Pigmentary Incontinence Research Articles

An histological and ultrastructural study of the ‘dirty neck’ appearance in atopic eczema

The 'dirty neck' appearance is a characteristic disorder of pigmentation, which has previously been found to affect approximately 2% of adult atopics. This disorder results in a rippled pattern of hyperpigmentation similar to that seen in macular amyloidosis. Biopsy specimens from affected skin of three patients were examined by histological and electron microscopical techniques. In addition to eczematous changes, marked pigmentary incontinence was observed. Amyloid-like material was detected by electron microscopy but not by light microscopy in all three specimens. Some deposition of amyloid occurs in this condition but the pigmentary changes are attributable to melanin incontinence. In 1987, two separate groups, Manabe et al. and Colver et al., described a distinctive type of hyperpigmentation, found particularly on the neck in some patients with chronic atopic eczema. Manabe's group found that 1.7% of 700 patients with atopic eczema showed this clinical characteristic, and that only adolescents and adults were affected. This condition has been called the 'dirty neck' appearance or 'ripple pigmentation of the neck in atopic dermatitis'. The clinical features are a rippled pattern of hyperpigmentation seen particularly on the anterior and lateral aspects of the neck (Fig. 1). We have also observed the same appearance in the inguinal areas. A number of factors could contribute to the development of hyperpigmentation in chronic atopic eczema. It is possible that the 'dirty neck' appearance is a form of post-inflammatory pigmentation due to previous eczema, ultraviolet exposure or even the application of photosensitizing products, and that the rippled appearance is related to the cutaneous anatomy of the neck. The pathogenesis of the 'dirty neck' is obscure, but in common with previous authors, we have noted that the rippled appearance of the hyperpigmentation resembles that seen in macular amyloidosis. In order to determine whether deposition of amyloid contributes to the development of this type of pigmentation in atopic dermatitis, an histological and ultrastructural examination of skin biopsies from three patients with the condition was undertaken.

多発性悪性外毛根鞘腫のｌ例

A case of multiple malignant trichilemmoma on the left forehead and the right chest in a 74-year-old man is reported. The lesion on the right chest was the annular tumor measuring 40×35mm in size with a granular surface. Histopathological examination revealed that no cancer cells were found in the atrophic epidermis on the central area of the annular tumor and that several lymphocytes surrounded and attached to cancer cells on the inner boundary of the annular tumor. Pigmentary incontinence and many mononuclear cell infiltrates were observed in the upper dermis of the central area. A recent review of 73 cases of malignant trichilemmoma reported in Japanese literatures revealed that the lesion in multiple forms was presented in four cases (5.5%) and the annular lesion was presented in only one case (1.4%) in which cancer cells existed in the central area.

Familial linear and whorled nevoid hypermelanosis

Two patients with familial linear and whorled nevoid hypermelanosis, a 33-year-old woman and her 3-month-old daughter, are described. These are the first cases of linear and whorled nevoid hypermelanosis of familial occurrence reported in the literature. Asymptomatic hyperpigmented macules in streaky configurations had appeared on the trunk and extremities of both patients several weeks after birth and then gradually spread. No previous inflammation or eruption was observed. Histologic examination revealed a slight increase in the number of melanocytes in the epidermis and an irregular basal melanosis. No pigmentary incontinence or melanophages were observed in the dermis. Ultrastructurally the only finding was an increase in the number of normal-appearing mature melanosomes in keratinocytes in the lesion. Chromosomal analysis of cultured peripheral blood lymphocytes and dermal fibroblasts from normal and pigmented skin revealed no evidence of mixoploidy or chimerism. An undescribed genetic abnormality is suggested as the cause.

Melanocytic lesions in a private pathology practice. Comparison of histologic features in different tumor types with particular reference to dysplastic nevi.

This study reviews a total of 1000 melanocytic lesions--two separate 500 consecutive sample groupings from 1980 and 1989, respectively--diagnosed in a private non-hospital-associated pathology practice. Lesions were classified as lentigo simplex, congenital nevus, "common" nevus, dysplastic nevus, blue nevus, Spitz's nevus or malignant melanoma. A comparison of the two periods reveals an increase in dysplastic nevi from one in 1980 to nine in 1989. The histologic changes in these nevi were compared to those of the other tumors. Pronounced cytologic atypia was seen in the melanocytes of a few "common" nevi, but more often in the dysplastic nevi and in all of the melanomas. Slight nuclear atypia was usual in "common" nevi and lentigines, and also fibroplasia, lymphocytic infiltration, vessel proliferation and pigment incontinence were seen in both "common" nevi and dysplastic nevi. It is concluded that no single histologic variable was specific for dysplastic nevi.

Linear and Whorled Nevoid Hypermelanosis

A case of hyperpigmentation distributed in streaks and whorls was recently delineated and separated from a confusing number of pigmentary disorders. A 13-year-old boy had hyperpigmented, reticulate spots on his trunk and neck in a linear and whorled distribution following the lines of Blaschko. Onset was at age 1 year. Histologically, the spots showed increased melanin in the basal layer of the epidermis, but no increase in melanocytes, incontinence of pigment, or melanophages were seen in the dermis. The karyotype was 46,XY and there was no evidence of chromosomal mosaicism or chimerism. This entity represents a disorder separated recently from other pigmentary alterations that follow Blaschko lines, such as Bloch-Sulzberger incontinentia pigmenti, hypomelanosis of Ito, early systematized epidermal nevus, and chimerism.

Pigmenting pityriasis alba.

We conducted a prospective study of 20 patients with pigmenting pityriasis alba (PPA) over a period of two years. Characteristic morphology revealed a central zone of bluish hyperpigmentation surrounded by a hypopigmented, slightly scaly halo of variable width. All patients displayed lesions on the face. Concomitant extrafacial involvement was uncommon. A significant finding was an associated dermatophyte infection in 13 patients (65%). These patients all received griseofulvin 10 mg/kg/day for eight weeks, resulting in the resolution of PPA in seven within 4 to 20 weeks. These were also treated with 1% hydrocortisone. Biopsy specimens from two patients showed similar features, namely, a subacute dermatitis with variable pigment incontinence. Immunohistochemical labeling revealed a preponderance of T lymphocytes. Pigmenting pityriasis alba seems to be a variant of classic pityriasis alba showing a strong association with dermatophyte infection, especially tinea capitis. It may be related to lichenoid melanodermatitis.

Pulse duration effects on cutaneous pigment.

Melanin, an endogenous chromophore in pigment containing cells in skin, is being specifically altered by lasers using the principle of selective photothermolysis (SPT). This implies that a combination of specific laser parameters of wavelength, pulse duration, spotsize, and energy density are required to confine the delivered laser energy to the targeted cells alone. Because the bulk of cutaneous pigment is localized to epidermal basal cells, pigmentary incontinence has been found to occur in skin exposed to laser irradiation. This study demonstrates that pulse duration or exposure time of the laser affect the severity of pigmentary incontinence induced. Pigment granules are more abundant, aggregated, and located deeper in the dermis following exposure to 500 nsec pulse duration than 100 nsec at a wavelength of 504 nm. This relationship appears to be independent of the laser energy density used.

Functional analyses of the superficial stratum corneum in atopic xerosis

• Dermatologists universally recognize that the unaffected skin of patients with atopic dermatitis tends to be dry and slightly scaly. To characterize the functional properties of the superficial stratum corneum in atopic xerosis, we studied the forearms of 28 patients with atopic dermatitis, aged 14 to 30 years, and 18 age-matched controls, with the use of mainly noninvasive methods. Patients with atopic xerosis showed markedly higher transepidermal water loss and markedly lower skin surface hydration levels than did the controls. The corneocytes in atopic xerosis tended to desquamate in clumps of cell aggregates instead of as individual cells. They contained a substantially lower amount of water-soluble amino acids, which play a role in the water-retaining capacity of stratum corneum, than did those of controls. Although the number of stratum corneum cell layers in atopic xerosis (21 ± 4) was substantially larger than that in controls (15 1), its turnover time (7 ± 2 days) was appreciably shorter than that for controls (14 ± 2 days). Like those noted in the skin with increased epidermal proliferation, the size of superficial corneocytes in patients with atopic xerosis was substantially smaller than in controls. Histopathologic examination revealed acanthotic epidermis, mild perivascular mononuclear cell infiltrate, and pigment incontinence. Atopic xerosis, the dry skin of patients with atopic dermatitis, shows various stratum corneum functional impairments, probably reflecting increased epidermal proliferation due to a low-level ongoing dermatitis. (<i>Arch Dermatol.</i>1991;127:1689-1692)

Functional Analyses of the Superficial Stratum Corneum in Atopic Xerosis

Dermatologists universally recognize that the unaffected skin of patients with atopic dermatitis tends to be dry and slightly scaly. To characterize the functional properties of the superficial stratum corneum in atopic xerosis, we studied the forearms of 28 patients with atopic dermatitis, aged 14 to 30 years, and 18 age-matched controls, with the use of mainly noninvasive methods. Patients with atopic xerosis showed markedly higher transepidermal water loss and markedly lower skin surface hydration levels than did the controls. The corneocytes in atopic xerosis tended to desquamate in clumps of cell aggregates instead of as individual cells. They contained a substantially lower amount of water-soluble amino acids, which play a role in the water-retaining capacity of stratum corneum, than did those of controls. Although the number of stratum corneum cell layers in atopic xerosis (21 +/- 4) was substantially larger than that in controls (15 +/- 1), its turnover time (7 +/- 2 days) was appreciably shorter than that for controls (14 +/- 2 days). Like those noted in the skin with increased epidermal proliferation, the size of superficial corneocytes in patients with atopic xerosis was substantially smaller than in controls. Histopathologic examination revealed acanthotic epidermis, mild perivascular mononuclear cell infiltrate, and pigment incontinence. Atopic xerosis, the dry skin of patients with atopic dermatitis, shows various stratum corneum functional impairments, probably reflecting increased epidermal proliferation due to a low-level ongoing dermatitis.

Familial acquired pigmented streaks in the nail

A 40-year-old man developed multiple longitudinal pigmented streaks in his nails. His sister was also found to have a single streak on a fingernail. Neither had any history of drug ingestion or recent illness and examination failed to reveal any other signs in the skin or mucosal surfaces. A biopsy of the nail matrix at the base of one streak showed no evidence of a naevus or lentigo, the principal finding being pigmentary incontinence and excess macrophages.

Dermatopathia Pigmentosa Reticularis

Dermatopathia pigmentosa reticularis (DPR) is a reticulate pigmentary disorder of as yet unclear inheritance that was first described in 1958. Since then only an additional eight cases have been reported, all in the European literature. To our knowledge, this is the first case from the United States of America and the first with an ultrastructural study.The patient is a 44-year-old man of mixed Black, Hispanic and American Indian descent with generalized, variably pigmented skin over the entire body present since birth.A punch biopsy of pigmented forearm skin was taken for light microscopy which demonstrated hyperkeratosis, an intact granular layer, and epidermal atrophy with focal liquefaction degeneration of the basal cell layer. The dermis showed pigment incontinence. Direct immunofluorescence was negative for immunoglobulins and complement at the basement membrane zone. A second skin biopsy of an elbow plaque showed the above changes except for the alteration of the basal layer.

Poikiloderma‐like Lesions on the Neck in Atopic Dermatitis: A Histopathological Study

Reticulate pigmentation with or without skin atrophy, depigmentation and telangiectasia is frequently encountered on the neck of severe cases of adult type atopic dermatitis. These skin changes were graded clinically into 3 stages. Based on histological features, hyperplasia of the sebaceous gland, dilated tortuous capillaries, and mild degeneration of elastic fibers were noted in stage I lesions. Lesions of both stages II and III contained increased melanin in the basal cell layer with incontinence of pigment, remarkable destruction and degeneration of elastic fibers, proliferated and dilated capillaries, and deposition of mucinous substances. The numbers of mast cells in papillary dermis were significantly increased in late stage I and stage II lesions. Poikiloderma-like lesions on the neck could be attributable to chronic inflammation and delay of wound healing process, possibly caused by long-standing topical corticosteroid therapy.

Epidermolysis bullosa simplex with mottled pigmentation

A patient with epidermolysis bullosa simplex with mottled pigmentation is described. Clinical features include blistering of the skin, especially of the extremities; healing without scars; slight atrophy of the skin; and striking mottled pigmentation of the trunk. Histologic examination of a biopsy specimen from freshly frictioned, clinically uninvolved skin indicated a split inside the basal keratinocytes, focal hyperpigmentation of the basal cells, and pigment incontinence without an inflammatory infiltrate. Indirect immunofluorescence demonstrated focal discontinuity of the basement membrane zone. Electron microscopic examination revealed basal keratinocytes with few intact intracellular organelles, aggregated tonofilaments, and subnuclear splitting with the basal parts of the cells adhering to the basement membrane, Both normal basement membrane and zones of irregular and interrupted structures were seen. Hemidesmosomes and anchoring fibrils appeared to be normal.

DRUG ERUPTIONS WITH LICHENOID HISTOLOGY PRODUCED BY CAPTOPRIL

Three cases are presented with a rash due to captopril which, on histology, showed a lichenoid pattern of bandlike upper dermal infiltrate of lymphocytes and histiocytes, together with hydropic degeneration of the basal layer of the epidermis producing Civatte bodies and pigmentary incontinence. The rash presented either as a pityriasis rosea-like picture which appeared about three to six months after the onset of treatment in patients taking low doses, or alternatively, as lichenoid plaques which appeared three to six months after commencement of medication in patients taking high doses. Although both clinical patterns produced a "saw tooth" pattern of the epidermal rete ridges, hypergranulosis and acanthosis were seen only with the lichenoid plaques.

Actinic lichen planus: A clinicopathologic study of 16 patients

The clinical and histopathologic features of actinic lichen planus in 16 patients were studied. The majority of the patients were young, and men and women were almost equally affected. The relative incidence of this condition, compared with the incidence of all forms of lichen planus, was smaller than that reported in the literature. The eruption was distributed over sun-exposed areas, with particular predilection for the face. Covered areas and mucous membranes were spared except for one patient who had involvement of the vermilion border of the lower lip. In most cases the lesions consisted of erythematous brownish plaques with an annular configuration. Less commonly, discrete and confluent papules and hypermelanotic patches, sometimes assuming a melasma-like appearance, were present. The prominent histopathologic features consisted of an interface dermatitis characterized by coarse vacuolar degeneration of the basal cell layer, a mid-dermal perivascular predominantly lymphocytic inflammatory cell infiltrate, and a significant degree of pigment incontinence. Parakeratosis, eczematous changes confined to the follicular epithelium, and a variable degree of solar elastosis were seen in some patients. Mucin deposition was noted in the reticular dermis in two cases.

Generalized Pinhead-Sized Papules in a Child

REPORT OF A CASE A 10-year-old girl presented for evaluation of a mildly pruritic eruption of one year's duration. The eruption was initially noted near the elbows but it slowly progressed to involve the entire body. The patient noted no ameliorating or aggravating factors. Her general health was excellent and she was receiving no medication. Physical examination revealed numerous 1-mm uniform, smooth, flesh-colored papules over the entire body (Fig 1). The elbows, buttocks, thighs, and knees showed grouping of the lesions without coalescence. Koebnerization was observed in an abrasion of the right anterior tibia. The palms, soles, and oral mucosa were spared. No nail changes were present. A skin biopsy specimen from the right anterior thigh exhibited a well-circumscribed lymphohistiocytic infiltrate adjacent to the epidermis (Fig 2). The infiltrate was surrounded by the rete ridges in a ball in claw configuration. Vacuolar change and incontinence of pigment were present

The mechanisms of histological incontinence of pigment: light and electron microscopic studies of lesions and patch test sites in female facial melanosis and DNCB reactions in guinea pigs.

AbstractHistological incontinence of pigment (HIP) was studied using light and electron microscopy in a pigmented lesion from a female facial melanosis (FFM) patient and in another lesion produced by isoeugenol when the patient was patch tested. The following sequence of events is thought to underlie HIP. Phagocytes invade the epidermis and phagocytize melanosomes either in keratinocytes or in cytoid bodies, which are degenerated keratinocytes. These phagocytes then return to the dermis through gaps in the basal lamina.In brown guinea pigs sensitized to DNCB, 0.02% DNCB in acetone was applied repeatedly to the same area of the abdomen once per week for six weeks. HIP was observed in 28% of the animals following the fifth application of DNCB. In contrast to the HIP process observed in the patient, phagocytes in brown guinea pigs phagocytized free melanosomes. In irritant reactions to DNCB in brown guinea pigs, only concentrations which produced epidermal necrosis induced HIP.

A sweating abnormality in incontinentia pigmenti

Three women presented with pale hairless streaks on the limbs as the only remaining sign of incontinentia pigmenti (IP). Seven other women in whom IP had been diagnosed in infancy were re-examined and this sign was present in all and in the otherwise unaffected mothers of three affected girls. Sweating was studied in these lesions because we were struck by the clinical overlap between IP and anhidrotic ectodermal dysplasia (AED) and because the lesions resembled those of hypomelanosis of Ito (HI), which are anhidrotic. Sweating was stimulated locally by iontophoresis of pilocarpine and stained with ferric chloride and tannic acid. Sweating was absent in these lesions. Biopsies showed absent sweat glands and follicles but no abnormal pigmentary incontinence. These are not the changes of post-inflammatory scarring and they differ in distribution, degree and depth from the early inflammatory lesions of IP. It is concluded that maldevelopment of hair follicles and sweat glands is a feature of IP which should be considered as another X-linked anhidrotic ectodermal dysplasia. This view is supported by the recent genetic localization of both conditions to the juxta centromeric region of the X chromosome.

Postsclerotherapy hyperpigmentation: a histologic evaluation.

Linear or macular pigmentation occurs in 10-30% of patients following sclerotherapy of vessels between 0.1 and 5 mm in diameter. Its occurrence is related to solution strength, vessel fragility, injection pressure, and the type of solution used. This adverse sequela of treatment has been assumed, by some, to represent post-inflammatory hyperpigmentation (incontinence of melanin pigment), and has been said to occur in individuals with this tendency. Histologic data presented in this paper suggest that this phenomenon does not represent melanocytic alteration, but is secondary to extravasation of red blood cells into the dermis following rupture of fragile vessels with resulting deposition of hemosiderin. Therapy has included bleaching agents (hydroquinones), trichloroacetic acid, and phenolic peeling agents with variable success. Eighty percent of patients who experience this adverse sequela will clear spontaneously within 6-24 months. The remaining patients will have persistence of pigmentation for up to 5 years, with a small number of patients having pigmentation persisting 5 years after therapy.

Pachyonychia congenita with cutaneous amyloidosis and y erpigmentation — a distinct variant

Two kindreds manifesting an unusual form of pachyonychia congenita are described. Clinical involvement consists of nail dystrophy, which tends to improve with age, and moderate palmoplantar hyperkeratosis. In addition, all affected members show a characteristic pattern of cutaneous hyperpigmentation, which resembles macular amyloidosis around the neck and waist, but which confers a dappled appearance to the axillae, popliteal fossae, thighs, buttocks, and lower aspect of the abdomen. With advancing age the pigmentation fades. Histologic and ultrastructural examination of the hyperpigmented skin has revealed pigmentary incontinence and deposition of amyloid within the papillary dermis. These features appear to constitute a distinct variant of pachyonychia congenita.