Papular mucinosis
Encyclopedia
Papular mucinosis is a rare skin disease. Localized and disseminated cases are called papular mucinosis or lichen myxedematosus while generalized, confluent papular forms with sclerosis
are called scleromyxedema. Frequently, all three forms are regarded as papular mucinosis. However, some authors restrict it to only mild cases. Another form, acral persistent papular mucinosis is regarded as a separate entity.
/AIDS
virus.
. Instead they contain mucin
, a substance of mucus, the body's natural and protective lubricant found in saliva and epithelial cells in lungs and the sensitive part of the nose. They usually come in clusters such as linear arrays. Less frequently, urticaria
l, nodular, or sometimes annular lesion
s may be appreciated. The dorsal aspect of the hands, face, elbows, and extensor portions of the extremities are most frequently affected. Mucosal lesions are absent. The coalescence of papules on the face, particularly on the glabella
, results in longitudinal folding and gives the appearance of a leonine facies
.
In scleromyxedema, symptoms can occur on larger part of the body. Erythema
and scleroderma
-like induration occurs on the skin. In addition, the mobility of the lips, hands, arms, and legs is reduced. Proximal myopathy
, inflammatory polyarthritis
, central nervous system
symptoms, esophageal
aperistalsis, and hoarseness are among the notable systemic symptoms. If viscera is involved, the disease will be fatal. The dermatoneuro syndrome is a rare neurological complication of the disease presenting with fever, seizures and altered mental status.
and cyclophosphamide
alone or in combination with prednisone
. Both isotretinoin
and etretinate
have also been shown to improve the conditions. All medications listed can cause adverse symptoms, with isotretinoin and etretinate particularly dangerous since they are both teratogens. Other attempted treatments include interferon
-alpha, cyclosporine, PUVA
photochemotherapy, electron-beam therapy, IVIg, and dermabrasion
. However, the overall prognosis
for the disease is poor. There are reported instances of remission of the disease when treated with a combination of Revlimid and Dexamethasone over a 24 month period.
It seems that the use of IVIG treatments is an effective treatment for this condition, with little or no side effects.
Sclerosis (medicine)
In medicine, sclerosis refers to the stiffening of a structure, usually caused by a replacement of the normal organ-specific tissue with connective tissue.Types include:...
are called scleromyxedema. Frequently, all three forms are regarded as papular mucinosis. However, some authors restrict it to only mild cases. Another form, acral persistent papular mucinosis is regarded as a separate entity.
Classification
Papular mucinosis may be divided into several types or variants:- Acral persistent papular mucinosisAcral persistent papular mucinosisAcral persistent papular mucinosis is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides, characterized by bilaterally symmetrical, flesh-colored papules localized to the hands and wrists....
- Self-healing papular mucinosisSelf-healing papular mucinosisSelf-healing papular mucinosis is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides, and may present in adult and juvenile forms. The juvenile variant is also called self-healing juvenile cutaneous mucinosis....
- Papular mucinosis of infancyPapular mucinosis of infancyPapular mucinosis of infancy is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides, characterized by skin-colored or translucent papules....
- Cutaneous mucinosis of infancy
- Nodular lichen myxedematosusNodular lichen myxedematosusNodular lichen myxedematosus is a cutaneous condition characterized by multiple nodules on the limbs and trunk, with a mild or absent papular component....
- Localized lichen myxedematosusLocalized lichen myxedematosusLocalized lichen myxedematosus is a group of skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides, a disease for which there is no treatment....
- Discrete papular lichen myxedematosusDiscrete papular lichen myxedematosusDiscrete papular lichen myxedematosus is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides characterized by the occurrence of waxy, flesh-colored papules....
Incidence
Papular mucinosis affects adults of both sexes equally and appears between ages 30 and 80. Recently, it has been reported in patients infected with the HIVHIV
Human immunodeficiency virus is a lentivirus that causes acquired immunodeficiency syndrome , a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic infections and cancers to thrive...
/AIDS
AIDS
Acquired immune deficiency syndrome or acquired immunodeficiency syndrome is a disease of the human immune system caused by the human immunodeficiency virus...
virus.
Characteristic
Papular mucinosis is chronic and may be progressive. The dermal layer of the skin breaks out into small and solid bumps, usually conical in shape and measured from 2 to 4 mm or sometimes flat-topped papules. Unlike pustules, these bumps do not contain pusPus
Pus is a viscous exudate, typically whitish-yellow, yellow, or yellow-brown, formed at the site of inflammatory during infection. An accumulation of pus in an enclosed tissue space is known as an abscess, whereas a visible collection of pus within or beneath the epidermis is known as a pustule or...
. Instead they contain mucin
Mucin
Mucins are a family of high molecular weight, heavily glycosylated proteins produced by epithelial tissues in most metazoans. Mucins' key characteristic is their ability to form gels; therefore they are a key component in most gel-like secretions, serving functions from lubrication to cell...
, a substance of mucus, the body's natural and protective lubricant found in saliva and epithelial cells in lungs and the sensitive part of the nose. They usually come in clusters such as linear arrays. Less frequently, urticaria
Urticaria
Urticaria is a kind of skin rash notable for pale red, raised, itchy bumps. Hives is frequently caused by allergic reactions; however, there are many non-allergic causes...
l, nodular, or sometimes annular lesion
Lesion
A lesion is any abnormality in the tissue of an organism , usually caused by disease or trauma. Lesion is derived from the Latin word laesio which means injury.- Types :...
s may be appreciated. The dorsal aspect of the hands, face, elbows, and extensor portions of the extremities are most frequently affected. Mucosal lesions are absent. The coalescence of papules on the face, particularly on the glabella
Glabella
The glabella, in humans, is the space between the eyebrows and above the nose. It is slightly elevated, and joins the two superciliary ridges.-Etymology:The term is derived from the Latin glabellus, meaning smooth, as this area is usually hairless....
, results in longitudinal folding and gives the appearance of a leonine facies
Leonine facies
Leonine facies: a face that resembles that of a lion. It is seen in multiple conditions and has been classically described for Lepromatous leprosy as well as Paget's disease of bone...
.
In scleromyxedema, symptoms can occur on larger part of the body. Erythema
Erythema
Erythema is redness of the skin, caused by hyperemia of the capillaries in the lower layers of the skin. It occurs with any skin injury, infection, or inflammation...
and scleroderma
Scleroderma
Systemic sclerosis or systemic scleroderma is a systemic autoimmune disease or systemic connective tissue disease that is a subtype of scleroderma.-Skin symptoms:...
-like induration occurs on the skin. In addition, the mobility of the lips, hands, arms, and legs is reduced. Proximal myopathy
Myopathy
In medicine, a myopathy is a muscular disease in which the muscle fibers do not function for any one of many reasons, resulting in muscular weakness. "Myopathy" simply means muscle disease...
, inflammatory polyarthritis
Polyarthritis
Polyarthritis is any type of arthritis which involves 5 or more joints simultaneously. It is usually associated with autoimmune conditions.Polyarthritis may be experienced at any age and is not gender specific.-Causes:...
, central nervous system
Central nervous system
The central nervous system is the part of the nervous system that integrates the information that it receives from, and coordinates the activity of, all parts of the bodies of bilaterian animals—that is, all multicellular animals except sponges and radially symmetric animals such as jellyfish...
symptoms, esophageal
Esophageal
Esophageal can refer to:* The esophagus* Esophageal arteries* Esophageal glands...
aperistalsis, and hoarseness are among the notable systemic symptoms. If viscera is involved, the disease will be fatal. The dermatoneuro syndrome is a rare neurological complication of the disease presenting with fever, seizures and altered mental status.
Treatment
There is no effective treatment for this condition. It has been reported that clearance of lesions can be done with melphalanMelphalan
Melphalan hydrochloride is a chemotherapy drug belonging to the class of nitrogen mustard alkylating agents.An alkylating agent adds an alkyl group to DNA...
and cyclophosphamide
Cyclophosphamide
Cyclophosphamide , also known as cytophosphane, is a nitrogen mustard alkylating agent, from the oxazophorines group....
alone or in combination with prednisone
Prednisone
Prednisone is a synthetic corticosteroid drug that is particularly effective as an immunosuppressant drug. It is used to treat certain inflammatory diseases and some types of cancer, but has significant adverse effects...
. Both isotretinoin
Isotretinoin
Isotretinoin, INN, is a medication used mostly for cystic acne. It was first developed for brain, pancreatic and other cancers. It is used to treat harlequin-type ichthyosis, a usually lethal skin disease, and lamellar ichthyosis. Its effects are systemic and nonselective...
and etretinate
Etretinate
Etretinate is a medication developed by Hoffmann–La Roche that was approved by the FDA in 1986 to treat severe psoriasis...
have also been shown to improve the conditions. All medications listed can cause adverse symptoms, with isotretinoin and etretinate particularly dangerous since they are both teratogens. Other attempted treatments include interferon
Interferon
Interferons are proteins made and released by host cells in response to the presence of pathogens—such as viruses, bacteria, or parasites—or tumor cells. They allow communication between cells to trigger the protective defenses of the immune system that eradicate pathogens or tumors.IFNs belong to...
-alpha, cyclosporine, PUVA
PUVA
PUVA is a psoralen + UVA treatment for eczema, psoriasis, graft-versus-host disease and vitiligo, and mycosis fungoides. The psoralen is applied or taken orally to sensitize the skin, then the skin is exposed to UVA. Long term use has been associated with higher rates of skin cancer.Psoralens are...
photochemotherapy, electron-beam therapy, IVIg, and dermabrasion
Dermabrasion
Dermabrasion is a surgical procedure that involves the controlled abrasion of the upper layers of the skin with sandpaper or other mechanical means. Nowadays it has become common to use CO2 or Erbium:YAG laser as well. The procedure requires a local anaesthetic...
. However, the overall prognosis
Prognosis
Prognosis is a medical term to describe the likely outcome of an illness.When applied to large statistical populations, prognostic estimates can be very accurate: for example the statement "45% of patients with severe septic shock will die within 28 days" can be made with some confidence, because...
for the disease is poor. There are reported instances of remission of the disease when treated with a combination of Revlimid and Dexamethasone over a 24 month period.
See also
It seems that the use of IVIG treatments is an effective treatment for this condition, with little or no side effects.