Delayed diagnosis in a case of lupus vulgaris with unusual localization

Lupus vulgaris is the most common form of cutaneous tuberculosis, and the usual sites of involvement are the head and neck. We present a forty-six-year-old woman with lupus vulgaris on the external surface of the left leg and foot, an unusual site. Based on histopathological and clinical features, this case was diagnosed as lupus vulgaris with unusual localization.     

A case of lobular capillary hemangioma (pyogenic granuloma), localized to the subcutaneous tissue, and a review of the literature

Lobular capillary hemangioma (pyogenic granuloma) is a common vascular proliferation that typically occurs in the superficial dermis, although rarely a subcutaneous form has been reported. Although these lesions are considered benign, localized recurrence after excision and satellite spread of the lesions are known phenomena. A case of lobular capillary hemangioma with the unusual features of both subcutaneous localization and locally aggressive behavior following surgery in a patient with a history of estrogen use and local trauma prior to the onset of the lesion is presented. The literature of lobular capillary hemangioma is reviewed, and the differential diagnosis is discussed.     

Clinial picture and localization of skin metastasis of visceral carcinomas]

Seven cases with secondary skin carcinomas are described. The primary tumor was located at the tongue, the larynx, the thyroid, the abdomen, the ovaries, the collum uteri and the rectum. From the literature 60 other patients with secundary skin carcinomas and 100 primary carcinomas were evaluated. Clinical evidences for a secundary skin carcinoma are: unusual localization, multiple occurrence and an even surface. It was examined whether the localization of the secundary skin tumor refers to the localization of the primary tumor.     

Unilateral proteus syndrome

Proteus syndrome is a complex developmental abnormality. It is characterized by both hypertrophic and hypoplastic changes. Deformities have been occasionally found to be localized in one half of the body in head or digit but presence of all signs in one half of the body in a wide spread manner is not reported in the literature. We report the case for its unusual presentation of unilateral localization of signs.     

An unusual clinical presentation of lupus erythematosus tumidus localized on the thigh

A 44‐year‐old woman with seronegative polyarthritis presented with a 2‐year history of a solitary, bluish‐red, oedematous, nonscaly, annular and partially reticulated macule on her right thigh. Histopathological findings revealed perivascular and periadnexal lymphocytic infiltrate in the dermis. Alcian blue and colloidal iron stains highlighted mucinous deposit in the upper and mid dermis. Direct immunofluorescence showed a linear deposit of IgG and C3 along the basement membrane zone. Antinuclear antibody was positive at a titre of 1 : 80, with homogenous and speckled patterns. Except for its unusual localization and lack of photosensitivity, our case had the clinical and histopathological features of lupus erythematosus tumidus. These characteristics were also reminiscent of reticular erythematous mucinosis and erythema annulare centrifugum, both of which are considered to be associated with cutaneous lupus erythematosus (CLE). Hydroxychloroquine 200 mg daily led to improvement of the skin lesion. The unusual clinical presentation of our case emphasizes the heterogeneity of clinical manifestations of CLE.     

Localized peeling skin syndrome: case report with ultrastructural study

We report a young woman in whom the history, clinical features, histopathological and ultrastructural findings led to a diagnosis of peeling skin syndrome (PSS). PSS is a rare and not well classified genodermatosis, mainly characterized by the spontaneous separation of the stratum corneum from the stratum granulosum. The unusual feature in our patient was the strict localization to the palm. PSS has been described as a more generalized disease frequently sparing palms and soles. We propose the diagnosis label of ‘localized PSS’ for this previously undescribed variant of a rare keratinization defect.     

Buschke-Ollendorff Syndrome, Otosclerosis, and Congenital Spinal Stenosis

Abstract: We report a family with the Buschke-Oltendorlf syndrome and several unusual features, Including one individual wtth congenital spinal stenosis and another with hearing loss, probably due to otosclerosia. Other reported abnormalities associated with this syndrome, including otosclerosis, are reviewed.     

Palmoplantar lichen nitidus: a rare cause of palmoplantar hyperkeratosis

BACKGROUND: Lichen nitidus is a rare condition, which may be a cause of palmoplantar hyperkeratosis. We report two cases. CASE REPORTS: A 53 year-old woman presented with a dry and fissured palmoplantar hyperkeratosis. Histological examination of a biopsy showed the typical features of lichen nitidus. Significant improvement was obtained with acitretin. A few months later, multiple lichen nitidus papules appeared on the limbs and the abdomen. A 67 year-old woman was referred to us for a fissured, disabling palmoplantar hyperkeratosis refractory to topical steroids. Histological examination led to the diagnosis of lichen nitidus. Local PUVA therapy resulted in the cleaning of her lesions. Later, typical papules of lichen nitidus appeared on her elbows. DISCUSSION: Nineteen cases of palmoplantar localization of lichen nitidus have been described. The features are usually tiny yellow papules but sometimes a non-specific keratoderma resembling chronic eczema. Palmoplantar involvement of lichen nitidus may be isolated or associated with cutaneous lesions on unusual sites. Oral retinoids and local PUVA are effective treatments.     

The baboon syndrome or intertriginous drug eruption: a report of eleven cases and a second look at its pathomechanism

Although drug eruptions can mimic a variety of idiopathic skin diseases, this has not been mentioned in the differential diagnosis of intertrigo. We draw attention to an unusual presentation of a drug eruption with a characteristic distribution pattern that is confined to the intertriginous areas. This condition has been given one of the most memorable names in dermatology, the baboon syndrome. Originally, the baboon syndrome was described as a special form of systemic contact-type dermatitis (SCTD) that occurs after ingestion or systemic absorption of a contact allergen in individuals previously sensitized by topical exposure to the same allergen in the same areas. We present eleven cases of intertriginous eruptions that resulted from adverse drug reactions. A flare-up of a previous contact with the same allergen (i.e., drug) on the same areas is not a reasonable explanation for the unusual localization of the eruption in our and others' cases. We believe that we are dealing with a type of recall phenomenon and that the characteristic localization and appearance of the eruption is determined by an earlier, unrelated dermatitis that had occurred in precisely the same areas. Adverse drug reactions should always be considered in the differential diagnosis of intertrigo, especially in atypical and therapy-resistant cases.     

Unique aspects of a lesion of erythema elevatum diutinum

A 70-year-old man with erythema elevatum diutinum developed new lesions on his right hand 4 years after initiation of treatment with dapsone. An unusual protruberant appearance of these lesions grossly and a peculiar fibrosing quality histopathologically of one lesion are features that, to our knowledge, have not been reported previously.     

Malignant melanoma with osteoclast-like giant cells: an unusual host response: immunohistochemical and ultrastructural study of three cases and literature review

Melanomas with unusual histologic features are very rarely reported in the literature and demonstrate the diversity of melanocytic expression. Three cases of malignant melanoma with osteoclast-like giant cells are reported. Two cases showed undifferentiated malignant cells without melanin pigment and one showed spindled cell morphology. Immunohistochemistry showed that the osteoclast- like giant cells expressed CD68, but not melanocytic markers (HMB45, Melan-A, and S100). Ultrastructural analysis further supports that these cells are reactive histiocytes rather than transformed malignant cells. This suggests they represent an unusual host response, similar to those rarely observed in other neoplasms. Awareness of this entity is important to avoid misdiagnosis of melanoma as a histiocytic tumor. Since only few cases have been reported, greater recognition and documentation may help to evaluate the prognosis of such cases with unusual morphology.     

Localized multiple glomus tumors of the face and oral mucosa

We describe a 54-year-old patient with an unusual localization of localized multiple glomus tumors. She had several nodules on the left mandibular area, lower lip, and anterior part of the buccal mucosa. A biopsy taken from the left mandibular area showed cystically dilated vessels lined by endothelial cells and a few outer layers of glomus cells in the dermis to subcutis. Localized multiple glomus tumors are rare, and both their facial and oral mucocal localization are extremely rare.     

Merkel cell carcinoma in organ-transplant recipients: report of two cases with unusual histological features and literature review

BACKGROUND: Non-melanoma skin cancers are the commonest malignancies after organ transplantation and are often associated with human papillomavirus (HPV). Merkel cell carcinoma is an uncommon neuroendocrine skin tumor, of which 67 cases have been reported up till now, usually briefly, in organ transplant patients. METHODS: Among a cohort of 2340 organ-transplant recipients, two patients (one renal, one heart) developed cutaneous Merkel cell carcinomas 5 and 12 years of post graft, respectively. These were studied histologically and immunohistochemically, as well as virologically for the presence of HPV. A thorough literature review of all reported cases of Merkel cell carcinoma following solid organ transplantation was performed. RESULTS: Despite a typical immunophenotype, the tumors showed unusual histological features: both were epidermotropic, and one was intermingled with a bowenoid squamous cell carcinoma. Search for HPV by immunohistochemistry and PCR proved negative in both cases. CONCLUSION: In the setting of organ transplantation, Merkel cell carcinoma is much rarer than other non melanoma skin cancers but may show unusual histologic features. HPV do not seem to be involved in its pathogenesis.     

Angiosarcomas in chronically lymphedematous extremities. Two cases of Stewart-Treves syndrome

Two cases of Stewart-Treves syndrome are presented. The first case consists of an angiosarcoma developing in long-standing postmastectomy lymphedema. The second case has an unusual localization on the lower extremity. The results of immunohistochemical stainings with Factor VIII-related antigen, UEA-1 lectin, and laminin support the assumption that Stewart-Treves syndrome is a hemangioendotheliosarcoma.     

Atopic dermatitis and pox-virus superinfections (author's transl)

Subjects afflicted with atopic dermatitis are particularly predisposed to such viral superinfections as verruca, herpes (eczema herpeticum) and vaccinia (eczema vaccinatum). Knowledge of superinfection by the molluscum contagiosum virus is more recent and the authors describe one case. The orf virus (responsible for ovine ecthyma contagiosum), however, has never been described as responsible for the profuse superinfection of an atopic eczema. The authors observed one case of this in a child, atypical as result of his age (16 months), the cervical localization and the aspect, which was a telangiectasic granuloma type of the initial element, and finally of the satellization observed one week later. This child suffered from a generalized atopic eczema. Propagation of the orf lesions could have been favored by scratching, local corticoid treatment and by the deficit in cellular immunity observed in patient with atopic dermatitis. Indeed, viral superinfections, which are unusual by their profusion or their chronic nature, are often observed in immunodepressed subjects.     

Syringomas localized to the penis (case report)

Syringomas localized in the genital areas are unusual. There are only three cases reported describing syringomas on the penis. Owing to this atypical localization it is necessary to consider a differential diagnosis involving other papular lesions more frequent found on the penile skin.     

Giant fibrokeratoma of the heel

We describe a 77-year-old patient with a giant acquired fibrokeratoma on the heel. The size and the localization of the tumor was unusual. Simple shave excision was curative.     

Epidermotropic secondary cutaneous involvement by relapsed angioimmunoblastic T‐cell lymphoma mimicking mycosis fungoides: a case report

Angioimmunoblastic T‐cell lymphoma (AITL) is frequently associated with skin lesions, but epidermotropic cutaneous involvement has never been described. A 37‐year‐old man presented with erythematous and pruriginous plaques, clinically suggestive of mycosis fungoides, distributed all over the body, 3 weeks after the last line of a polychemotherapy, given for an AITL diagnosed 1 year earlier on a lymph node biopsy. Skin biopsy showed an epidermotropic CD4⁺ T‐cell lymphoma, so that a diagnosis of mycosis fungoides was first proposed. Further investigations showed that atypical lymphocytes strongly expressed CD10 and markers of follicular helper T cells (T_FH) including PD1, BCL‐6 and CXCL13. The diagnosis of an unusual epidermotropic cutaneous localization of the AITL was finally made, supported by the presence of the same T‐cell clone in the initial lymph node biopsy and the skin. We therefore recommend performing markers of T_FH cells in patients with unusual epidermotropic cutaneous T‐cell lymphomas, particularly if they have any clinical features suggestive of AITL.     

Cutaneous histiocytic lymphangitis: an unusual manifestation of rheumatoid arthritis

Two cases are presented of unusual cutaneous lesions associated with rheumatoid arthritis in underlying joints. The lesions were evanescent, erythematous and violaceous partly macular and partly indurated plaques, with a livedo-like pattern of erythema at the edge in one case. Histological changes were identical in the two cases. The major features were dilated, dermal lymphatics containing aggregates of inflammatory cells, mainly histiocytes, with adjacent perivascular lymphoid aggregates. An appropriate name for this reaction would appear to be cutaneous histiocytic lymphangitis.     

Unusual features of primary dermatofibrosarcoma protuberans and its myxoid recurrence

A case of Dermatofibrosarcoma Protuberans (DFSP) with unusual clinical and histological features is presented. The lesion recurred one year after surgical removal and showed myxoid degeneration of the entire lesion. Only a few cases with myxoid degeneration of the entire lesion are described in the literature. We found CD 34 stain, in combination with a panel of antibodies including fXIIIa, S100 and actin, useful in the diagnosis of this type of tumor in order to differentiate it from other neural, fibrous or muscular neoplasms.