Brenner, Sarah, and Yael Politi. "Dermatologic Diseases and Problems of Women Throughout the Life Cycle," International Journal of Dermatology 34, no. 6 (June 1995): 369-379.
The last century has brought dramatic changes in the lives of Western women. To the normal physiologic developments that take place in menarche, pregnancy, and menopause are added the stresses of combined family life and career. These in turn are exacerbated by longevity and the cultural norms against aging, which have given rise to esthetic interventions that offer options as well as pose new threats. This review surveys dermatologic diseases of women throughout the life cycle, from infancy and childhood through the childbearing years to menopause and old age. The size of the subject precludes complete coverage of all diseases and problems; rather, topics were selected in an attempt to cover a variety of dermatologic issues in women's lives.
INFANCY TO ADOLESCENCE
A small number of genodermatoses are highly predominant in or exclusive to the female gender. They usually present at birth or soon after.
X-Linked Dominant Diseases. Incontinentia pigmenti is transmitted as an X-linked dominant trait and is believed to be lethal in boys. It is theorized that the few reported male patients represent the results of a spontaneous mutation,1,2 a theory that is supported in part by similar severity of the reported cases.3 The disease presents at birth or during the first weeks of life and has three stages: vesicular, verrucous, and hyperpigmentation that eventually fade completely in most cases. The majority of cases are affected with other congenital abnormalities, including central nervous system, skeletal and eye and teeth abnormalities, and patchy alopecia of the vertex.
Goltz syndrome (focal dermal hypoplasia), a very rare syndrome that predominates in girls, is characterized by various ectodermal and mesodermal abnormalities. An X-linked dominant gene is probably responsible for the disease4 that is lethal to homozygous boys. A new mutation may explain the few reported cases in boys and chromosomal mosaicism has been proposed as well.5
CHILD syndrome (congenital hemidysplasia with ichthyosiform erythroderma and limb defects) is a very rare syndrome. Because it is reported almost exclusively in girls, its X-linked dominant inheritance is suggested to be lethal to boys.6
Oral facial-digital syndrome type 1 (OFD-1) is inherited in an X-linked dominant pattern. Because it affects only girls, it is probably lethal to boys.7 It is characterized by abnormal development of the oral cavity, digital, skeletal, and neurological abnormalities, and diffuse alopecia.
Rothmund-Thompson syndrome (congenital poikiloderma) is considered to predominate in girls, but the prevalence is unclear due to the small number of reported cases. The disease is inherited in an autosomal recessive pattern, although autosomal-dominant inheritance, probably due to a mutant gene, was suggested in some cases.8,9
Lichen Sclerosus et Atrophicus (LSA) in Childhood
Although most common in postmenopausal women, this disease appears in children, most frequently in girls.10 Manifestations are similar to those seen in adult women11 but with a better prognosis.10 About two-thirds of cases improve or undergo involution before or around the menarche.10, 12
Certain manifestations of LSA, such as hemorrhage, purpura, and labial adhesions, may raise the suspicion of child abuse and should be carefully checked. Topical testosterone is contraindicated, in contrast to adults, and treatment is symptomatic.
Cosmetic-Acne Cosmetica and Contact Dermatitis
Even before adolescence, preteenage girls are exposed to the use of cosmetics and beauty products. But the dermatologic problems that may occur when the growing girl tries on her mother's or older sister's cosmetics are apt to be overlooked, because acne cosmetica and contact dermatitis to beauty products are usually associated with adult women. The everyday occurrence of acne devoid of a cosmetic link in adolescents of both genders contributes further to the tendency to overlook this connection in young girls.
Trichotillomania is a neurosis manifested as irregular patches of hair loss resulting from a neurotic urge to pluck or manipulate hair, usually on the scalp, eyelashes, eyebrows, and beard. It is generally limited, but may be diffuse,11 and trichotillomania is most common in children and adolescents (Fig. 1), especially girls.13 The affected area is irregular, with twisted and broken hairs of different lengths on normal appearing skin. Diagnosis is aided by other signs and symptoms of emotional disturbance, such as nail-biting and thumb-sucking. The main histologic features are normal hairs scattered among empty hair follicles and signs of damage such as trichomalacia without significant inflammation.16
Trichotillomania differs from alopecia areata by the irregular borders and varying lengths of the broken hairs, a different microscopic appearance, lack of nail pitting, and the presence of psychologic disturbance in the former. The differential diagnosis can be confirmed by biopsy, preferably at the site of the most recent hair damage.17 A frank conversation with the child18 and referral for psychiatric treatment are recommended.
Dermatitis artefacta is an artificial, self-inflicted dermatosis. It is caused by mechanical and chemical means that produce lesions of varying shape according to the method used. The lesions are often bizarre, unlike any recognized dermatosis, and the subcutaneous tissue may be affected as well.19 The distribution is typically symmetric or linear and confined to accessible body locations; it is less prominent in the dominant hand. Dermatitis artefacta is seen mainly in young or adolescent girls,20, 21 often with a hysterical component. Although diagnosis can be difficult because patients as a rule deny self-infliction, dermatitis artefacta should be suspected in bizarre lesions confined to accessible areas in patients with hysterical tendencies. Occlusive dressings are strongly recommended since they heal existing lesions and prevent the infliction of new lesions without the patient being aware of the suspected diagnosis.
Although not limited to women, the special aspects of some infectious diseases in women and the female genitalia require a specific approach.
Human papillomavirus (HPV). Infection with HPV is the most common sexually transmitted disease in the United States, considered by some to have reached epidemic proportions.22 Venereal transmission is responsible for the development of most vulvar warts that often appear first in areas traumatized during coitus.23 Genital warts are highly infective with frequent latent and subclinical infections, but the reason for their persistence and high infectivity is unclear.
Vulvar warts may grow markedly during pregnancy23 and vulvar obstruction may necessitate cesarean section. During delivery, the infant may acquire anal or genital lesions as well as laryngeal papillomatosis,23 with a higher risk for the later development of nasopharyngeal and laryngeal carcinoma.
Although most HPV types are associated with benign lesions, there is evidenced of the oncogenic potential of certain HPV types,24 in particular types 16 and 18, which are most frequently associated with advanced dysplasia and invasive carcinoma.23, 25-27 Although a higher risk for cervical neoplasia is a matter for concern, HPV infection appears to be necessary but not sufficient, and the coexistence of other factors is required for the development of the cell to a malignant phenotype.28
Treatment of clinical lesions must be supplemented with cytologic examination of infected women and women partners of infected men, and with a follow-up.
Herpes simplex virus (HSV) and pregnancy. Genital HSV-2 infection is common: the seroprevalence in diverse North American populations of reproductive age ranges from 7 to 65%.29-31 Some 78 to 97% of reported cases are asymptomatic.29-31 Lesion-producing effect of HSV infection of the genital tract was augmented during pregnancy in a study of 256 patients,32 possibly resulting in neonatal herpes. It was estimated that 40-70% of neonatal herpes cases were attributed to reactivation of herpes and shedding of virus from the mother's genital tract during delivery.33-35 Despite antiviral agents, neonatal herpes has significant morbidity and mortality. In mothers with primary genital herpes, intrauterine infection or stillbirth may occur, although the risk is probably small.36-37
HIV infection. Although women were previously considered to constitute only a minority of HIV patients, the proportion of women patients is rising constantly.38-39 In the HIV pandemic today, AIDS represents a leading cause of death among young women aged 25-34 years.40 A high rate of HIV infections is being reported among adolescent girls and in some urban areas the figures approach those for adolescent boys.40 The incidence of AIDS is greater in women of racial and ethnic minorities,41 with higher rates reported among black and Hispanic than among white women.42 Man-to-woman transmission of HIV occurs more readily than woman-to-man or woman-to-woman.42
Heterosexual contact is increasing as a risk factor, joining needle sharing in intravenous drug users as the leading risk factors responsible for most cases of HIV in women.40, 42 In addition, a significant proportion of infected women previously classified as drug users probably acquired it through heterosexual contact.40
The fact that most affected women are in the reproductive age group43 raises concern about the impact of pregnancy on the course of AIDS and transmission of the disease to the child. Pregnancy does not appear to significantly alter the natural progress of HIV disease in women,43 at least not in early HIV infection;41 however, it is estimated that one-third of the children born to HIV-infected women are HIV-positive.44
Pregnancy is associated with significant physiologic changes, largely due to hormonal factors, that are manifested in the skin as well. Pathologic processes, unique or not to the pregnant women, are not the subject of this report.
Physiologic Changes in the Skin
Changes in pigmentation. Hyperpigmentation is a common manifestation, occurring in approximately 90% of women. Although it many be generalized, hyperpigmentation is more commonly circumscribed and located on the face, axillae, areolae, external genitalia, inner thighs, and linea alba ('white line') that changes to linea nigra ('black line').45 A larger number of melanocytes or their greater sensitivity to hormonal stimulation in these locations are possible explanations for these predilections.45-59 Hyperpigmentation clears gradually after delivery but usually not completely45-47 and is more marked, frequent, and persistent in women with darker complexion.48, 50
Melasma 'the mask of pregnancy,' is derived from the Greek word melas meaning black. This mask-like facial hyperpigmentation or roughly symmetric, pigmented brown patches, typically on the central part of the face and forehead, appears in 50-75% of pregnant women.45, 48, 50-53 Pigmentation in susceptible women can be aggravated by sun exposure and contraceptives. Deposition of melanin may be found in the epidermis, dermis, or both. Examination with a Wood's lamp reveals accentuation of the manifestation if it is located in the dermis.48 The mechanism is unclear. A hormonal etiology is strongly suggested, including increased production of melanocyte-stimulating hormone (MSH), estrogen, and progesterone, but these are inconsistent findings.45, 48, 49, 52 Melasma usually clears up within a year after delivery, but persists longer in up to 30% of cases.45 Treatment in persistent cases includes hydroquinone 2 to 5% alone or in mixed formulations, such as Kligman formulation,54 and avoidance of sun exposure, trauma, or cosmetics.45, 49, 55 One study demonstrated satisfactory results with azelaic acid in 65% of patients.56
The darkening of other pigmented lesions during pregnancy, such as ephelides, freckles, and nevocellular nevi, as well as increased appearance of new nevi is well established57 and may be a source of concern.
The impact of pregnancy on melanoma remains controversial. MacKie et al.58 studied the effect of pregnancy on the prognosis in 388 women with stage 1 melanoma before, during, after, and between pregnancies. They concluded that pregnancy probably does not affect the prognosis of melanoma, provided that the thickness of the tumor is controlled. Melanoma thickness was found to be greater in women diagnosed in pregnancy, possibly because pregnancy may darken existing nevi and may stimulate more rapid growth of a melanoma. Wong et al.59 found that pregnancy did not affect survival rate and thickness of melanoma of women with melanoma. Singluff et al.60 concluded that while thickness of melanoma was greater in pregnant women, their overall survival did not differ from that of pregnant women with melanoma.
Vascular changes. Hyperemia, congestion, proliferation of vessels, and vascular instability occur in the pregnant woman and may result in a number of common cutaneous manifestations.
Vascular spiders are angiomas with a central dilated arteriole and small, radiating branches under the epidermis that blanch under pressure on the central point. They are usually seen in areas drained by the superior vena cava, in the neck, face, and arms. They are observed in two-thirds of white45, 51 and 10% of black women.50, 51 Spider nevi begin to appear in the late first trimester45, 51 and usually fade within 3 months postpartum.48 The etiology is attributed to high estrogen levels from an overtaxed liver.51
Palmar erythema appears diffusely on the palm and on the thenar and hypothenar eminences. It is common and occurs in two-thirds of white and one-third of black women;45, 50, 51 it disappears during the postpartum period.45, 47, 48, 51
Varicosities appear in approximately 40% of pregnant women in the legs, hemorrhoidal veins, and in vulvar and vaginal areas.45, 46, 48, 57 Varicosities may cause pain and thrombosis, and usually regress postpartum.45 Thromboses occur more frequently in hemorrhoidal veins than in legs.51
Edema due to fluid retention in dependent areas such as ankles and feet are frequent in pregnancy. Seventy percent of patients may experience edema of the legs in the third trimester; it usually resolves postpartum.48
Pyogenic granuloma (granuloma gravidarum) is a smooth red-to-violaceous nodule, most often seen in the gingiva, that occurs in 2% of pregnancies.50 It generally develops early in pregnancy and regresses spontaneously postpartum.51 It may bleed following trauma,48 cause pain, and even ulcerate,51 necessitating a simple excision. Good dental care is recommended.
Vascular fragility is especially common in the second half of pregnancy,47, 48 and may result in purpura of the lower extremities. Vascular instability may manifest as cutis marmorata, flushing, and increased sensitivity to changes in temperature.48, 50
Striae. Striae (Stretch marks) are an atrophic phenomenon of unknown etiology consisting of purple bands that often appear on the breasts or the lower abdomen.48, 49, 57 They bleach and gradually fade, but never disappear.48
The appearance of postadolescent, usually mild, acne in women seems to be quite common.61, 62 The etiology is not completely clear, but cosmetics and stress appear to play an important role. Some cases present a persistent adolescent acne. Kligman and Mills63 assigned a causative role to cosmetics, calling the condition "acne cosmetica," and identified a variety of substances commonly found in cosmetics as comedogenic. Some 20 years later (1991) Kligman revised this view to emphasize the importance of stress.64 Furthermore, there is evidence of psychologic correlates to the tendency to develop acne, such as conflicts about family and career.65
The typical features of postadolescent acne include scattered lesions, mainly closed comedones, with no or only minimal scarring, predominantly on the chin, the forehead, and the cheeks, usually with premenstrual flares. As a rule there is no history of adolescent acne.64
Complications of Cosmetic Treatment of Nails
Various cosmetic methods have been developed for the care and cultivation of nails, including nail polish, nail polish removers, and nail hardeners. Sculpted artificial nails have been added to the woman's armamentarium, which can cause local damage to the nails and surrounding areas as well as allergic contact reactions. These effects usually occur within a few months of the first application,66 and resolve in a similar period of time. Preformed plastic nails left for 3 or more days may completely disrupt the nail.67
Autoimmune Progesterone Dermatitis
The regular premenstrual appearance of a skin eruption (5 to 10 days before menses)68-70 and subsequent clearance characterize autoimmune progesterone dermatitis (Fig. 2). This rare dermatitis may be mild to severe and is manifested in a wide range of eruptions including dermatitis, particularly pompholyx, urticaria, and erythema multiforme.71 The trunk, extremities, and oral mucosa may be involved. The mechanism is unclear, but Hart72 showed prior exposure to synthetic progesterone preparations to be strongly associated with the phenomena in six of seven women. Provocative tests to aid the diagnosis include intradermal and intramuscular test injection of progesterone: the former generally results in delayed hypersensitivity reactions or an urticarial reaction, while the latter usually evokes immediate reactions.71
Autoerythrocyte Sensitization Syndrome (Painful Bruising Syndrome)
First described by Gardner and Diamond in 1955 in four women,73 this rare syndrome is characterized by recurrent painful ecchymoses and apparently results from autosensitization to red blood cells. It is almost always associated with psychiatric disturbances, often following the trauma of injury, surgery, or stress. It is confined almost exclusively to young and middle-aged women, although it has also been reported in children74 and men.75
The extremities are most commonly affected, where sensations of stinging, tingling, or burning may appear, followed, usually within hours, by painful swellings that develop into ecchymoses of varying sizes. The process may spread to the entire extremity and may require surgical intervention to relieve the pressure.76, 77 The inflammation subsides within one to two days and the ecchymoses fade in a week or two. Systemic symptoms can precede the dermatologic onset by years and may bring the patient to medical attention, including surgery.77, 78 Many patients describe noncutaneous bleeding such as menometrorrhagia, leading often to hysterectomy,77 hematemesis, melena, hematuria, and epistaxis. As Ratnoff stated, "Some believe that they have 'always' bruised easily."77
Neurologic symptoms, mainly severe recurrent headaches, are frequent.77 Abdominal pain and other gastrointestinal complaints as well as genitourinary and musculoskeletal symptoms are reported. Many patients experience cutaneous drug reactions.77 Characteristic personality patterns and psychological traits ascribed to these patients include hysterical and masochistic features.79, 80 It is noteworthy that a high proportion of patients see themselves in self-sacrificing roles. including nursing and other medically related jobs.77, 80
Histologic examination of the skin lesions may show vasculitis with dermal edema, perivascular infiltrate of neutrophils and monocytes, and extravasation of erythrocytes. The etiology is uncertain, although Gardner and Diamond theorized that extravasation of erythrocytes during an earlier trauma result in autosensitization to the stroma of the patient's own erythrocytes. This was supported by findings that typical skin lesions could be provoked by intradermal injections of autologous red blood cells; but, because the lesions appear inconsistently, negative results do not exclude the diagnosis.76
Therapy in disappointing and a large number of agents including steroids, antimalarials, antihistaminics, and antimicrobials have been proven to be of no benefit or effective only in isolated cases.76 Psychotherapy may help some patients, especially teenagers.76, 77 Plasmapheresis was reported as successful,81, 82 but more patients are needed to assess its benefits. The course and outcome vary greatly, and the symptoms resolve spontaneously in a few months in some cases and persist for years in others.
The uncontrolled urge to pick one's own skin is common and observed predominantly in women,83 especially young and middle-aged women (Fig. 3). It may be done with full awareness, although some women carry out the picking ritual in a trance-like state.84 Some patients use accessory tools.85 The distribution is symmetrical, often linear, usually in accessible parts of the body, often in extensor aspects of the extremities, on the face, and the upper back. The lesions range in size from a few millimeters to 1 to 2 cm, and can be observed at any given time. Healing may leave scars and hypo- or hyperpigmentation. An obsessive compulsive personality is considered to be present, with the excoriations serving to relieve tension and anxiety. Therapy includes topical antibiotics and psychiatric referral.
MENOPAUSE AND OLD AGE
An increased life expectancy means that nearly one-third of a woman's life today is spent after menopause. This has raised the need to deal with a number of problems specific to this growing group of women. The climacteric period is characterized by typical physiologic changes, many of them related to decreased estrogen production by the ovaries.
Lichen Sclerosus et Atrophicus (LSA)
Lichen sclerosus et atrophicus, first described in 1889 by Hallopeau,86 is an infrequent, chronic atrophic disease most commonly affecting the vulva of postmenopausal white women,87, 88 although it may appear in any age, in all races and both sexes.11 When it occurs in children, it affects mainly girls.11, 87
The disease manifests as small, flat, ivory or porcelain-white polygonal macules and papules, often with follicular yellow-to-black horny plugs, forming crinkly atrophic plaques. Bullae and purpura may also be seen. Genital lesions show characteristic 'hourglass configuration' of white atrophy. Anogenital lesions are most common, and may itch severely. Extragenital lesions occur less frequently, are usually asymptomatic, and are usually confined to the upper trunk and extremities, although any part of the body can be affected, including the mucosa and tongue.
The etiology is unknown. A genetic link is suggested by observations of successive generations with the disease10, 89 and the identification of some HLA types.10, 87 A hormonal factor was proposed because of the predilection for postmenopausal women, the postpubertal improvement in girls, and reports of abnormally low levels of testosterone, dihydrotestosterone, and androstenedione.88
Morphea and a number of autoimmune diseases such as vitiligo were reported associated with LSA.90, 91 The association with autoimmunity is also seen in the increased frequency of organ-specific antibodies in patients with LSA and autoimmune diseases reported in both patients and relatives.89-91 A relation to infectious agents was proposed when Borrelia burgdorferi was reported associated with LSA.10 Preceding chronic vulvovaginitis and balanitis in some reports92 also support an infectious etiology. Occasional exhibition of isomorphic phenomenon was suggested following reports of LSA after trauma.92 These include old surgical scars,93 scratch marks,94 a vaccination scar,95 radiation scars,96 and sunburn.92
Typical histologic features are hyperkeratosis and follicular plugging, a thin epidermis with effected rete ridges and hydrophic degeneration of the basal layer, the appearance of edamatous hyalinized collagen in the upper dermis, and a mid-dermal band-like inflammation infiltrate.
The relationship of LSA to the development of malignancy varies greatly in different studies. In a review of 1356 patients with LSA, the coincidence with cancer was found to be 4.1%.10 Lichen sclerosus et atrophicus was detected in up to 53% of patients with invasive vulvar carcinoma.10 Such an association was not reported with extragenital lesions. The increased frequency of malignancy with genital LSA demands a long follow-up and repeated biopsies when necessary. Treatment includes topical corticosteroids or testosterone.
Paget's Disease of the Nipple
This unilateral, erythematous, scaling eruption first appears in the nipple or the areolae of the breast as sharply delimited lesions that expand slowly, developing over months and years with infiltration, ulceration, and nipple retraction. It appears almost exclusively in women. It is considered to have a worse prognosis in men,97 in whom it has been associated in some cases with estrogen treatment for carcinoma of the prostrate.98
The disease represents an underlying ductal adenocarcinoma of the breast, and axillary lymph node metastases are a leading prognostic factor, as in other breast malignancies. These metastases are reported in different series to accompany 0 to 33% of cases when there is no palpable mass, and 67% of cases when there is a palpable mass.99, 100
Because of the clinical similarity to dermatitis of the nipple, Paget's disease should be considered in the differential diagnosis of any unilateral persistent dermatitis that does not respond to conventional therapy. biopsy is mandatory in such cases; typical histologic features, such as Paget's cells, aided by histochemical stainings are diagnostic. The treatment is mastectomy.
Some 75 to 85% of perimenopausal women experience hot flashes of varying severity proportional to the actual rate of decrease in estrogen.101, 102 These vasomotor symptoms reflect an instability between the autonomic nervous system and the hypothalamus and may last a few months to several years.101-103
The appearance of hyperkeratosis on the palms and soles, usually in menopausal women, was first reported by Haxthausen in 1934.104 Typically, plantar pressure points are first affected,105 more often in obese women. No specific hormonal factor could be identified and keratoderma may be seen in men as well. Treatment includes keratolytics; topical steroids are not effective. Etretinate was reported to improve the hyperkeratosis and pain.105
Complications of Hormone Replacement Therapy
Hormone replacement therapy may prevent many of the manifestations related to reduced estrogen levels that are associated with menopause. The main reasons for this treatment are to decrease the risk of cardiovascular diseases106, 107 and the prevent further osteoporosis.108 It can ameliorate other disturbing symptoms as well, such as vasomotor symptoms (e.g., hot flushes), vaginitis, and atrophy. A higher risk for breast and endometrial cancer connected with hormone replacement therapy raises concern, although low-dose estrogen does not appear to increase the risk of breast cancer, and adding progesterone eliminates the risk of endometrial cancer.102
Cutaneous manifestations reported with estrogen therapy include some of the changes seen in pregnancy: development of nevocellular nevi, vascular spiders and chloasma, hyperkeratosis of the nipples, and acanthosis nigricans.109
Complications of Esthetic Techniques
Advances in esthetic techniques and a longer life span have generated and popularized various nonsurgical means for correcting the damage of aging and sun exposure to the skin. These methods are more successful when patients are carefully selected and the practitioner is familiar with the possible adverse side effects.
Topical tretinoin and alpha hydroxy acids have been found to improve small wrinkles, while purified bovine collagen, silicone and fat, and chemical exfoliation are well documented treatments for larger wrinkles. Purified bovine collagen injections110-121 have gained popularity in the last few years, despite the high cost from the requisite repeated injections every few months. Untoward effects that are not common include allergic reactions, anticollagen antibodies, arthralgia, and myalgia. The development of granulomatous reactions has been reported.118 Intradermal tests for hypersensitivity113, 114 should be done prior to injections.
The effect of deep phenol peeling is long lasting and may persist for 20 years.122 Unfortunately, phenol is cardiotoxic and can induce cardiac arrhythmias as well as hepatic and renal toxicity, necessitating careful evaluation before treatment and cardiac monitoring during the procedure.123 Trichloroacetic acid is another chemical widely used for exfoliation, with the advantage of not being significantly absorbed and therefore not causing systemic complications.123
Both phenol and tricholoroacteic acid may produce a number of adverse skin reactions. Pigmentary irregularities that can occur with both agents were the most common problems observed in a study of hundreds of patients undergoing chemical face-peeling with phenol.124 Pigmentary changes are reduced by avoidance of sun exposure for 3 to 6 months following peeling.123 Hypertrophic scars or keloids are most often observed periorally or near the mandible.123 Concurrent surgery and chemical peeling should be avoided because of increased risk of development of keloids.123, 125, 126 Chemical peeling may exacerbate preexisting abnormalities such as prominent pores, darkening of nevi, and telangiectasias.123 Other adverse reactions are milia, local persistent erythema,123 and (rarely) a full-thickness skin loss.123, 125 A previous herpes simplex infection may flare up123, 127 and can be prevented by pretreatment with acyclovir that also eliminates secondary scarring. The procedure should be avoided in the presence of an active herpetic flare.123
Dercum's Disease (Adiposis Dolorosa)
Adiposis dolorosa was first described by Dercum in 1892129 in three patients with multiple painful fatty deposits, asthenia, and emotional disturbances. The disease is characterized by the development of painful or tender fatty deposits in the subcutis, usually in obese menopausal women, often accompanied by psychic disturbances, ecchymoses, weakness, and asthenia that develop with progression of the disease. There is a predilection of the fat tissue to be deposited in the extremities, especially near the joints.130, 131 It is 30 times more common in women than men.130 An autosomal dominant trait with incomplete penetrance was suggested in some families.132, 133
Various etiologies have been proposed, including an endocrine basis and disorders of fat metabolism. Blomstrand et al.134 found local biochemical defects in lipid metabolism, especially fatty acid synthesis with blocks in C16 and C18, in a small number of patients. In another study, capillary microthrombi were found in the symptomatic lipomas.135 The reason for the pain that occurs spontaneously of by palpation is obscure and has been attributed to ischemia due to microthrombi135 and pressure on nerves by fatty deposits.136 Adiposis dolorosa has been reported associated with several psychological disturbances ranging from irritability to depression, memory disturbances, dementia, and hypochondriasis. The histological picture is nonspecific and not diagnostic.
Spontaneous resolution has been reported and a few cases have responded to symptomatic medications.136 Most patients resist conventional analgetics. Procaine solutions137 and I.V. lidocaine produced successful results,138-142 with an increasing during of symptom relief when therapy was repeated after a few pain-free months.139 Surgical excision and weight reduction were found beneficial in some cases.143
The unique aspects of a woman's life and physiologyincluding menarche, pregnancy, and menopauseas well as the ramifications of modern lifestyle and longevity require special approaches to the accompanying dermatologic phenomena. This review is meant to focus the spotlight on a subject that both merits and demands attention.
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From the Department of Dermatology, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
© 1995 International Journal of Dermatology. Reprinted here with the kind permission of the Editor-in-Chief, Dr. Larry Parish, and author Dr. Sarah Brenner.
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