Neonatal haemangiomatosis in identical twins with twin-twin transfusion syndrome

INTRODUCTION: Benign natal haemangiomatosis is characterised by the presence of multiple congenital haemangiomas restricted to the skin. It is differentiated from diffuse neonatal haemangiomatosis in which there is both cutaneous and visceral involvement, with higher morbidity and mortality. PATIENTS AND METHODS: Two identical twins, I and II (monochorionic placenta, biamniotic), born prematurely at 30 weeks' amenorrhoea, presented twin-transfusion syndrome resulting in retarded intrauterine growth in twin I, the donor, and incipient anasarca in twin II, the recipient. Twin I weighed 960 g while twin II weighed 1 200 g. At birth, miliary haemangiomatosis was observed in both infants (16 haemangiomas in I, 19 in II). Abdominal ultrasound and whole-body MRI performed in the two children revealed multiple angiomatous hepatic nodular lesions in I. Subsequent routine clinical and ultrasound monitoring (hepatic and cardiac) showed increased size of the haemangiomatous lesions over the first 4 months followed by stabilisation and gradual regression. No systemic therapy was required. In twin I an episode of ulceration of a neck haemangioma occurred at 5 months and a favourable outcome was obtained on administration of topical hydrocolloid therapy. DISCUSSION: Twin-transfusion syndrome affects 15 to 30% of monochorionic biamniotic pregnancies. It is a serious complication of twin pregnancies resulting from a dynamic process of interfoetal blood transfusion as a result of venous-venous or arteriovenous vascular anastomoses. In the present case, which appears to be the first reported case, it seems that these monochorionic twins, who shared the same placenta, presented haemangiomatosis simultaneously in utero, if we accept the hypothesis of grafting of emboli of placental microvessels in the formation of congenital haemangiomas.     

Multifocal haemangioma with extracutaneous involvement associated with hypergalactosaemia

Neonatal haemangiomatosis, characterized by multiple haemangiomas, is a rare disease that develops during the neonatal period with or without visceral involvement. We report a 1‐month‐old Japanese boy with multifocal haemangiomas with extracutaneous involvement. A haemangioma on his left lower eyelid, present at birth, increased in size during the first postnatal month and more lesions developed during the same period. Neonatal mass screening showed hypergalactosaemia. Laboratory investigations found raised total bile acid and ammonia. Computed tomography and abdominal ultrasonography studies showed multiple hepatic haemangiomas and intrahepatic portovenous shunts. The child’s cutaneous and hepatic haemangiomas disappeared spontaneously with normalization of laboratory data, and galactose accumulation improved with the feeding of lactose‐free milk. There were no complications and the child has had no recurrence of the symptoms. Our case implies a possible association of multiple haemangioma and hypergalactosaemia, suggesting the necessity for visceral investigation.     

A retrospective randomly selected cohort study of D-penicillamine treatment in rapidly progressive diffuse cutaneous systemic sclerosis of recent onset

BACKGROUND: Several uncontrolled studies in systemic sclerosis have shown that D-penicillamine may cause improvement in skin sclerosis, decrease the rate of new visceral organ involvement, and improve overall survival. OBJECTIVES: To undertake a single-centre retrospective randomly selected cohort study to examine the effects of D-penicillamine treatment on skin and visceral organ involvement in patients with rapidly progressive systemic sclerosis of recent onset. METHODS: Eighty-four patients with diffuse cutaneous systemic sclerosis who had received D-penicillamine within 24 months of clinically detectable onset of skin sclerosis were randomly selected from the systemic sclerosis cohort followed at the Scleroderma Center of Thomas Jefferson University. Employing a previously described severity scale, disease severity and skin involvement were compared from initiation of D-penicillamine to end of study and a correlated matched t-test was used to establish statistical significance. RESULTS: At a mean+/-SD duration of D-penicillamine therapy of 29.2+/-5.5 months and at a median dose of 750 mg per day statistically significant improvement in skin (P<0.01) and cardiac, pulmonary and renal involvement (P<0.05) was observed. At last follow-up, 17 (20%) patients were still receiving D-penicillamine, 25 (30%) had discontinued it owing to disease improvement, and 18 (21%) had discontinued it owing to side-effects. CONCLUSIONS: In a population of patients with diffuse cutaneous systemic sclerosis, with progressive disease of recent onset, D-penicillamine treatment at a median dose of 750 mg per day caused a statistically significant reduction in skin involvement and improvement of renal, cardiac and pulmonary involvement.     

Absent/reduced glucose transporter-1 protein expression in infantile subglottic haemangiomas

BACKGROUND: Positive immunohistochemical staining for glucose transporter-1 protein (GLUT1) is a characteristic of cutaneous infantile haemangiomas. OBJECTIVES: To examine GLUT1 expression in subglottic haemangiomas. METHODS: Review of clinical notes and biopsy tissue with immunostaining for GLUT1 in 14 patients with subglottic haemangiomas. RESULTS: GLUT1 immunostaining was negative in 11 cases, and focally positive in three. No subglottic haemangiomas demonstrated the intense diffuse positive GLUT1 staining seen in cutaneous infantile haemangiomas. Five patients had cutaneous as well as subglottic haemangiomas, one of whom had a GLUT1-negative subglottic haemangioma and a GLUT1-positive cutaneous haemangioma of the lip. CONCLUSIONS: Subglottic haemangiomas appear immunohistochemically different from cutaneous infantile haemangiomas, which may reflect differences in endothelial cell differentiation or underlying aetiology.     

Cutaneous involvement in Hodgkin's lymphoma: report of two cases

Hodgkin's lymphoma (HL) comprises 20 to 30% of all lymphomas. Skin involvement is almost always secondary to visceral or nodal involvement. Secondary cutaneous HL is rare, occurring in only 0.5 to 3.4% of the cases. Herein we report two cases of skin involvement in Hodgkin's disease. One was a 25-year-old female admitted with a draining sinus in the neck. The other was a 19-year-old female admitted with a neck mass and skin papules on her chest and arm. Skin involvement may antecede or can be seen during the course of HL. Better understanding of such cutaneous involvement, which occasionally may be the initial sign of HL, is needed, and a biopsy of any suspicious skin lesion should be considered.     

Multiple cutaneous reticulohistiocytosis

BACKGROUND: Multicentric reticulohistiocytosis is a non Langerhans cell histiocytosis. This rare disease is characterized by cutaneous papules and nodules and a destructive polyarthritis; multisystem involvement may occur. Multiple and diffuse cutaneous reticulohistiocytosis have been more rarely reported. We present a case which was distinctive by the existence of multiple cutaneous plaques. CASE REPORT: A 65-year-old woman presented cutaneous papules and nodules associated with a destructive arthritis affecting the hands. Histological examination of a cutaneous biopsy associated with immunophenotyping and electronic microscopy permitted us to make the diagnosis of multicentric reticulohistiocytosis. The search for visceral involvement or underlying neoplasia was negative. Rapidly, cutaneous aggravation occurred with multiple and diffuse infiltrated plaques on the back, the face, the ears, the thighs and the forearms. The same histological aspect was found for these lesions. Treatment with corticosteroids and cyclophosphamide was successful. DISCUSSION: This case report is the first one with diffuse cutaneous lesions of multicentric reticulohistiocytosis with aspect of infiltrated plaques. Diffuse cutaneous lesions in multicentric reticulohistiocytosis have been rarely reported with diffuse papules or nodules pattern. A visceral involvement seems to be more frequent for diffuse cutaneous involvement. In all cases, the association of multicentric reticulohistiocytosis with neoplasia in up to 25 p. 100 is of interest. Treatment of multicentric reticulohistiocytosis consists in corticosteroids at the initial phase associated with alkylants agents or methotrexate.     

Pemphigus vulgaris in Iran: a clinical study of 140 cases

BACKGROUND: Pemphigus vulgaris (PV) is a rare autoimmune blistering disease of the skin and mucous membranes. It varies in its clinical profile and epidemiologic characteristics in different parts of the world. OBJECTIVE: To determine the clinical features of PV in Iran in a prospective manner. METHODS: The study included 140 patients with newly registered PV attending our dermatology clinic between January 2003 and June 2004. RESULTS The mean age at the onset of the disease was 41.5 +/- 15.7 years, with a female to male ratio of 1.59 : 1. At presentation to our clinic, both skin and mucosal involvement was observed in 95 patients (67.9%). Cutaneous lesions without the involvement of the mucous membranes were seen in nine patients (6.4%), and exclusive mucosal involvement was present in 36 patients (25.7%). The most common initial localization of the disease was the oral cavity, which was involved in 93 patients (77.5%). The most frequent cutaneous and mucosal sites involved were the thorax and oral cavity, respectively. CONCLUSION: Although minor differences were noted, the results of this study are in relatively good agreement with the literature with regard to the age, gender, and initial presentation of PV in Iran. Some skin sites, such as the scalp, thorax, and axilla, may be more commonly affected in men. Patients with initial mucosal lesions were significantly younger than those with initial cutaneous involvement. Mucosal lesions other than the oral mucosa may be more common than previously thought.     

Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis

BACKGROUND: Multiple hemangiomas of the skin have traditionally been recognized as a clue to potential visceral hemangiomas. Recently, hemangiomas have been recognized to have subcategories, localized and segmental, which correlate with risk of complications. While less common, segmental hemangiomas of the skin have a higher risk of being life- or function-threatening and/or having associated structural anomalies such as those that occur in PHACE (posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities) syndrome (PHACES, if sternal clefting/supraumbilical raphe is included). However, the potential association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis has not been previously emphasized. OBSERVATIONS: A total of 47 cases of segmental hemangiomas of the skin in association with visceral hemangiomatosis were found. The location of the cutaneous hemangiomas most commonly, but not exclusively, involved the face (37 cases [79%]). The most common site of internal organ involvement was the liver (20 cases [43%]), followed by the gastrointestinal tract (16 [34%]), brain (16 [34%]), mediastinum (9 [19%]), and lung (7 [15%]). The percentages of reported cases of hemangiomas of the pancreas, spleen, bones, or kidneys were 6% or less. Forty percent of patients met criteria for the diagnosis of PHACE(S) syndrome. In this subgroup, internal organ hemangiomas were most commonly found in the brain or mediastinum (18 cases [53%]). Overall, 12 patients (25%) died during infancy, most commonly because of gastrointestinal involvement or congestive heart failure secondary to liver involvement.Conclusion Segmental hemangiomas of the skin have an associated risk of visceral hemangiomatosis, with the potential of causing vital organ compromise.     

The lupus band test in oral mucosa, conjunctiva and skin

The prevalence and clinical significance of subepithelial immunoglobulin and complement deposition (the lupus band) were examined in the uninvolved sun-protected skin of the forearm, the uninvolved sun-protected lip mucosa and sun-protected bulbar conjunctival mucosa in systemic lupus erythematosus (SLE) and chronic cutaneous lupus erythematosus (CCLE). In SLE, linear deposition of an immunoreactant at the BMZ was detected in 32% (6/19) of skin biopsies; 21% (4/19) of lip mucosal biopsies and 42% (5/12) of conjunctival biopsies. There was no significant difference in the sensitivity of the test at different sites in SLE and no correlation between a positive test in skin, lip or conjunctiva and clinical mucosal involvement. In CCLE, linear deposition of an immunoreactant at the BMZ was found in 3% (1/32) of skin biopsies; 3% (1/29) of lip mucosal biopsies and 50% (10/20) of conjunctiva and clinical mucosal involvement. In the conjunctiva, IgG was present in all but one of the biopsies and was the only immunoreactant in 90% (9/10) of positive CCLE biopsies and 60% (3/5) of positive SLE biopsies. In lupus erythematosus immunoreactants may be deposited in the basement membrane zone beneath non-keratinizing mucosal surfaces of the lip and the eye as well as the skin. In CCLE, the test may be positive in conjunctiva when skin and lip are negative.     

Cutaneous polyarteritis nodosa: a clinical and histopathological study of 20 cases

Twenty cases diagnosed as cutaneous polyarteritis nodosa (CPN) and confirmed by skin biopsy over the last 17 years were reviewed in our department. Based upon their clinical features, laboratory findings, and long-term observation of the disease course, they were divided into three groups. 1) Group 1 comprised 16 cases which were classified as the mild cutaneous form. The disease was confined to the skin with occasional involvement of peripheral nerves and skeletal muscles of the affected extremity. They generally followed a benign course. 2) Group 2 comprised 2 cases classified as the severe form. Despite severe clinical manifestations and several abnormal laboratory findings, the disease was limited to the skin, muscles, and peripheral nerves without any visceral involvement over follow-up periods of 11 years and 5 years, respectively. 3) Group 3 comprised 2 cases of the progressive form; in these the disease had begun with a cutaneous lesion and progressed to the systemic form after 19 and 18 year periods of recurrent episodes of cutaneous lesions, respectively. One died of gastrointestinal bleeding. In group 3, serum antinuclear antibodies and rheumatoid factor were positive. The autoimmune mechanism seems to play a role in this group. It is clear from the results of this study that not all patients whose vasculitic lesions are apparently limited to the skin remain in a benign course. Long-term follow-up is essential.     

Histologic features of cutaneous sinus histiocytosis (Rosai-Dorfman disease): study of cases both with and without systemic involvement

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a systemic proliferation of cells that resemble the sinus histiocytes of lymph nodes. Whereas initial reports highlighted the often striking cervical adenopathy, more than 40% of patients have extranodal involvement. Cutaneous lesions are the most common form of extranodal disease, but cases that present as cutaneous disease without lymph node involvement are rare. We examined biopsies from three patients with cutaneous lesions of sinus histiocytosis, none of whom had detectable systemic involvement, and compared them to those of two patients whose lymph nodes were involved by the disease. The histology of skin lesions in all five cases showed dense nodular or diffuse infiltrates of "histiocytes," emperipolesis of lymphocytes, neutrophils, and plasma cells. "Histiocytes" were present in lumens of dilated lymphatics. At the peripheries of the lesions were lymphoid aggregates and thick-walled vessels surrounded by plasma cells. Staining with anti-S-100 protein antibody showed marked cytoplasmic positivity in the "histiocytes" in each case. The only features that differed among the two groups were the presence of pseudoepitheliomatous hyperplasia and infiltrates of eosinophils in one case in which the disease was limited to the skin. We believe that cutaneous sinus histiocytosis can be specifically diagnosed by skin biopsy. Because cutaneous sinus histiocytosis may be unfamiliar to general pathologists, dermatopathologists, and dermatologists, cases limited to the skin may be under-recognized.     

The Efficacy of Topical 0.1% Adapalene Gel for Use in the Treatment of Childhood Acanthosis Nigricans: A Pilot Study

Background:

Vitiligo is one of the disorder that has social impact. Both skin and mucous membrane show depigmentation in vitiligo. Depigmentation in oral cavity can be more easily observed and the patient can be given awareness regarding the condition if they are unaware of vitiligo elsewhere in their body and can be guided for treatment.

Aim and objectives:

The aim of this study is to determine the frequency of occurrence of oral mucosal vitiligo in vitiligo patients and to determine the most commonly involved oral mucosal site.

Materials and methods:

The study sample included 100 vitiligo patients. The patients of all age groups and both genders were included. Vitiligo patients associated with systemic conditions such as thyroid disorders, juvenile diabetes mellitus, pernicious anemia, Addison''s disease were excluded in this study.

Results:

Out of 100 vitiligo patients 44 % male and 56% were female. The oral presentation of vitiligo in this study showed depigmentation of buccal mucosa in 5% of patients, labial mucosa in 5% of patients, palate in 8% of patients, gingiva in 2% of patients and alveolar mucosa 1%. Depigmentation of lip was seen in 42% of patients. Lip involvement refers to depigmentation of both the lips or either lip. Also vermilion border involvement was noted in majority of cases. In some cases, the depigmentation of lip extended to the facial skin also.

Conclusion:

In this study 55 patients out of 100 patients showed depigmentation in the oral cavity. Lip involvement was most common in this study showing about 42% of patients. Intraoral mucosal involvement was found in 21% of patients. Among intraoral mucosal site palate was common followed by buccal and labial mucosa, gingiva. Two patients had lip pigmentation as the only manifestation without any depigmentation in the skin.     

Diffuse infantile hepatic hemangiomas: a report of four cases successfully managed with medical therapy

We report four cases of diffuse infantile hepatic hemangioma, a rare but potentially life-threatening subset of hepatic hemangiomas. All patients demonstrated distinctive dome-shaped red-purple cutaneous hemangiomas. Two patients responded to steroids and propranolol (one in combination with vincristine), and two responded to steroids and vincristine. After a systematic literature review, we identified 26 previously reported cases of diffuse infantile hepatic hemangioma. Diffuse infantile hepatic hemangioma had a mortality rate of 17% and a >70% incidence of hypothyroidism, often severe (n = 30). More than one-third of patients developed heart failure (high output in half the cases). Based on our experience, early aggressive medical management, as well as thyroid replacement when indicated, should be initiated early in the course of diffuse infantile hepatic hemangioma pending evaluation for liver transplant.     

The successful treatment of diffuse neonatal hemangiomatosis with flashlamp pulsed dye laser

Diffuse neonatal hemangiomatosis is a rare disease with the distinctive features of multiple hemangiomas of the skin and visceral organs. These lesions have been treated with systemic corticosteroids, interferon-alpha, and their combination. We report a patient with diffuse neonatal hemangiomatosis who had multiple cutaneous and hepatic hemangiomas. Single therapy with the flashlamp pulsed dye laser was effective for cutaneous hemangiomas, whereas the hemangiomas of the liver remained stable and no liver dysfunction or hemorrhage has occurred so far, even with no treatment.     

Preliminary study of analyzing mucosal human papillomaviruses in cutaneous warts by restriction fragment mass polymorphism

Cutaneous human papillomavirus (HPV) types 1, 2, 4, 7 and 57 are reportedly found in cutaneous warts. However, there are few reports that have investigated the prevalence of mucosal HPV types in cutaneous warts. The aim of this study is to investigate the prevalence of mucosal HPV types in patients with cutaneous warts and to determine any association between HPV types and patient characteristics. We analyzed 62 wart samples that were taken from patients who were diagnosed with cutaneous warts, and 30 normal skin samples were used as negative control. We recorded the following characteristics: sex, age, type of warts, duration of warts, number of warts and patient's immune status. A matrix‐assisted laser desorption ionization time‐of‐flight (MALDI‐TOF) mass spectrometry (MS)‐based restriction fragment mass polymorphism (RFMP) assay was used for HPV genotyping. Of the total 62 wart samples, 50 samples (81.6%) were positive for HPV genotypes. All of the negative controls (30 samples) using normal skin showed negative reaction. Mucosal HPV types (49 samples, 84.4%) were highly detected, and high‐ or probable high‐risk HPV types (39 samples, 67.2%) were more common than lower risk HPV types (10 samples, 17.2%). A statistically significant association was observed between sex, age, duration of warts and the risk of HPV types. To the best of our knowledge, this is the first study to use the RFMP assay to analyze cutaneous wart‐associated mucosal HPV types. The high prevalence of high‐risk and probable high‐risk HPV in this study is of great significance.     

Kindler'S syndrome: a case series of three Indian children

Kindler's syndrome is a very rare genodermatosis characterized by acral blistering starting in infancy, photosensitivity, progressive poikiloderma, cutaneous atrophy, and various forms of mucosal involvement. A large number of other cutaneous and extracutaneous features have also been described. We report here three cases of Kindler's syndrome from eastern India for the rarity of the syndrome and to emphasize the importance of considering this condition in the differential diagnosis of disorders that can cause blistering, cutaneous atrophy, and/or poikilodermatous skin changes.     

Primary Cutaneous Diffuse Large B-Cell Lymphoma of the Upper Limb: A Fascinating Entity

Primary cutaneous lymphomas are defined as lymphoid neoplasms that present themselves clinically on the skin and do not have extra-cutaneous disease, when the diagnosis is made or even after 6 months of the diagnosis. Primary cutaneous lymphomas of B-cells are less frequent than lymphomas of T-cells. Primary B-cell lymphomas have a better prognosis than secondary B-cell lymphomas. Primary B-cell cutaneous lymphomas are classified into five types according to the World Health Organization and European Organization for Research and Treatment of Cancer classification. The primary diffuse large B-cell cutaneous lymphoma – leg type corresponds to approximately 5-10% of the B-cell cutaneous lymphomas. It is predominantly seen in elderly people and has a female preponderance. Skin lesions can be single, multiple, and even grouped. A 5-year survival rate ranges from 36 to 100% of the cases. The expression of Bcl-2, presence of multiple lesions, and involvement of both the upper limbs lead to a worse prognosis. Very few cases have been described in the literature.     

SKIN DISEASES ASSOCIATED WITH RENAL TRANSPLANTATION

A retrospective analysis was undertaken of skin lesions occurring in 100 renal transplant patients. Post-transplant problems were largely iatrogenic and the result of immunosuppression. Prominent among these findings were Cushing's syndrome (56%), and a wide range of skin and mucosal infectious including moniliasis (67%). Staphylococcal infection (45%), and herpes simplex (39%). Many of these lesions were more persistent and widespread than usual, of bizarre appearance, or frankly opportunistic. Five post-transplant patients developed multiple squamous malignancies. Other skin changes appeared to be associated with chronic renal failure, prolonged haemodialysis, drug reactions, or cutaneous manifestations of systemic disease with renal involvement.     

Disseminated lupus vulgaris

Lupus vulgaris is the most common form of cutaneous tuberculosis, and usually presents as a solitary lesion on the face. We report two patients with multiple lesions on different skin areas. The first patient presented a diffuse involvement of the right foot, and reddish-brown plaques on the right leg, the back and the face. Spreading of the lesions followed a prolonged application of topical corticosteroids. The second patient showed a large plaque on the nape and occipital area resulting in scarring alopecia, and plaques on the right inguinal and thigh regions. Ziehl-Neelsen staining was negative in both cases, but diagnosis was supported by histology and polymerase chain reaction analysis. No visceral involvement was present. Antituberculosis polychemotherapy was rapidly effective.