急性痘疮样苔藓样糠疹1例及文献复习

患者男,47岁,躯干、四肢皮肤见泛发粟粒至黄豆大红色斑丘疹20天,丘疹上覆少许稀薄鳞屑,Auspit征(-),部分皮损中央破溃,坏死、结痂。患者无发热、淋巴结及肝脾肿大。组织病理示:表皮呈灶性变性坏死,区域性淋巴浸润。诊断:急性痘疮样苔藓样糠疹。本文并就其病因、临床表现、病理进展和治疗进行了文献复习。     

Follicular Porokeratosis at alae Nasi; A Case Report and Short Review of Literature

Porokeratosis are disorders of keratinization, characterized histologically by a parakeratotic column, the cornoid lamellae, and clinically by a distinct peripheral ridge. Porokeratosis usually occurs on the trunk or extremities, and facial lesions are rare. Follicular involvement, identified by follicular localization of cornoid lamellae, is uncommon and described only in association with disseminated superficial actinic porokeratosis (DSAP) and porokeratosis Mibelli (PM). A 34-Year- old woman is presented with asymptomatic 1 cm plaque lesion with hyperkeratotic ridge and depressed center on her nose since three years. Histopathological examination of a biopsy revealed cornoid lamellae confined exclusively to the follicle.     

Linear Unilateral Basaloid Follicular Hamartoma Following Blaschko's Lines: Two Case Reports and Review of the Literature

Basaloid follicular hamartoma (BFH) is a rare follicular malformation characterized by variable clinical presentations and identical histopathologic features. We present the cases of a 3‐month‐old boy and an 8‐year‐old boy with linear unilateral BFH. To the best of our knowledge, only 14 cases of linear unilateral BFH have been described in the English‐language literature.     

Lichen Nitidus Presenting with Nail Changes—Case Report and Review of the Literature

Lichen nitidus of the nail is rare and can precede the onset of skin lesions. Delayed diagnosis is common. We present an unusual case of lichen nitidus–associated nail changes that preceded the onset of skin lesions in a 4‐year‐old Indian girl. We also conduct a review of six other cases of lichen nitidus with nail involvement from the English‐language literature. Clues to the diagnosis of lichen nitidus include violaceous or pigmentary changes of the nail fold and subtle lichenoid papules on the affected digits. Lichen nitidus of the nails appears to be less severe than nail changes of lichen planus and is generally self‐limiting. Understanding the natural history of lichen nitidus of the nails will help physicians better counsel patients and their families.     

Mucoceles of the Oral Cavity in Neonates and Infants—Report of a Case and Literature Review

Oral mucoceles are common lesions that normally appear in the second decade of life. They are uncommon in neonates and infants. We present a case of a mucocele in a 10‐month‐old infant and review the literature regarding the epidemiology and treatment in this age group.     

Dyschromatosis Universalis Hereditaria with Oral Leukokeratosis—A Case of Mistaken Identity and Review of the Literature

Dyschromatosis universalis hereditaria (DUH) is a rare pigmentary genodermatosis characterized by reticulated hyper‐ and hypopigmented macules distributed over the trunk and extremities in otherwise healthy patients. DUH presents in a fashion similar to that of a variety of reticulate and pigmentary dermatoses, some of which are associated with precancerous entities and other comorbidities. It is therefore imperative that the clinician recognize and differentiate these disorders so that appropriate screening and counseling can be offered to the patient. We report a case of DUH in a 13‐year‐old boy presenting with oral leukokeratosis, with a review of the literature exploring the differential diagnoses.     

A Case of Tumor of the Follicular Infundibulum with Sebaceous Differentiation

Tumor of the follicular infundibulum (TFI) is an uncommon benign adnexal tumor that usually presents as a solitary keratotic papule on the face or scalp of elderly patients. Histopathologically, it typically manifests as a plate-like fenestrated proliferation of monomorphic pale-staining cells. A 76-year-old male presented with about a 2 cm, well-defined, yellowish to brownish, slightly elevated, twisted and bent, interrupted, cord-like plaque on his left lower abdomen. Microscopic examination revealed a sharply demarcated plate-like proliferation of pale cells localized in the papillary dermis with multiple connections to the overlying epidermis. The histopathological features were compatible with TFI, except for foci of sebaceous differentiation. There has been one previous case report of TFI with sebaceous differentiation in the English medical literature. Herein, we report on a singular case of TFI with sebaceous differentiation.     

Phototherapy for Pityriasis Lichenoides in the Pediatric Population: A Review of the Published Literature

Background

Pityriasis lichenoides (PL) is a dermatologic disorder that manifests in either the acute (pityriasis lichenoides et varioliformis acuta) or the chronic form (pityriasis lichenoides chronica, also known as parapsoriasis chronica). Traditional first-line therapy consists of corticosteroids or antibiotics; however, these treatments are often accompanied with multiple side effects and may be ineffective.

Objective

The goal of this study was to review the use of phototherapy for treating PL in the pediatric population.

Materials and methods

We performed a systematic review of the literature in the National Library of Medicine’s PubMed database and the SCOPUS database discussing phototherapy for treatment of PL in the pediatric population. The following search terms were used: ‘pityriasis lichenoides’, ‘pityriasis lichenoides chronica’, ‘pityriasis lichenoides et varioliformis acuta’, and ‘febrile ulceronecrotic Mucha-Habermann disease’.

Results

The systematic search and screening of articles resulted in 14 articles including a total of 64 patients with PL treated with phototherapy. Three different modalities were utilized, with five studies using broadband ultraviolet B (BB-UVB) radiation, nine studies utilizing narrowband UVB (NB-UVB), and two studies employing psoralen with ultraviolet A (PUVA) therapy. Overall, the use of BB-UVB had an initial clearance rate of 89.6 % with 23.1 % recurrence, whereas NB-UVB cleared 73 % of the lesions with no recurrence, and PUVA therapy initially cleared 83 % of the lesions with 60 % recurrence. The side-effect profiles were similar and revealed limited toxicity.

Conclusion

Phototherapy shows promising results and a favorable side-effect profile in the treatment of PL. Ultimately, large randomized controlled trials are needed to determine optimal treatments.

     

Cowden Disease: Case Report and Review of the Literature

Cowden''s disease is a rare autosomal dominant, multiple hamartoma syndrome with characteristic mucocutaneous lesions. It is associated with abnormalities of the breast, thyroid, and gastrointestinal tract; and is characterized by multiple hamartomas in the gastrointestinal tract and mucocutaneous lesions such as trichilemmomas, oral papillomatosis, facial papules, and acral keratosis. A 21-year-old male patient presented with erythematous facial papules, oral mucosal papillomatosis, and punctate palmoplantar hyperkeratosis indicating a definite case of Cowden''s disease. This disease derives from variable expression resulting from a mutation in the PTEN gene. Gastrointestinal endoscopy and colonoscopy revealed multiple hamartomas in the stomach and colon. On thyroid ultrasonography, several probable benign nodules were noted in the right thyroid gland. He had no pertinent family history and no other systemic findings. Further regular laboratory and image studies will be planned for our patient, as well as his family members. Sporadic Cowden''s disease is rarely observed. Herein, we report a case of Cowden''s disease without known family history. Dermatologists should be aware of the possibility of Cowden syndrome based on its several dermatologic findings.     

Poikiloderma Congenitale: Case Report and Review of the Literature

Since birth an 8-year-old girl had a rash on both cheeks, consistent clinically and histologically with poikiloderma. She also had visual impairment to the extent of light perception only, due to microthalmia and dense scleralization of the corneas. These features were considered consistent with a diagnosis of Rothmund-Thomson syndrome.     

Subungual Exostosis: Case Report and Review of the Literature

Abstract: Subungual exostosis is typically a benign, acquired tumor of cartilaginous bone occurring on the medial surface of the distal hallux. A 16-year-old boy with subungual exostosis of his left great toe is presented. We evaluated the English language literature on this topic from 1857 to 1994; including our patient, 312 cases have been described. The diagnostic characteristics (histologic and radiologic), etiologic features, and therapeutic management were reviewed. Subungual exostosis more commonly affects women than men In a ratio of 2:1. Most lesions occur in the early twenties; however, a minimum of 51 (16%) of the 312 cases of subungual exostoses appeared in children 18 years of age or younger. Seventy percent of lesions occurred on the first hallux. There was frequently an association with trauma. The diagnosis of subungual exostosis may be suspected from the clinical presentation and confirmed with radiographic examination. The treatment is surgical.     

Encephalocraniocutaneous Lipomatosis: Case Report and Review of the Literature

Abstract: Encephalocraniocutaneous Hpomatosis is a congenital disorder characterized by unilateral cerebral malformations and ipsilateral scalp, face, and eye lesions. Distinguishing histopathologic features are dysgenesis and neoplasia of the adipose tissue. A Caucasian boy had soft tumors and elastic papules on his head since birth, associated with atrophic areas, and a bllobed lesion on the upper right eyelid. On the bulbar conjunctiva of the right eye, an oval 6-mm lesion was present. Ultrasonogram, computerized tomographic scan, and magnetic resonance imaging revealed a dilation of the right lateral ventriculus, a mass on the pontocerebellar angle, agenesia of the corpus callosum, an arachnoldal cyst on the right hemisphere, microcalcifications, and pachygyria. The histology of a soft cutaneous tumor was consistent with a fibrollpoma, and dispersed extracellular lipid globules in the upper dermis were found on electron microscopy. The diagnosis suggested by these findings was encephalocraniocutaneous lipomatosis. Even in view of the rarity of the syndrome (11 cases described in the literature), this patient seems unusual because of the bilateral distribution of the cutaneous lesions and because of the agenesia of the corpus callosum. The peculiar ultrastructural findings require further confirmation.     

Agminated Lentiginosis: Case Report and Review of the Literature

Abstract: Agminated lentiglnosis (AL) is characterized by numerous lentfgines confined to a body segment, with a sharp demarcation at the midline. So far, only 13 cases have been reported in the literature. We report a 30-year-old woman with a multifocal AL The cutaneous lesions first appeared at 5 years of age and gradually increased in number with time. Clinically, they appeared as numerous brown macules, ranging in size from 1 to 5 mm in diameter, in a peppered distribution over an area extending bilaterally on the neck, chin, and cheeks. On the trunk the lesions were localized to the left shoulder and breast, involving the axilla and upper part of the abdomen down to the umbilicus. Four cafe au lait macules ranging in diameter from 10 to 15 mm were present, with no evidence of neuroflbromas. Histopathology of a macute showed the features of lentigo; in addition, groupings of melanocytes were observed at the dermoepidermal junction. The differential diagnosis of AL includes speckled lentiginous nevus and segmental neurofibromatosis.     

Erythrokeratoderma Variabilis: Case Report and Review of the Literature

Abstract: A 5-month-old boy with erythrokeratoderma variabilis is presented. The parents noted that the asymptomatic erythematous plaques changed over the course of hours to days, Topical therapy with retinoic acid proved ineffectlve. A brief review of the literature is presented.     

Wegener's Granulomatosis: Case Report and Literature Review

Abstract: Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis usually affecting the upper and lower respiratory tracts and kidneys. Any organ system can be affected by the pathologic process, which remains an etiologic enigma. Limited forms of the disease are recognized in which few extrapulmonary and no renal lesions occur. Cutaneous manifestations occur in 40% to 50% of patients with WG (1). Early diagnosis is imperative because treatment regimens reduce morbidity and mortality in this potentially fatal disease. We report WG in a 10-year- old boy whose upper respiratory tract symptoms began at age 8 years. The case is illustrative of the difficulties that can be encountered in attempting to make this diagnosis. Skin is an easily accessible organ for biopsy, thus an increasing familiarity with the typical biopsy specimen findings within a consistent clinical setting may aid in earlier diagnosis of WG.     

Prepubertal Hidradenitis Suppurativa: Two Case Reports and Review of the Literature

Hidradenitis suppurativa (HS) is a chronic suppurative scarring disease of apocrine sweat gland-bearing skin in the axillary, anogenital, and, rarely, the breast and scalp regions. Females are more commonly affected than males and it is usually seen at puberty or later. We report two girls with prepubertal hidradenitis suppurativa whose initial presentation predated any signs of puberty. This early onset is very rare and its etiology remains unknown. Severe disease can be seen in prepubertal children and surgical intervention is effective in these cases.     

Nevus Comedonicus: Case Report and Brief Review of the Literature

Abstract: We report extensive nevus comedonicus in a female patient that involved half of her body entirely, with infected cystic lesions as well as typical scars, limited by the midline. The lesions worsened at the beginning of puberty. A brief review of the literature highlights the histopathologic, etiopathogenic, and therapeutic aspects.     

Congenital Glomangioma: Case Report and Review of the World Literature

Abstract: Glomangiomas, or multiple glomus tumors, occur in disseminated, localized, or congenital plaquelike forms. The first two cases of congenital plaquelike glomangioma were described in 1990. We report a 9-year-old girl with a congenital, violaceous, 75-cm² indurated plaque of the left abdomen that showed the classic histologic findings of glomangioma. In our review of the world literature, we found 11 additional, welldocumented cases of glomangioma present at birth. Ten of these patients had violaceous indurated plaques, and the other two had clusters of discrete nodules. The majority of lesions were painless and enlarged with body growth. Many patients developed satellite lesions at sites distant from the original glomangiomas later in life. Family history of glomangioma was positive in 4 of the 12 patients.     

Cockarde Nevus: A Case Report and Review of the Literature

Benign acquired melanocytic nevi usually display an even and uniform architecture and coloration easily recognized by the dermatologist. The cockarde nevus, a variant of the melanocytic nevus, is an exception. It displays a targetlike morphology with histologic characteristics of a compound nevus. We believe because the lesion has been rarely reported in the American literature, it is often mistaken in this country for a dysplastic nevus.     

Syringocystadenocarcinoma Papilliferum: A Case Report and Review of the Literature

Syringocystadenocarcinoma papilliferum (SCACP) is a rare malignant adnexal neoplasm, which is considered as a malignant counterpart of syringocystadenoma papilliferum (SCAP). Clinically, SCACP appears as a nodule, inflammatory plaque, or tumor. The lesion is usually covered with crusts, which are formed by secretion of the apocrine epithelial cells. Histologically, SCACP resembles SCAP, with cystic papillomatous invaginations connected to the skin surface by funnel-shaped structures lined by infundibular epithelium. The stroma of the tumor consists of a dense inflammatory infiltrate of plasma cells and lymphocytes. SCACP differs from SCAP in terms of the architectural and cytological features of the tumor cells, and is characterized by higher nuclear cytoplasmic ratio, nuclear irregularity, coarse chromatin, and increased mitotic activity. However, the immunohistochemical findings of SCACP vary. Since only 49 cases of SCACP have been reported in the English literature, the clinical and histologic characteristics of SCACP have not been fully established. Further studies on the diagnostic criteria for SCACP are warranted. Here, we report a rare case of SCACP and present a review of other relevant literature.