Unusual forms of alopecia areata in a Trichology Unit.

BACKGROUND: Alopecia areata (AA) occurs with the apparition of asyntomatic non inflamatory alopecia plaques without scars. We distinguish several variants which are divided into two groups: typical forms (AA in single or multiple plaques) and atypical forms (by its presentation, evolution or paradoxical regrowth). OBJETIVES AND METHODS: We describe the cases of AA treated in our Trichology Unit between January 2000 and December 2011. RESULTS: We obtained 488 cases of AA. 114 (23.36%) were unusual form of AA or had paradoxical regrowth. The most common unusual form of AA was sisaipho type (7.37%), followed by AA for black and blonde hair (5.32%), atypical diffuse forms (4.30%), androgenetic alopecia type and (3.89%) and AA rectangular occipital (0.68%). Furthermore, we found nine cases of paradoxical regrowth (1.84%). CONCLUSIONS: Atypical variants of AA in our series are less than 25% of all cases, although it should be noted that since it is a specialized unit, we may be making a selection bias to be more difficult to diagnose cases or poor outcome.

Three cases of Lipschutz vulval ulceration.

A Lipschütz ulcer or 'ulcus vulvae acutum' is an acute simple ulceration of the vulva or vagina of non-venereal origin which can be associated with lymphadenopathy. Three cases are described with accompanying clinical photographs. Two cases refer to adolescents, one an infant, all without any history of sexual contact. The cases serve to illustrate a little known but potentially important differential diagnosis of vulval ulceration.

Latanoprost in the treatment of eyelash alopecia in alopecia areata universalis.

OBJECTIVES: The aim of this study was to test the efficacy of latanoprost in eyelash alopecia areata (AA). DESIGN: This study is a 2-year prospective, non-blinded, non-randomized, bilateral eyelash alopecia controlled study. SETTING: The setting of this study was Trichology Unit, Virgen Macarena University Hospital, Seville, Spain. PATIENTS: We conducted a survey of 54 subjects with AA universalis treated with the protocol of the Trichology Unit of our Department. Control group comprised 10 subjects who received injections of 0.5 mg/cm(2) of triamcinolone acetonide (TAC) in their eyebrows and 1 mg/cm(2) of TAC injections in affected scalp. The treatment group included 44 subjects who received the same treatment as the control group in scalp and eyebrows but they also applied a drop of latanoprost 0.005% (50 microg/mL) ophthalmic solution in their eyelid margins every night. Subjects were reviewed every 3 months for 2 years. RESULTS: Forty subjects finished the study and four subjects were lost to follow-up. In the treatment arm of this study, the course was well tolerated and uncomplicated. Both investigators and patients evaluated the regrowth. The results we obtained were: complete regrowth in 17.5%, moderate regrowth in 27.5%, slight regrowth in 30% and without response in 25%. Moderate and total regrowth constituted a cosmetically acceptable response. The therapy was continuous and the response remained without any side effects. No patients had cosmetically acceptable eyelash regrowth in the control group. CONCLUSIONS: Latanoprost may be an effective drug in the treatment of eyelash AA because it induces acceptable responses (total and moderate) in 45% of the patients. A formal, blinded prospective unilateral controlled study will permit further understanding about this promising therapeutic agent for eyelash AA.

[Neurocutaneous syndrome with hair alterations]. / Síndromes neurocutáneos con alteraciones del pelo.

There are multiple neurocutaneous syndromes that may show hair alterations such as the interglabellar peak or 'widow's peak', which is an alteration of the hair implantation, in addition to the genohypotrichosis, hypertrichosis and hair shaft dysplasias. In this chapter we will focus on the latter. Out of the unspecific hair shaft dysplasias the only ones showing neurological alterations are trichorrhexis invaginata, observed in the syndrome of Netherton. Among the specific dysplasias we would like to point out monilethrix, and very especially the moniliform hair syndrome, the trichorrhexis nodosa, the pili torti and trichotiodystrophy. The latter is actually a group of syndromes which associates a series of diverse symptoms that have in common hair brittleness, fertility problems and physical and mental retardation, and they constitute the basic syndrome know as 'BIDS syndrome.

[Incontinentia pigmenti. Study of 7 cases, 2 in men (author's transl)]. / Incontinentia pigmenti. Etude de 7 cas dont 2 mâles.

We describe 7 observations of incontinentia pigmenti (0.0026 p. 100 of the patients visited in the Department of Medical-Surgical Dermatology, Faculty of Medicine, University of Granada). Two cases with congenital pigmentation and one of these with secondary inflammatory lesions. The electron microscopy shows relation between the classic stages of the process. In two patients there were defects of the Central Nervous System: brain paralysis and microcephaly. Only two patients with blood eosinophilia (13 p. 100-21 p. 100). Evolution benign, except in the case with brain paralysis. There were familiar antecedents in three women and these were not found in two women and two men with normal karyotype. It suggests a mutation. This study has shown a high rate men/women: 2/5; in comparison to other series. We think that it is possible for a mutation and that this disease is more frequent than it seems. It is necessary to think in this disease in all pigmentary abnormalities in children.

[Incontinentia pigmenti. Study of 3 families]. / Incontinentia pigmenti. Etude de trois familles.

Ten cases of incontinentia pigmenti are reported. All patients, corresponding to three families, were female. In one case a Turner's syndrome phenotype (XO) with mosaicism 46 XX/46 X; i (Xq) was observed. This finding seems to confirm that the disease is transmitted through a dominant gene carried on the X chromosome. Dental alterations were the most frequent of associated abnormalities, being present in all 10 patients. We regard these abnormalities as important as they permit a retrospective diagnosis in adult patients after the skin lesions have disappeared. EEG alterations (essentially an increase in the slow component) were also observed in the majority of cases.

[Epidermal nevus syndrome (of Solomon, Fretzin and Dewald)]. / Syndrome du naevus épidermique (de Solomon, Fretzin et Dewald).

Epidermal naevus syndrome is an entity which has been well established by Solomon, Fretzin and Dewald since 1968, presenting cutaneous, visceral, ocular, osseous and neurological malformations. We report a case with multiple osseous manifestations affecting the entire skeleton, but mainly the skull (osteolysis with absence of frontal bone), the vertebral column (scoliosis with triple deviation), the upper right limb (demineralisation and multiple pathological fractures) and lower members (fractures of the right and left femur) with enlargement of the entire ventricular system and well marked scissures, signs of an cortico-subcortical atrophy shown by scanogram.

[Alagille's syndrome. Clinical and histo-pathologic study]. / Le syndrome D'alagille. Etude clinique et histo-pathologique.

Alagille 's syndrome, described in 1970, associates a chronic intrahepatic cholestasis (hypoplasia of the interlobulary biliary ducts), vertebral and visceral malformations, retarded physical, mental and sexual development and neonatal jaundice. A series of cutaneous manifestations are reviewed in their clinical, histological and ultrastructural aspects. Its relation with the zinc deficiency syndrome is discussed.

[Behçet's syndrome. Apropos of 12 cases]. / Síndrome de Behçet. A propósito de doce observaciones.

Twelve observations of Behcet's syndrome are described, which comply with the diagnostic criteria of O'DUFFY. The clinic symptomatology is reviewed and compared with those found by other authors.

[Bullous pemphigoid in a 5-month-old infant]. / Penfigoide ampolloso en un lactante de cinco meses.

After observing a case of pemphigoid in a 5-month-old infant, verified inmunologically, we comment on the clinical, histological, biochemical and immunological tests and review the benign chronic bullous dermatosis of the childhood.

[Pseudoxanthoma elasticum. Apropos of 7 cases]. / Pseudoxantoma elástico. A propósito de siete observaciones.

Seven cases of P X E, four of them seen in the last six months in our clinic, are revised in the Department of Dermatology Medical-Surgical and Venereology of Sevilla (Faculty of Medicine), realising clinical, histological and ultrastructural studies.

[Clinical and epidemiological study of boutonneuse fever in Andalusia: apropos of 36 cases]. / Contribución al estudio clínico y epidemiológico de la fiebre botonosa en Andalucía: a propósito de 36 casos.

Boutonneuse fever is a rare disease, it accounts for 0.091% of all the patients who consult the Department of Medical-Surgical Dermatology in the Faculty of Medicine of Seville. It is clearly a disease of seasonal incidence, appearing most frequently during the summer, which affects both sexes equally at middle age, and is most frequent in rural areas, although it is increasing in urban districts. The term "Mediterranean fever" is correct, understanding that it is a disease afflicting Mediterranean countries, but has nothing to do with proximity to the sea.