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Linear psoriasis is a rare entity. It is commonly confused clinically and histopathologically with verrucous epidermal nevus. We report a child who had linear psoriasis in a unilateral distribution with palmoplantar, nail, and scalp involvement.
Monilethrix is a rare developmental hair shaft defect characterized by small elliptical node-like deformities with increased hair fragility resulting in partial or diffuse alopecia. The disorder is usually transmitted in an autosomal dominant fashion with incomplete penetrance and variable expressivity, but autosomal recessive inheritance has also been reported. It is thought to be without systemic involvement, whereas keratosis pilaris and follicular papules are almost invariably associated features. We describe an instance of monilethrix in a 9-year-old boy from consanguineous parents, characterized by universal dystrophic alopecia associated with intractable scalp pruritus, diffuse keratosis pilaris, and bilateral posterior subcapsular cataracts. His disease was further characterized by physical underdevelopment and distinct features of hypertelorism, a wide-based nose, long philtrum, relatively large mouth with thick lower lip, enlarged forehead, small, receding chin, short neck, and rounded (ultrabrachycranial) skull. The findings in our patient suggest that "monilethrix syndrome" is an appropriate term for defining the instances of monilethrix associated with other abnormalities. We conclude that our patient may represent a new and severe, autosomal recessive variant of monilethrix syndrome.
An unusual association of scrofuloderma of the scalp, psoas abscess, and caries spine was seen in a 4-year-old girl. The diagnosis was confirmed by relative and absolute criteria. The peripheral T-lymphocytes and their subsets were within normal limits, although her immunohistopathological changes indicated an intermediary response across the spectrum depicting an unstable immunity. This may have been responsible for the silent spread of Mycobacterium tuberculosis and the current association.
Giant seborrheic keratosis was observed on the frontal scalp of a 44-year-old man. The lesion was successfully treated with topical fluorouracil.
Terminal hair follicles extracted after ethylenediaminetetraacetic acid treatment preserved the basal cell surface of the outer root sheath well enough to examine the outer surface in detail. Light microscopic observations of whole mount specimens revealed several sawtooth-like structures of follicular epithelium in the lower portion of terminal hair follicle. Vertical sections of the human scalp also showed several invaginations of the outer root sheath of terminal hair follicles in the lower portion, not in association with the arrector pili muscle. Scanning electron microscopy of the same specimens demonstrated accordion-like structures below the sebaceous gland in the lower half of anagen terminal hair follicles and surrounding the entire circumference of the follicles. Sawtooth-like structures were unilaterally observed at the level of the middle or lower portion below the sebaceous gland. With transmission electron microscopy, these structures were seen as undulations of basal cells which preserved hemidesmosomes, although the basal lamina had been peeled off during the extraction procedure. Thus, this accordion-like structure is a genuine structural variation of some human anagen hair follicles and not an artifact.
A rudimentary meningocele, a variant of primary cutaneous meningioma, was seen on the scalp of a 9-month-old Japanese boy. Clinically, the lesion on the left parietal area was round, about 1.6 cm in diameter, alopecic, and slightly elevated. Histologically, the lesion, located from the dermis to the subcutis, consisted of scattered foci of meningothelial cells, an anastomosing network of empty spaces with psammoma bodies and collagen bodies, and small vessels. Immunohistochemically, the meningothelial cells were positive for vimentin and desmin. Ultrastructurally, they had elongated cytoplasmic processes, intermediate filaments in the cytoplasm, and desmosomal junctions.
We report here a 7-year-old Japanese girl with cerebriform intradermal nevus (CIN). By placement of expanders on the galea, her scalp was expanded more easily with less discomfort than is expected when the expanders are placed under the galea. An immunohistochemical study on the expression of proliferating cell nuclear antigen suggested higher proliferative activity of nevus cells from the CIN lesion than that of cells from congenital or acquired intradermal nevi. The high proliferative activity appeared to be associated with a growth spurt of the lesion.
Epidermotropic metastatic malignant melanoma (EMMM) is a form of metastatic malignant melanoma that has dermal cell nests with epidermotropism and specific histopathological features. We report a patient with eight nodular lesions of the scalp with histopathological findings compatible with EMMM. The tumors developed one year before consultation and increased in size simultaneously. The histopathological findings of all eight tumors were very similar. The tumor cells were located mainly in the dermis and partly in the basal layer of the epidermis. They contained melanin pigments and were positive for anti-HMB45 antibody. The tumors did not respond to combination chemotherapy with dacarbazine, nimustine, vincristine, and interferon-beta. Therefore, all the tumors were surgically removed. No local relapse, distant metastasis or re-elevation of plasma 5-S-cysteinyldopa was identified during nine months of follow-up. Histopathologically, all eight tumors lacked apparent vascular invasion, which may be related to a slow clinical course of the present case.
A syndrome, consisting of a congenital abnormality of the scalp, ears and nipples inherited as an autosomal dominant trait, is reported. Ten affected members of a family over five generations are recorded and the four living affected members are described in detail. A review of congenital localized aplasias of skin suggests that this may be a new syndrome.
A case of angiosarcoma of the scalp is reported. The histogenesis of this tumour is discussed in terms of the ultrastructural and immunohistochemical findings. This type of angiosarcoma is uncommon and carries a poor prognosis: the therapeutic alternatives are discussed.
A giant cellular blue naevus of the scalp is described in a man with presentation in the third decade of life. In contrast with previous reports of children with this condition, there was no abnormality of the underlying skull bones and no evidence of malignancy.
The efficacy, tolerability and safety of calcipotriol solution and betamethasone 17-valerate solution were compared in a multicentre, prospective, randomized, double-blind, parallel group study. Four hundred and seventy-four patients with scalp psoriasis were recruited from six European countries and Canada. Following a 2-week washout period, either calcipotriol solution (50 micrograms/ml) or betamethasone 17-valerate solution (1 mg/ml) was applied twice daily for 4 weeks. After this time, patients who required no further active treatment were observed for relapse. Retreatment with calcipotriol was offered to those patients who relapsed, and who were originally in the calcipotriol-treated group. The two treatment groups were well matched at baseline. At the end of treatment, the proportion of patients who had 'cleared' or 'markedly improved' was statistically significantly greater in the betamethasone group (75%) than in the calcipotriol group (58%) (P < 0.001) (95% confidence interval of difference 25.3-->8.6). The decrease in total sign score (sum of scores for erythema, thickness and scaliness) at the end of treatment was also statistically significantly greater in the betamethasone group (61%) than the calcipotriol group (45%) (P < 0.001) (95% confidence interval of difference 9.7-->23.1). Adverse events were reported by 87 patients in the calcipotriol group, and 31 patients in the betamethasone group; the most common was lesional or perilesional irritation, which occurred significantly more frequently with calcipotriol (26%) than with betamethasone (8%) (P < 0.001). Fifteen patients (6%) in the calcipotriol group and four (1%) in the betamethasone group withdrew from the study because of adverse events or unacceptable treatment response (P = 0.017).(ABSTRACT TRUNCATED AT 250 WORDS)
A 53-year-old woman presented with erosive pustular dermatosis of the scalp 6 weeks after a bifrontal skin flap and right frontal craniotomy had been performed for removal of a suprasellar meningioma. Although some authors consider that acute local trauma is a precipitating factor for this condition, we believe that this is the first reported case following a neurosurgical procedure.
Two patients, a mother and son, with a conspicuous redundant unique scalp fold are reported. No underlying disorder was found. Light microscopy was unremarkable. An atypical familial form of cutis verticis gyrata is suggested.
Thirteen cases of angiosarcoma of the face and scalp have been examined using immunohistochemistry and electron microscopy. Endothelial cell markers have been employed in an immunoperoxidase technique on tissue that has either been routinely processed, periodate-lysine paraformaldehyde fixed (PLP) and cold processed, or fixed in methacarn. A consistent pattern of endothelial cell labeling was only achieved in the PLP fixed tissue. In this fixative the angiosarcomas were factor VIII related antigen negative, Ulex europaeus lectin positive, laminin positive, unlabelled by the monoclonal antibody PAL-E, and positively labelled by the monoclonal antibody EN4. Ultrastructural examination of four cases showed evidence of vascular lumina in all tumours. Weibel-Palade bodies were seen in only one case but three tumours showed some evidence of tight junction formation and marginal folding. Thus, our cell marker studies can be interpreted as consistent with a lymphatic derivation for this type of angiosarcoma but in contra-distinction the ultrastructural studies showed tumour channels with features suggestive of blood vessel differentiation.
A case of giant pigmented tumour of the scalp which developed in a 47-year-old woman is reported. Macroscopically, the tumour showed a peculiar two-layered structure, consisting of an upper non-pigmented and a lower pigmented portion. Histologically, it was composed of elongated neurofibromatous tumour cells with abundant collagen fibres in the non-pigmented portion and round naevus-like cells with abundant melanin pigment in the pigmented portion. S-100 protein and neurone-specific enolase were demonstrated in most of the tumour cells, but neurofilament and myelin basic protein were not detected. Electron microscopy revealed melanosomes in the tumour cells of the pigmented portion. These findings might support a melanocytic origin for the tumour, but the lack of superficial pigmentation and the associated hair loss were against this. The tumour may represent an example of duality of neural crest differentiation.
A man developed acne keloidalis-like lesions in the scalp during treatment with diphenylhydantoin and carbamazepine for epilepsy. These drugs were suspected to play a role in the pathogenesis of this skin disease in an unusual location, based on clinical evidence and on the in vitro test, mast cell degranulation (MCD).
Angiosarcoma of the face and scalp developed in 12 patients. The patients were five women and seven men with an average age of 71 years. Initial features were solitary or multiple violaceous vascular nodules or plaques. The clinical course was complicated by ulceration, secondary infection, bleeding, anemia, infiltration into the underlying bones, tumor cachexia, and death. Metastases were not observed. Histologically, seemingly benign hemangiomatous capillary-like structures were found in some areas of the tumors, with richly cellular, solid sarcomatous proliferations in other areas. Early and extensive surgical excision is the therapy of choice, but generally it does not alter the relentless course of the disease. Neither palliative radiation therapy nor polychemotherapy is capable of interfering with tumor progression.