paraproteinemia, typically an IgG lambda, is observed in more than 80% of patients with scleromyxedema. Here, we report a 38-year-old woman with a 1-year history of a progressively spreading of eruption of small papules on the entire body, including the face, neck, arms, legs, and trunk. Laboratory tests were within normal limits, except lambda light chain monoclonal gammopathy. We administered oral retinoid and topical steroid with slight clinical improvement. To our knowledge, this is the first reported case of scleromyxedema in Korea.

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 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.

Primary Cutaneous CD30(Ki-1)-Positive Pleomorphic Large Cell Lymphoma in a Patient with Generalized Lichen Myxedematosus

A case of primary cutaneous CD30(Ki-1)-positive pleomorphic large cell lymphoma in a 51-year-old woman with generalized lichen myxedematosus is presented. Histopathological findings of the biopsy specimens from the lichenoid papules of the left forearm and the dorsum of the right hand were compatible with papular mucinosis. The mucinous material in the papillary dermis stained with alcian blue at pH 2.5 and was susceptible to hyaturonidase, but serum protein electrophoresis showed no abnormal bands and an abnormal paraprotein was not detected in our patient. Histopathological examination of a solitary, firm, purplish nodule on the right popliteal area showed diffuse and extensive infiltration in the reticular dermis composed of large, atypical, often hyperchromatic, sometimes multinucleated and markedly pleomorphic cells. The majority of the large atypical cells were CD30(Ki-1) positive. The TCR gene re-arrangement analysis demonstrated the presence of a monoclonal rearrangement of the y-TCR gene in the skin biopsy specimen of our patient. The organ-system survey revealed no evidence of internal organ involvement. We concluded that this was primary cutaneous CD30(Ki-1)-positive pleomorphic large cell lymphoma developing in a patient with generalized lichen myxedematosus.