Raised limb bands developing in infancy

Congenital cutaneous constriction bands are rare and often occur with other abnormalities, including the presence of rudimentary digits. This diverse syndrome lacks a precise definition and a satisfactory explanation. We describe two unusual cases with features previously undescribed, in which predominantly raised, annular limb bands became apparent postnatally. One infant was also born with foreshortened digits and a constricting limb band, suggesting a shared aetiology with other congenital cases. The development of raised bands during infancy would be difficult to reconcile with the widely held 'amniotic band hypothesis', and would be more in keeping with a multifocal developmental abnormality in limb growth.     

Acquired port-wine stain occurring in association with a congenital lesion

Acquired port‐wine stains (PWSs) are rare but well recognized, and have often been reported in association with localized trauma. Various drugs have also been implicated as aetiological factors. We report the case of a 71‐year‐old man with an extensive congenital PWS affecting the left side of the face, upper chest and upper back. At the age of 69 years, he began to develop similar areas on his right hand and forearm, left hand and left knee. This corresponded temporally to being started on simvastatin and metformin, both of which may promote angiogenesis.     

Carotenemia in infancy and its association with prevalent feeding practices

Carotenemia in infancy is a relatively rare but benign condition, invariably of dietary origin, that can be confused with jaundice. It is characterized by an abnormal yellowish orange pigmentation of the skin, most prominently seen in the palms, soles, and naso-labial folds. Infant feeding patterns have shown an increasing trend toward the usage of homogenized and pureed vegetables as well as meat-based commercial preparations. Whether this is reflected in an increased incidence of carotenemia in this age group still remains unclear. We report a series of infants identified by a retrospective review of records, observed over a 3-year period (1999-2002) in a tertiary children's hospital, who developed the condition that resolved spontaneously without intervention, as they grew older, on a changing diet.     

Acquired Blaschko Dermatitis: Acquired Relapsing Self-Healing Blaschko Dermatitis

Acquired Blaschko dermatitis is a rare disease with acquired unilateral relapsing inflammatory linear lesions along Blaschko's lines. Histopathology reveals spongiotic dermatitis. “Blaschkite de l'adulte” and “acquired relapsing self-healing Blaschko dermatitis” have been suggested as names for this condition. Our patient was a 27-year-old man with a 6 month history of repeated, unilateral, slightly pruritic, discrete and grouped erythematous papules and papulovesicles on the left side of the upper limbs and trunk along Blaschko's lines. Histologic examination showed subacute spongiotic dermatitis. The condition showed excellent improvement after treatment with systemic corticosteroid for 2 months. Only a few cases have been reported. We propose a new designation and describe a patient who represents the first reported case of acquired Blaschko dermatitis in Korea.     

Psoriasis in infancy: therapy with calcipotriene ointment.

Psoriasis in infancy is often more therapeutically challenging than atopic and seborrheic dermatitis. The generalized nature of psoriasis and the intensity of inflammation often reduce the efficacy of topical corticosteroids. Furthermore, involvement of intertriginous skin and the presence of scalp disease limit the potency of the topical steroids that can be prescribed. We report on an infant treated with topical calcipotriene for infantile psoriasis who experienced greater benefit than he had with standard corticosteroid medications. Laboratory testing for calcium metabolism was normal during the course of therapy. We conclude that calcipotriene can be a safe and effective therapy for psoriasis in early infancy.     

Three cases of osteoma cutis occurring in infancy. A brief overview of osteoma cutis and its association with pseudo-pseudohypoparathyroidism

We report three cases of primary osteoma cutis in children, two of whom (siblings) were associated with Albright's hereditary osteodystrophy (AHO), manifesting as short stature with autosomal dominant inheritance from the father, but no dysmorphic features and no parathyroid hormone (PTH) resistance. Osteoma cutis can manifest as an isolated skin disease, a secondary condition to other skin diseases (such as acne), or in association with several syndromes, including AHO, which in turn may be associated with PTH resistance. The management and prognosis of patients diagnosed with osteoma cutis is determined by whether the skin manifestation has occurred in isolation, in association with a syndrome, or as a secondary skin disease. These three paediatric cases highlight the importance of understanding the aetiology and associations of osteoma cutis in order to appropriately investigate and manage patients who present with this rare skin disease.     

大疱性类天疱疮合并获得性血友病一例

获得性血友病A(acquired hemophilia A, AHA)死亡率为15%～42%,约50%左右的AHA为特发性,而另外50%与一些基础疾病有关,如系统性红斑狼疮、肿瘤、药物过敏、皮肤病(如银屑病,大疱性类天疱疮)等。本文报道一例大疱性类天疱疮合并获得性血友病,采用泼尼松、人凝血酶原复合物联合环孢素治疗后取得了显著疗效。     

Acquired and Inherited Porphyria:

Inheritance in 30 cases of porphyria cutanea tarda (PCT) and their relatives was investigated. Seventeen families were studied using the clinical criteria, quantitation and thin layer chromatography of urinary porphyrins. Thirteen families (13 propositus and 48 relatives) were investigated by using the above criteria and in vitro porphyrin biosynthesis by erythrocytes from δ-aminolevulinic acid. Three different types of PCT were identified: overt, subclinical and latent. Among 61 members examined, 13 had overt PCI. In six families, ten members had subclinical PCT and six latent PCT showing that in these six families PCT was a hereditary disorder. In seven other families inheritance could not be demonstrated.     

Acquired tufted angioma associated with pregnancy

Acquired tufted angioma is a distinctive condition that is different from other types of acquired vascular proliferation. Despite the progressive spread of these angiomas, they appear to be benign, and malignant change has not been encountered. We describe a case of recurrent acquired tufted angioma associated with pregnancy, an association which has not been previously recorded.