Atrophic Plaques Research Articles

Focal Dermal Hypoplasia Due to a Novel Mutation in a Boy with Klinefelter Syndrome

A boy was born with multiple anomalies, including right hemifacial microsomia, eye abnormalities, syndactyly, right hand ectrodactyly, hypoplastic nails, omphalocele, bladder exstrophy, renal dilatation, and splayed symphysis pubis. The skin was also abnormal, with atrophic skin plaques and areas of telangiectasia along the lines of Blaschko. The karyotype was 47,XXY (Klinefelter syndrome). He was found to have a heterozygous mutation in the PORCN gene. He exhibited the classical features of focal dermal hypoplasia. Fewer than 15% of reported cases are male when it is thought to be due to postzygotic mutation and thus mosaic. This is the first reported boy to have heterozygous mutation for Goltz syndrome who survived due to the extra X chromosome.

Fluoroscopy-Induced Chronic Radiation Dermatitis

A 62-year-old man with a history of 2 previous cardiac catheterizations presented with an itchy, nontender skin lesion over his right scapula. The skin lesion had been present for >5 years. Review of the medical records found evidence of a prolonged and complicated cardiac catheterization 8 years previously. Physical examination revealed an 8 × 6 cm, well-demarcated, erythematous reticulated atrophic plaque with telangiectasias and ulceration. Biopsy confirmed histologic changes consistent with radiation dermatitis. In conclusion, the characteristic histologic findings of radiation dermatitis, along with the location over the right scapula and the history of prolonged fluoroscopic exposure during cardiac catheterization, led to the clinical diagnosis of fluoroscopy-induced chronic radiation dermatitis.

Dermoscopy of atypical lichen sclerosus involving the tongue

Involvement of tongue during the course of lichen sclerosus is extremely rare, with only five - to our knowledge - described cases in the English literature. We present clinical and dermoscopic findings of a female patient who concomitantly developed genital, skin and oral lesions of lichen sclerosus. Tongue involvement consisted of a linear atrophic whitish plaque, strikingly mimicking "en coupe de sabre" scleroderma. Dermoscopic examination revealed a well demarcated, linear, dense, white, homogenous area, with papillae projections hardly seen in the affected area. Dermoscopy of lesions located on the trunk revealed whitish plaques with comedo-like openings in the center and an erythematous halo at the periphery. Since clinical differentiation of oral LS and other oral diseases appearing as white atrophic plaques is almost impossible without using histology, we believe that dermoscopy might represent an additional tool, contributing to the final diagnosis, and thus avoiding an ablative procedure at this site-sensitive localization.

Infiltrated leishmaniasis recidivans cutis on the face: a rare clinical presentation

Cutaneous leishmaniasis is a protozoan disease caused by Leishmania and transmitted by the bite of some species of sand flies. Usually it presents with variety of clinical manifestations depending on both the infecting species of Leishmania and the immune response of the host. Leishmaniasis recidivans cutis (LRC) is a unique form of cutaneous leishmaniasis characterized by unusual clinical features and its chronic relapsing nature. It is an evolving form of cutaneous leishmaniasis which clinically presents as a spreading of the initial nodule, leading to a plaque formation simulating discoid lupus erythematosus. A clinical course of leishmania recidivans is probably related to changes in cell-mediated immunity leading to localized or diffuse lesions. We report a case that presented with infiltrated, atrophic plaque on a patient's face. Clinically, the lesion resembled the lesion of discoid lupus erythematosus and lupus vulgaris but the cutaneous biopsy proved the diagnosis to be LRC.

Numerous Mucin Nodules in a Patient with Seropositive Wide Spread Discoid Lupus Erythematosus

Numerous Mucin Nodules in a Patient with Seropositive Wide Spread Discoid Lupus Erythematosus

Management of Congenital Midline Cervical Cleft

Congenital midline cervical cleft (CMCC) is a rare developmental defect of the anterior neck normally characterized by an atrophic mucosal plaque with a cranial nipple-like skin tag, a short caudal sinus, and may be attached to a subcutaneous fibrous cord of variable length. Clinically, patients present at an early age with, white females being the most commonly affected population. In addition to aesthetic concerns, CMCC can prevent full extension of the neck, result in micrognathia and torticollis, predispose patients to infection, and can coexist with other clefting defects or cysts. Fewer than 50 cases have been published in the English-language literature. Herein, we report a case of CMCC that also presented with a mild contracture of the right sternohyoid muscle. The embryopathogenesis, histopathology, diagnosis, and treatment of this rare condition are also discussed.

Lupus Erythematosus–Associated Primary and Secondary Anetoderma

Anetoderma (focal loss of dermal elastic tissue) can either be primary, which is an idiopathic occurrence of anetoderma in normal areas of the skin, or secondary, which is preceded by an inflammatory dermatosis in the same location. Sporadic reports of lupus erythematosus-associated anetoderma have been described in the literature. All reported cases were positive for antiphospholipid antibodies. We present a patient with primary and secondary anetoderma with chronic lupus dermatitis and negative antiphospholipid antibodies. A middle-aged woman presented with a soft nodule with a wrinkled surface on her left arm and an erythematous atrophic plaque with a nodular surface on the chest. Skin biopsy from the left arm showed epidermal atrophy without inflammatory changes. Histologic findings of the lesion on the chest were consistent with chronic lupus dermatitis and secondary anetoderma. Laboratory investigations showed positive antinuclear antibody anti-double-stranded deoxyribonucleic acid (DNA) antibody but negative antiphospholipid antibodies. Primary and secondary anetodermas may occur in patients of lupus dermatitis without positive antiphospholipid antibodies.

Syphilis of fungal world: Novel skin manifestations of histoplasmosis in an immunocompetent host

A case of chronic disseminated cutaneous histoplasmosis with unusual skin manifestations in an immunocompetent host is reported. Presence of cutaneous ulcers, linear erythematous plaques, skin coloured atrophic plaques and recurrent self-limiting oral ulcers in a single patient has not been documented in literature so far. Diagnosis was established by identifying small intracellular yeast-like cells of Histoplasma in tissue smear and skin biopsy. Leishman stained tissue smear proves to be an easy and simple procedure for diagnosis of histoplasmosis.

Multicentric dermatofibrosarcoma protuberans in patients with adenosine deaminase–deficient severe combined immune deficiency

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant skin tumor associated with a characteristic chromosomal translocation (t[17;22][q22;q13]) resulting in the COL1A1-platelet-derived growth factor β(PDGFB) fusion gene. This malignancy is rarely diagnosed in childhood. We observed an unexpected high incidence of this DFSP in children affected with adenosine deaminase-deficient severe combined immunodeficiency (ADA-SCID) and set out to evaluate the association of these 2 clinical entities. Twelve patients with ADA-SCID were evaluated with a complete dermatologic examination and skin biopsy when indicated. Conventional cytogenetic and molecular analyses (fluorescence in situ hybridization, RT-PCR, or both) were performed when possible. Eight patients were found to have DFSP. Six patients had multicentric involvement (4-15 lesions), primarily of the trunk and extremities. Most lesions presented as 2- to 15-mm, round atrophic plaques. Nodular lesions were present in 3 patients. In all cases CD34 expression was diffusely positive, and diagnosis was confirmed either by means of cytogenetic analysis, molecular testing, or both. The characteristic DFSP-associated translocation, t(17;22)(q22;q13), was identified in 6 patients; results of fluorescence in situ hybridization were positive for fusion of the COL1A1 and PDGFB loci in 7 patients; and RT-PCR showed the COL1A1-PDGFB fusion transcript in 6 patients. We describe a previously unrecognized association between ADA-SCID and DFSP with unique features, such as multicentricity and occurrence in early age. We hypothesize that the t(17;22)(q22;q13) translocation that results in dermal overexpression of PDGFB and favors the development of fibrotic tumors might arise because of the known DNA repair defect in patients with ADA-SCID. Although the natural course of DFSP in the setting of ADA-SCID is unknown, this observation should prompt regular screening for DFSP in patients with ADA-SCID.

Persistent Subcutaneous Nodule

An otherwise healthy 3-year-old Iraqi boy was referred to the Infectious Diseases Clinic for evaluation of a chronic cellulitis on the right temple, which was followed by development of right-sided preauricular adenopathy. Two months before presentation, the child sustained a minor abrasion to the right eyebrow. Cellulitis developed, which prompted a visit to his primary care physician who prescribed treatment with cephalexin for 2 weeks. On day 10 of cephalexin treatment, a small nodule was noted on the patient's right cheek in the preauricular area. His physician changed the therapy to amoxicillin for 2 weeks to treat both the cellulitis and the suspected adenopathy. When this antibiotic proved ineffective, further workup was initiated. Review of systems revealed no fever, cough, or weight loss. There was no significant medical history or family history of disease. The child was born in the United States; however, his parents were born in Iraq and moved to the United States 7 years ago. They reported no travel out of United States in the last 7 years. There were no pets in the home. Physical examination revealed an afebrile male with mildly tender, erythematous, 1.1 × 0.5 cm reticular atrophic plaque lateral to the right eyebrow, and a right preauricular 1-cm smooth, mobile, nontender, subcutaneous nodule. The remainder of his examination was normal. Laboratory tests revealed the diagnosis in this child. For denouement see p. 1126.

Comedogenic acne following radiation therapy

ejd.2011.1537 Auteur(s) : Evren Odyakmaz Demirsoy1 evrenodyakmaz@yahoo.com, Rebiay Kiran1, Burcu Ozturk1, Sevgiye Ozkara Kacar2, Aysun Sikar Akturk1 1 Department of Dermatology 2 Department of Pathology, Kocaeli University Medical Faculty Kocaeli, Turkey Different types of radiation may induce acneiform eruptions. Favre-Racouchot syndrome, which is characterized by yellow atrophic plaques with large comedones, wrinkles and furrows on sun-exposed areas, is the most commonly seen form of radiation-induced [...]

Lichen sclerosus in the genitourinary region

Lichen sclerosus (LS) is a chronic inflammatory disorder of the skin and mucosa, presenting to genitourinary physicians, pediatricians, and dermatologists. It affects both sexes with a lower incidence in men than in women. Autoimmune factors, infections, and genetic disposition are implicated in its pathogenesis. The classic clinical picture is of atrophic white plaques in the anogenital region. Pruritus, soreness, difficulty retracting the foreskin, and obstructive urinary symptoms are frequent complaints. Early histological confirmation by biopsy is recommended. The disorder is usually highly responsive to ultrapotent topical steroids. This may help arrest or delay the progressive nature of this disorder. Surgical treatment may be needed in cases of disease progression despite medical therapy. Circumcision is a successful treatment, if LS is limited to the glans and foreskin. In cases of meatal stenosis meatoplasty and in cases of urethral involvement one- or two-stage oral graft urethroplasty is necessary.

Hypocellular medallion-like dermal dendrocyte hamartoma (plaque-like CD34-positive dermal fibroma)

Medallion-like dermal dendrocyte hamartoma (MLDDH) is a recently described congenital dermal neoplasm. Only 11 cases have been reported in the English literature and therefore its clinical and pathological manifestations are not completely defined. We report the case of a 20-year-old male presenting with a round, erythematous, atrophic plaque on the midline of the anterior aspect of the neck. The lesion was asymptomatic and was stable since birth. A skin biopsy was performed. Histological examination showed a band like hypocellular fibrotic area in the superficial reticular dermis, which did not spread to subcutaneous tissue. The cells were CD34-positive and S100 and CD56-negative. Elastic fibers were present. Altogether the morphological and immunostaining features were neither suggestive of dermatofibrosarcoma protuberans nor neurofibroma. Thus, the pathological findings were consistent with MLDDH. Clinical differential diagnosis includes anetoderma, aplasia cutis, or atrophic DFSP. Histological differential was made with atrophic scar and striae distensae. Although the histological findings were not identical to those described recently as characteristic, the clinical features were suggestive enough to make the diagnosis of MLDDH. Therefore in our experience, the MLDDH spectrum might include lesions with variable cellular density, which can show similar clinical manifestations.

Fluoroscopy-induced chronic radiation dermatitis: a report of two additional cases and a brief review of the literature

Fluoroscopy-induced chronic radiation dermatitis (FICRD) can be a challenging diagnosis for the dermatopathologist, as often a history of radiation exposure is not provided. With the expanded use of minimally invasive procedures, there is increased fluoroscopic exposure and a high index of suspicion for FICRD is prudent. Recognition of characteristic clinical and histopathological features can be helpful in distinguishing chronic radiation dermatitis from entities in the histological differential diagnosis such as morphea and lichen sclerosus. We present a brief review of the literature as well as two additional cases of FICRD. Case 1 is a 44-year-old man with an atrophic lateral back patch for several years. Chart review revealed a history of cardiac radiofrequency ablation and congenital heart disease with correction. Case 2 is a 64-year-old woman with an ulcerated, atrophic left flank plaque, with a history of mesenteric artery angiography and stent placement. In our two cases, as well as the cases in the literature, a diagnosis of FICRD is associated with key features. In the evaluation of a sclerosing process, chronic radiation dermatitis should be suspected histologically by the findings of ulceration, prominent telangiectasias, atypical stellate fibroblasts, absence of a lymphocytic infiltrate/inflammation and/or presence of hyperkeratosis.

The many faces of necrobiosis lipoidica: a report of three cases with histologic variations

Necrobiosis lipoidica (NL) is a granulomatous disease with unknown etiology and pathogenesis. Clinically, it is characterized by yellow-brown atrophic plaques with inflammatory rims on shins. Histologically, it shows diffused palisade and interstitial granulomatous dermatitis with focal connective tissue degeneration (necrobiosis). We described three non-diabetic NL cases with unusual histologic features. All patients presented erythematous to brownish plaque(s) on the shin(s). In Case 1, in addition to the characteristics of NL, the biopsy showed dense perineural infiltration, which may explain the pathogenesis of the anesthesia of NL lesions. In Case 2, well-formed tuberculoid granulomas were of special interest. In Case 3, there was conspicuous lobular panniculitis. These histologic features present difficulties in the diagnosis and represent the broad spectrum of NL lesions, histologically.

A Case of Lichen Sclerosus et Atrophicus Accompanying Bullous Morphea

Bullous morphea is a rare form of morphea characterized with bullae on or around atrophic morphea plaques. Whereas lichen sclerosus et atrophicus (LSA) is a disease the etiology of which is not fully known, and which is characterized with sclerosis. Coexistence of morphea and LSA has been identified in some cases. Some authors believe that these two diseases are different manifestations which are on the same spectrum. The 70-year-old patient stated herein, presented to us for 6 months with annular, atrophic plaques, ivory color in the middle, surrounded by living erythema, on the front and back of the trunk. Occasionally bulla formation on the plaques on the trunk lateral was identified. Fibrotic and atrophic plaques of ligneous hardness were present on the front side of tibia of both legs. In the histopathologic examination, the lesions were found concordant with bullous morphea and LSA. With colchicine 1.5 mg/day, pentoxifylline 1,200 mg/day, topical calcipotriol ointment and clobetasol propionate cream, the erythema in the patient's lesions faded and softening in the fibrotic plaques was observed. Concomitance of bullous morphea and LSA is a rarely seen, interesting coexistence which suggests a common, as yet unknown, underlying pathogenesis.

Necrobiosis lipoidica of the scrotum

Auteur(s) : Yu SAWADA, Tomoko MORI, Daiki NAKASHIMA, Motonobu NAKAMURA, Yoshiki TOKURA long-ago@med.uoeh-u.ac.jp Department of Dermatology, University of Occupational and Environmental Health, 1-1, Iseigaoka Yahatanishi-ku, Kitakyushu, Japan Necrobiosis lipoidica (NL) is a distinctive chronic skin disease characterized clinically by yellowish telangiectatic, atrophic plaques with occasional ulceration. Histologically, it shows palisading granuloma surrounding a large foci of necrobiosis [1]. The [...]

Actinic Cheilitis: A Case Report and a Review of the Literature

In actinic cheilitis, the current view is that the keratinocytes have undergone transformation forming a field of epithelium with the potential for neoplastic transformation. Clinical features include diffuse and poorly demarcated atrophic, erosive or keratotic plaques that may affect some parts of, or the entire vermilion border. Fair-complexioned people, those with albinism and people with eversion of the lip are all subject to actinic cheilitis. Prophylactic measures against all forms of sunlight-induced lesions must include limitation of exposure to the sun during peak sunlight hours, the use of appropriate protective clothing, and the use of a sunscreen cream. In this article, a case of albinism is used to illustrate the nature of actinic cheilitis, its clinical features and its treatment.

Activation of the signaling cascade in response to T lymphocyte receptor stimulation and prostanoids in a case of cutaneous lupus

Context:Discoid lupus erythematosus is a chronic inflammatory skin disorder presenting with scarring lesions that occur predominately on sun exposed areas of the face and scalp.Case Report:A 22-year-old male was evaluated after presenting with reddish-purple, atrophic and erythematous plaques on the scalp, with loss of hair within the plaques. Biopsies for hematoxylin and eosin examination, direct immunofluorescence and immunohistochemistry analysis were performed. The hematoxylin and eosin staining demonstrated classic features of cutaneous lupus. Direct immunofluorescence revealed strong deposits of immunoglobulins IgG and IgM, fibrinogen and Complement/C3, present in 1) a shaggy pattern at the epidermal basement membrane zone, and 2) focal pericytoplasmic and perinuclear staining within epidermal keratynocytes. Immunohistochemisty staining revealed strongly positive staining with antibodies to cyclooxygenase-2, Zeta-chain-associated protein kinase 70, and HLA-DPDQDR in areas where the inflammatory infiltrate was predominant, as well as around dermal blood vessels and within the dermal extracellular matrix.Conclusions:Noting the autoimmune nature of lupus and its strong inflammatory component, we present a patient with active discoid lupus erythematosus and strong expression of Zeta-chain-associated protein kinase 70, cyclo-oxygenase-2, and HLA-DPDQDR in the inflammatory areas. We suggest that these molecules may play a significant role in the immune response of discoid cutaneous lupus, possibly including 1) the biosynthesis of the prostanoids and 2) activation of the signaling cascade in response to T-lymphocyte receptor stimulation.

Forthcoming Issues

Necrobiosis lipoidica (NL) is a rare chronic granulomatous disease that has historically been associated with diabetes mellitus. Debate exists regarding the etiology and pathogenesis of NL with a widely accepted theory that microangiopathy plays a significant role. NL typically presents clinically as erythematous papules on the front of the lower extremities that can coalesce to form atrophic telangiectatic plaques. NL is usually a clinical diagnosis, but if the clinical suspicion is uncertain, skin biopsy specimen can help differentiate it from sarcoidosis, necrobiotic xanthogranuloma, and granuloma annulare. NL is a difficult disease to manage despite a large armamentarium of treatment options that include topical and intralesional corticosteroids, immunomodulators, biologics, platelet inhibitors, phototherapy, and surgery. Randomized control trials are lacking to evaluate the many treatment methods and establish a standard regimen of care. Disease complications such as ulceration are common, and lesions should also be monitored for transition to squamous cell carcinoma, a less common sequelae.