1/23. Dermopathic changes in hypozincemia.In seven patients with chronic debilitating gastrointestinal disease who were being given total parenteral nutrition using L-amino acids and concentrated dextrose solution, severe hypozincemia (less than 60 micrograms/dL) developed with characteristic skin lesions and alopecia. The lesions were similar to seborrheic dermatitis, and they occurred in areas of high sebaceous gland concentration. Hyperkeratotic lesions were also seen on the extensor surfaces of large joints. The skin lesions and alopecia disappeared when serum zinc values were raised to levels above 60 micrograms/dL, with appropriate zinc sulfate replacement. A hypothesis on the etiology and clinical manifestations is presented.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/23. Coexistence of common variable immunodeficiency and autoimmune polyglandular syndrome type 2.A six year-old boy with common variable immunodeficiency developed insulin dependent diabetes mellitus, autoimmune thyroiditis, and total alopecia leading to the diagnosis of autoimmune polyglandular syndrome type 2. Previously unreported co-occurence of these two entities may be explained by strong autoimmunity and HLA association of both conditions.- - - - - - - - - - ranking = 5keywords = gland (Clic here for more details about this article) |
3/23. Lichen planopilaris.P, a 20-year-old laborer displayed initial symptoms of the disease in question when he was 10 years old. Initially he had an asymptomatic progressive loss of hair on the scalp. A couple of years later he had mild to moderate pruritis, and the appearance of slate-blue eruptions on the scalp and elsewhere on the body. This resulted in a complete loss of hair on the vault of the scalp, which led him to seek specialist opinion. Skin surface examination revealed the presence of grayish-blue acuminate follicular papules, disposed singly and in groups (plaques). The pilo-sebaceous orifices were conspicuously obliterated and filled by keratin plugs. Perifollicular erythema was a predominant feature on the scalp. The lesions were present over the scalp, around the neck, chest, back, axillae, groin and legs. Shiny atrophied scalp skin depicting scarring alopecia mimicking male-type baldness was a salient feature. In addition, it was studded with conspicuous acuminate papules in its center (Fig. 1a). The known nonhairy (glabrous) skin had classic lichen planus lesions (Fig. 1b). Hemotoxylin-eosin stained microsections prepared from typical lichen planus (LP) lesions over the abdomen and those of lichen planopilaris (LPP) of the scalp were simultaneously studied. The former revealed changes in the epidermis comprising of hyperkeratosis, increase in thickness of stratum granulosum, hydropic degeneration of the basal cell layer and band-like lympho-histiocytic infiltrate pressing against and invading the epidermis, while the latter revealed uniform atrophy of the epidermis and vacuolization of basal cells. The hair follicles were dilated and were filled with keratin plugs. In addition to fibrosis of the dermis, pigment laden microphages and lympho-histiocytic infiltrate was prominent. The follicles and the sebaceous glands were absent. However, arrectores pilorum and sweat glands were preserved (Fig. 2a,b).- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
4/23. ichthyosis follicularis: a case report and review of the literature.ichthyosis follicularis (IF) is a very rare neurocutaneous, X-linked recessive condition affecting the skin, hair, eyes, and central nervous system (CNS). This report describes a child with facial dysmorphism, mental retardation, psychomotor delay, congenital alopecia of the scalp, eyebrows, and eyelashes, and extensive spiny follicular papules. A skin biopsy specimen showed the characteristic absence of sebaceous glands. We also reviewed the literature on this very rare entity. Additional findings observed in our patient, including hepatosplenomegaly, undescended testicles, and ptosis, have not been reported before.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
5/23. Polyglandular autoimmune syndrome type 1 without chronic mucocutaneous candidiasis in a 16 year-old male.A 16 year-old boy presented with adrenal crisis and was incidentally found to have features of latent tetany. Also detected were Hashimoto's thyroiditis, alopecia and subnormal T cell function, and he was diagnosed as having polyglandular autoimmune syndrome type 1 (PGA-1), although chronic mucocutaneous candidiasis, a hallmark of PGA-1, was absent. The presentation of several components of this disorder at one time and at this age is uncommon, and the features of overt hypoparathyroidism were probably masked by associated adrenal insufficiency.- - - - - - - - - - ranking = 5keywords = gland (Clic here for more details about this article) |
6/23. Erythrodermic syringotropic cutaneous T-cell lymphoma.Syringotropic cutaneous T-cell lymphoma (CTCL) is a rare localized variant of CTCL, characterized histologically by eccrine gland and ductal hyperplasia surrounded by a dense syringotropic lymphocytic infiltrate. Previously reported only in men, we describe the first woman with syringotropic CTCL. Unusually, she presented with erythroderma, cutaneous nodules, poikilodermatous patches, widespread alopecia and lymphadenopathy.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
7/23. central nervous system involvement in autoimmune polyglandular syndrome.We present a 33 year-old man, admitted because of transient deterioration of visual acuity. magnetic resonance imaging showed diffuse central nervous system (CNS) demyelination, which largely resolved spontaneously within 4 months. The patient fulfilled the diagnostic criteria of APS type III, having autoimmune thyroiditis and alopecia universalis. In this patient, autoimmune activation against CNS antigens is thought to be the cause of reversible CNS demyelination.- - - - - - - - - - ranking = 4keywords = gland (Clic here for more details about this article) |
8/23. A rare case of autoimmune polyglandular syndrome type 3.A 57-year-old female was admitted to our hospital suffering from a lower lip tumor, small ulcers in the arms and alopecia of the head. Because she had type 2 diabetes mellitus (DM) for the past 3 years, she was referred to our department of internal medicine for its treatment. Her endogenous insulin secretion was much decreased despite the short duration of diabetes. glutamic acid decarboxylase antibodies (GADA) and islet cell antibodies (ICA) were both positive. Therefore, she was diagnosed as having slowly progressive form of type 1 DM. Type 1 DM is sometimes complicated with autoimmune disorders. After further examinations, she was diagnosed as having sjogren's syndrome, Graves' disease and autoimmune neutropenia (AIN). According to the histological examinations of the lip tumor and peripheral site of the skin ulcer, the patient was diagnosed as having carcinoma spinocellulare and chronic cutaneous lupus erythematosus. The examination also showed positive anti-intrinsic factor and anti-ribonucleoprotein (RNP) antibodies. She is a rare case of an autoimmune polyglandullar syndrome (APS) type 3 simultaneously manifesting these seven diseases with multiple autoimmune antibodies.- - - - - - - - - - ranking = 5keywords = gland (Clic here for more details about this article) |
9/23. Three successive generations of women with anhidrotic/hypohidrotic ectodermal dysplasia.We describe the findings of anhidrotic/hypohidrotic ectodermal dysplasia in three successive generations of a family. All three women had variable alopecia, anhidrosis, hypodontia and malar hypoplasia. Chromosomal studies revealed a defect of the 2q12 region in all three patients. Previous studies have reported rare cases of autosomal dominant ectodermal dysplasia associated with defects in the 2q11-13 region1. These rare disorders are characterized by common anomalies of at least two elements of the ectoderm and its appendages--namely, the skin, teeth, hair, nails and sweat glands. These patients also frequently have chronic dental problems with early loss of teeth and recurrent lung, ear and nose infections secondary to a defect in mucous membrane function. The majority of reported cases of ectodermal dysplasias have historically been X-linked recessive, but our findings indicate that an autosomal version may be more prevalent than previously thought.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
10/23. Further delineation of the ichthyosis follicularis, atrichia, and photophobia syndrome.We describe an 18-month-old male infant suffering from the ichthyosis follicularis, atrichia, and photophobia (IFAP) syndrome and further delineate the clinical phenotype. Severe retardation of growth and psychomotor development, chill-like seizures, bronchial asthma, urticaria, a proneness to skin infections and transient nail dystrophy observed in our patient are non-obligatory manifestations of this disorder. Histological examination of the atrichia revealed poorly developed, shortened hair follicles and a complete absence of sebaceous glands. The sex ratio of published cases suggests an X-linked recessive inheritance. The marked clinical variability of the IFAP syndrome might be the expression of a contiguous gene defect.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
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