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Alopecia mucinosa (also known as "Follicular mucinosis," "Mucinosis follicularis,", "Pinkus’ follicular mucinosis," and "Pinkus’ follicular mucinosis–benign primary form") is a skin disorder that generally presents, but not exclusively, as erythematous plaques or flat patches without hair primarily on the scalp and face. This can also present on the body as a follicular mucinosis and may represent a systemic disease.
Cutaneous focal mucinosis is a skin condition characterized by a solitary nodule or papule.
Urticaria-like follicular mucinosis is a rare cutaneous disorder that occurs primarily in middle-aged men.
The main diagnosis technique is observing the area. Then blood tests can be done to determine if there is a pre-existing condition. Family history can be considered because some of the related causes/conditions can be inherited.
Papular mucinosis of infancy (also known as "Cutaneous mucinosis of infancy") is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides, characterized by skin-colored or translucent papules.
Acral 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.
There is no effective treatment for this condition. It has been reported that clearance of lesions can be done with melphalan 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.
Perifollicular mucinosis is a cutaneous condition characterized by mucinosis, and described in HIV-infected patients.
Eccrine mucinosis is a cutaneous condition characterized by mucinosis, and described in HIV-infected patients.
There is currently researching being done to find more treatments dependent on the different pre-existing conditions.
Studies are being conducted in which madarosis can be related to malignancy. A study by Groehler and Rose found that there was a statistical significance between these two. They concluded that patients malignancy lesions on the eyelid have a higher chance of having madarosis than a patient with a benign lesion. They stated that despite the fact that it is significant, the absence of madarosis does not mean the lesion cannot be malignant.
In many leprosy cases, madarosis is a symptom or a quality after diagnosis. However, in India, leprosy is common and researchers report a case of madarosis before diagnosis of leprosy with no skin lesions, only madarosis. This allowed for quicker treatment.
A main reason many people have madarosis is due to the chemotherapy drugs. There was a clinical trial in 2011 that tested an eyelash gel called bimatoprost. This gel enhanced the eyelashes in quantity and thickness. They tested this on 20 breast cancer patients who were undergoing chemotherapy. Results seemed positive, in that the group of people who used the gel had growth of eyelashes after the chemotherapy drugs.
Self-healing juvenile cutaneous mucinosis is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides, and is characterized by the sudden onset of skin lesions and polyarthritis.
Many conditions affect the human integumentary system—the organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. The major function of this system is as a barrier against the external environment. The skin weighs an average of four kilograms, covers an area of two square meters, and is made of three distinct layers: the epidermis, dermis, and subcutaneous tissue. The two main types of human skin are: glabrous skin, the hairless skin on the palms and soles (also referred to as the "palmoplantar" surfaces), and hair-bearing skin. Within the latter type, the hairs occur in structures called pilosebaceous units, each with hair follicle, sebaceous gland, and associated arrector pili muscle. In the embryo, the epidermis, hair, and glands form from the ectoderm, which is chemically influenced by the underlying mesoderm that forms the dermis and subcutaneous tissues.
The epidermis is the most superficial layer of skin, a squamous epithelium with several strata: the stratum corneum, stratum lucidum, stratum granulosum, stratum spinosum, and stratum basale. Nourishment is provided to these layers by diffusion from the dermis, since the epidermis is without direct blood supply. The epidermis contains four cell types: keratinocytes, melanocytes, Langerhans cells, and Merkel cells. Of these, keratinocytes are the major component, constituting roughly 95 percent of the epidermis. This stratified squamous epithelium is maintained by cell division within the stratum basale, in which differentiating cells slowly displace outwards through the stratum spinosum to the stratum corneum, where cells are continually shed from the surface. In normal skin, the rate of production equals the rate of loss; about two weeks are needed for a cell to migrate from the basal cell layer to the top of the granular cell layer, and an additional two weeks to cross the stratum corneum.
The dermis is the layer of skin between the epidermis and subcutaneous tissue, and comprises two sections, the papillary dermis and the reticular dermis. The superficial papillary dermis with the overlying rete ridges of the epidermis, between which the two layers interact through the basement membrane zone. Structural components of the dermis are collagen, elastic fibers, and ground substance. Within these components are the pilosebaceous units, arrector pili muscles, and the eccrine and apocrine glands. The dermis contains two vascular networks that run parallel to the skin surface—one superficial and one deep plexus—which are connected by vertical communicating vessels. The function of blood vessels within the dermis is fourfold: to supply nutrition, to regulate temperature, to modulate inflammation, and to participate in wound healing.
The subcutaneous tissue is a layer of fat between the dermis and underlying fascia. This tissue may be further divided into two components, the actual fatty layer, or panniculus adiposus, and a deeper vestigial layer of muscle, the panniculus carnosus. The main cellular component of this tissue is the adipocyte, or fat cell. The structure of this tissue is composed of septal (i.e. linear strands) and lobular compartments, which differ in microscopic appearance. Functionally, the subcutaneous fat insulates the body, absorbs trauma, and serves as a reserve energy source.
Conditions of the human integumentary system constitute a broad spectrum of diseases, also known as dermatoses, as well as many nonpathologic states (like, in certain circumstances, melanonychia and racquet nails). While only a small number of skin diseases account for most visits to the physician, thousands of skin conditions have been described. Classification of these conditions often presents many nosological challenges, since underlying etiologies and pathogenetics are often not known. Therefore, most current textbooks present a classification based on location (for example, conditions of the mucous membrane), morphology (chronic blistering conditions), etiology (skin conditions resulting from physical factors), and so on. Clinically, the diagnosis of any particular skin condition is made by gathering pertinent information regarding the presenting skin lesion(s), including the location (such as arms, head, legs), symptoms (pruritus, pain), duration (acute or chronic), arrangement (solitary, generalized, annular, linear), morphology (macules, papules, vesicles), and color (red, blue, brown, black, white, yellow). Diagnosis of many conditions often also requires a skin biopsy which yields histologic information that can be correlated with the clinical presentation and any laboratory data.
Inverted follicular keratosis is a skin condition characterized by asymptomatic, firm, white–tan to pink papules and considered as a subtype of seborrheic keratosis.
Microscopically, inverted follicular keratosis is characterized as a well-circumscribed inverted acanthotic squamous proliferation containing squamous eddies and without significant atypia.
The differential diagnoses are: acrodermatitis enteropathica, erythema infectiosum, erythema multiforme, hand-foot-and-mouth disease, Henoch–Schönlein purpura, Kawasaki disease, lichen planus, papular urticaria, papular purpuric gloves and socks syndrome, and scabies.
Atrophodermia vermiculata presents with erythematous follicular papules on the cheeks in childhood and, with time, the lesions develop into pit-like depressions.
The diagnosis of Gianotti–Crosti syndrome is clinical. A validated diagnostic criteria is as follows:
A patient is diagnosed as having Gianotti–Crosti syndrome if:
1. On at least one occasion or clinical encounter, he/she exhibits all the positive clinical features,
2. On all occasions or clinical encounters related to the rash, he/she does not exhibit any of the negative clinical features,
3. None of the differential diagnoses is considered to be more likely than Gianotti–Crosti syndrome on clinical judgment, and
4. If lesional biopsy is performed, the histopathological findings are consistent with Gianotti–Crosti syndrome.
The positive clinical features are:
- Monomorphous, flat-topped, pink-brown papules or papulovesicles 1-10mm in diameter.
- At least three of the following four sites involved – (1) cheeks, (2) buttocks, (3) extensor surfaces of forearms, and (4) extensor surfaces of legs.
- Being symmetrical, and
- Lasting for at least ten days.
The negative clinical features are:
- Extensive truncal lesions, and
- Scaly lesions.
Atrophodermia vermiculata is also known as "Acne vermoulante", "Acne vermoulanti", "Atrophoderma reticulata symmetrica faciei", "Atrophoderma reticulatum", "Atrophoderma vermiculata", "Atrophoderma vermiculatum", "Atrophodermia reticulata symmetrica faciei", "Atrophodermia ulerythematosa", "Atrophodermie vermiculée des joues avec kératoses folliculaires", "Folliculitis ulerythema reticulata", "Folliculitis ulerythematous reticulata", "Folliculitis ulerythemosa", "Honeycomb atrophy", "Ulerythema acneforme" and "Ulerythema acneiforme"
Papular mucinosis (also known as "Scleromyxedema," "Generalized lichen myxedematosus," and "Sclerodermoid lichen myxedematosus") 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.
Rombo syndrome is a very rare genetic disorder characterized mainly by atrophoderma vermiculatum of the face, multiple milia, telangiectases, acral erythema, peripheral vasodilation with cyanosis and a propensity to develop basal cell carcinomas.
The lesions become visible in late childhood, began at ages 7 to 10 years and are most pronounced on the face, At that time a pronounced, somewhat cyanotic redness of the lips and hands was evident as well as moderate follicular atrophy of the skin on the cheeks. In adulthood, whitish-yellow, milia-like papules and telangiectatic vessels developed. The papules were present particularly on the cheeks and forehead, gradually becoming very conspicuous and dominating the clinical picture. Trichoepitheliomas were found in 1 case. In adults, the eyelashes and eyebrows were either missing or irregularly distributed with defective and maldirected growth. Basal cell carcinomas were a frequent complication. The skin atrophy was referred to as vermiculate atrophoderma. Basal cell carcinomas may develop around the age of 35. Histological observations during the early stage include irregularly distributed and atrophic hair follicles, milia, dilated dermal vessels, lack of elastin or elastin in clumps. After light irradiation a tendency to increased repair activity was observed both in epidermis and in the dermal fibroblasts.
Histologic sections showed the dermis to be almost devoid of elastin in most areas with clumping of elastic material in other areas. The disorder had been transmitted through at least 4 generations with instances of male-to-male transmission.
Reticular erythematous mucinosis (REM) — also known as Midline mucinosis, Plaque-like cutaneous mucinosis, and REM syndrome — is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides. It is a disease that tends to affect women in the third and fourth decades of life.
Follicular atrophoderma is a skin condition consisting of follicular indentations without hairs, notably occurring on extensor surfaces of the hands, legs and arms.
Proliferating trichilemmal cysts (also known as a "Pilar tumor", "Proliferating follicular cystic neoplasm", "Proliferating pilar tumor", and "Proliferating trichilemmal tumor") are a cutaneous condition characterized by proliferations of squamous cells forming scroll-like structures.
Treatments for CCCA remain investigational. Altering hair care practices has not been proven to assist in hair rejuvenation. High-dose topical steroids, antibiotics, immunomodulators such as tacrolimus (Protopic) and pimecrolimus (Elidel), and anti-androgen/5alpha Reductase inhibitors have been used with unknown efficacy.
Self-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.