Progeria in siblings: a rare case report

Progeria, also known as Hutchinson-Gilford syndrome, is an extremely rare, severe genetic condition wherein symptoms resembling aspects of aging are manifested at an early age. It is an autosomal dominant disorder. It is not seen in siblings of affected children although there are very few case reports of progeria affecting more than one child in a family. Here we are presenting two siblings, a 14-year-old male and a 13-year-old female with features of progeria, suggesting a possible autosomal recessive inheritance.     

Zosteriform relapse of B-cell lymphoma

Infiltrating lipoma is a rare variety of lipoma, characterized by an infiltration of the adipose tissue of the muscles. Infiltrating lipomas are usually classified in two groups: intermuscular infiltrating lipoma and intramuscular infiltrating lipoma. Most are acquired, and they usually appear in middle-aged individuals. Exceptionally, they are congenital. In such cases they are not related to other diseases. We report an 8-year-old boy with a congenital infiltrating lipoma of the upper limb and von Willebrand disease. Both diseases are linked to an alteration in chromosome 12, but this clinical association seems to be random rather than causal.     

Phototoxicity and photoallergy in man

Photosensitivity is the broad term used to denote adverse reactions to the sun. These reactions may be phototoxic or photoallergic. Phototoxic reactions are much more common than photoallergic reactions. Phototoxic reactions may be photodynamic or nonphotodynamic in nature, the primary difference being that photodynamic reactions require oxygen. Porphyrins are examples of molecules which produce photodynamic responses. The 8-methoxypsoralen photobinding to DNA is an example of a nonphotodynamic reaction. The UVB sunburn response is the most common phototoxic reaction, and it appears to be nonphotodynamic in nature. Photoallergy is uncommon. As with all allergies it is an acquired, altered reactivity dependent on an antigen-antibody or cell-mediated hypersensitivity response. Solar urticaria due to UVB rays is an example of a circulating antibody-dependent reaction, and allergic photocontact dermatitis induced by the halogenated salicylanilides is an example of a cell-mediated response. The mechanisms of these reactions are discussed.     

Digital acrometastasis

Cutaneous metastases are infrequent and in some cases represent the first manifestation of an unknown neoplasm. Acrally located metastases are particularly rare, and the prognosis is very poor, with a survival time of only a few months. Although the clinical presentation varies, they are generally confused with an infectious or inflammatory process, which delays diagnosis. When they are located on the fingers, the most frequent cause is lung carcinoma, while those located on the toes are usually due to tumors in the genitourinary tract. The histology of the metastasis is similar to that of the primary tumor, although less differentiated. In most acrometastases, first the bone and then the skin are affected. Treatment is palliative. We present the case of a patient with a metastasis on the big toe as an initial sign of a previously unknown lung tumor.     

Dermal melanocytosis: A clinical spectrum

While most dermal melanocytoses are congenital or have an onset in early childhood, there is a group which is clearly acquired, with an onset in adult life. While the Mongolian spot typically disappears in childhood, other dermal melanocytoses persist for life. A brief review of the clinical spectrum of the dermal melanocytoses is undertaken and three illustrative cases are described: a case of congenital naevus of Ota, a case of acquired bilateral naevus of Ota-like macules, and an unusual case of a congenital dermal melaaocytotic lesion on the left hand which began to spread in adulthood. The possibilities regarding the pathogenesis of this intriguing group of disorders are considered.     

Airborne contact dermatitis from n-alkyl dimethylbenzylammonium chloride and n-alkyl dimethylethyl-benzylammonium chloride in a detergent

Occupational airborne contact dermatitis from disinfectants are not frequent. They are usually seen in hospitals, were the exposure is more common. We report a case of a young woman, with an intense eczematous eruption that developed hours after an accidental contact with a cleaning solution.     

Pustular allergic contact dermatitis to isoconazole nitrate

The topical imidazole antimycotics are widely used and are an infrequent cause of contact allergy. We report on a woman, who developed an unusual clinical picture of allergic contact dermatitis, namely papulopustular reaction, evoked by an isoconazole nitrate-containing cream. The histopathologic changes included subcorneal pustules, spongiosis, and an inflammatory infiltrate composed mainly of lymphocytes and some eosinophils and neutrophils. The patient demonstrated a severe vesicular reaction to isoconzole nitrate as is and at an imidazoles concentration of 0.5%. No cross-reactivity with other imidazole antimycotics was noted.     

Cheyletiella dermatitis: a report of fourteen cases

Cheyletiella dermatitis is an infrequently reported eruption caused by an ectoparasite whose normal hosts are household pets. Fourteen cases, documented over an eight-year period, are reported. All cases were found in one practice in a small community. Typical patients are female, aged forty years or younger, who experience pruritic papules in the winter months. Cheyletiella dermatitis is not a rare problem.     

Phacomatosis pigmentokeratotica associated with a suprasellar dermoid cyst and leg hypertrophy

Phacomatosis pigmentokeratotica (PP) is a mosaic disorder that represents a distinct epidermal naevus syndrome. Its defining features are an epidermal naevus that is usually of the sebaceous type and a speckled lentiginous naevus arranged in a chequerboard pattern. In addition, there are neurological, ophthalmological and skeletal abnormalities, including limb hemiatrophy with muscular weakness, ptosis, seizures and ipsilateral segmental hyperaesthesia and hyperhidrosis. We report a 44-year-old man with an extensive epidermal naevus and an ipsilateral speckled lentiginous naevus. He also had ipsilateral right leg hypertrophy and a suprasellar dermoid cyst with associated neurological abnormalities. We propose that this case represents an unusual example of PP.     

The value of the intersystem model for cosmetic nursing practice

Conceptual nursing models are a framework for practice. However, these models are often not used in an outpatient dermatology clinic that provides cosmetic services or a plastic surgeon's office. In a cosmetic setting, clients are receiving care on an elective basis. Clients are generally healthy and educated as to what type of care and services they want, and they wish to be active participants in the decision-making process. Therefore, this type of atmosphere requires a model that allows for negotiation of ideas and a joint plan of care, such as the Intersystem Model.     

Bullous secondary syphilis

An earlier standard syphilology textbook states that 'If vesicles are an essential part of an eruption in an adult, the lesions are not due to secondary syphilis'. However, vesicular and bullous eruptions do occur in congenital syphilis and rare reports of both vesicular and pustular eruptions in adults with secondary syphilis have been published. We describe a patient with a bullous-pemphigoid-like eruption and a positive VDRL in whom treatment with a course of procaine penicillin resulted in rapid permanent resolution of the eruption.     

Cutaneous ganglioneuroma: A case report and discussion of the literature

Ganglioneuromas (GNs) are benign tumors composed of ganglion cells in a Schwannian stroma. They are derived from neural crest cells that give rise to the sympathetic nervous system. Hence, GNs can be found anywhere a sympathetic ganglion is present. Most commonly, GNs are found in the posterior mediastinum and abdominal cavity. Within the abdominal cavity, they are most likely to be found in the retroperitoneal space or adrenal glands. Cutaneous involvement is uncommon and rarely reported in literature. We report an interesting case of a cutaneous ganglioneuroma on the abdomen of an 83‐year‐old male.     

Bulimia and anorexia nervosa: cutaneous manifestations

Bulimia and anorexia nervosa (BN, AN) are considered psychiatric disorders that have physical complications. Several factors may play a role in the onset of AN and BN, including a familial predisposition to these disorders, as well as individual personality characteristics. Dissatisfaction with body shape and an overwhelming desire to be thin are considered as risk factors for the development of eating disorders. Skin changes are characteristic and are cardinal diagnostic symptoms and pointers to the diagnosis of eating disorders. They are a consequence of starvation and/or malnutrition, self-induced vomiting, drug consumption and concomitant psychiatric illness. A careful examination of the skin can suggest a diagnosis of eating disorder. Cosmetic dermatologists, whose patients are mostly women, have a unique opportunity to detect these signs. This is very important as an early diagnosis will influence prognosis.     

Eosinophilic annular erythema treated with dupilumab

Eosinophilic annular erythema is a rare, benign, recurrent condition characterized by annular skin lesions, tissue eosinophilia, and resistance to a variety of treatments. There are fewer than 30 cases reported in the English literature, 7 of which are in children. We present a case of recurrent eosinophilic annular erythema in an adolescent that was successfully treated with dupilumab, an interleukin‐4 receptor alpha antagonist.     

A collection of rare anomalies: multiple digital glomuvenous malformations,epidermal naevus,temporal alopecia,heterochromia and abdominal lipoblastoma

Glomuvenous malformations are a subtype of venous malformations, which present in infancy or childhood. We describe a teenage girl who presented with multiple digital glomuvenous malformations from birth. In addition, she had an epidermal naevus on the upper lip, an area of congenital alopecia of the scalp, heterochromia irides and an abdominal lipoblastoma. We are unaware of any reports of the association of multiple glomuvenous malformations with the other uncommon developmental anomalies seen in our patient, and a common link eludes us.     

Angiomatoid and desmoplastic Spitz nevus presenting as a keloidal nodule

Angiomatoid and desmoplastic Spitz nevi are rare histologic variants of Spitz nevi that present most frequently on the extremities of children and young adults. Although Spitz nevi are clinically heterogeneous, one presenting as a keloidal nodule has not been previously published. We present a case of an angiomatoid and desmoplastic Spitz nevus clinically akin to a keloid on an African‐American teenager and describe its unique histopathologic features.     

Association between Olfactory Receptors and Skin Physiology

Olfactory receptors are chemosensory receptors that detect odorants and function in the initial perception of a smell. Intriguingly, olfactory receptors are also expressed in cells other than olfaction sensory cells, an expression pattern termed ectopic expression. Ectopically expressed olfactory receptors have a distinct role depending on the type of tissues or cells in which they are expressed. This review introduces current research on the ectopic expression and function of olfactory receptors in skin and provides insight into directions for future research.     

Atypical cellular neurothekeoma: A potential diagnostic pitfall for benign and malignant spindle cell lesions in skin

Cellular neurothekeomas (CNTs) are rare, benign cutaneous tumors that arise primarily on the head and neck, with a slight female predominance. CNTs with atypical features have been described, including those with an infiltrative growth pattern. Although CNTs with atypical features are benign, recognition of this entity can pose diagnostic challenges. Here, we report a case of CNT with an unusual clinical presentation on the left second digit, and with atypical histological features including an infiltrative growth pattern, which could have been mistaken for features of malignancy.     

Morphologische Veränderungen in melanozytären Nävi durch exogene Faktoren

Malignant melanoma is the most important differential diagnosis in both clinical and histologic examination of melanocytic nevi. UV exposure with an erythemogenic dose and mechanical irritation of melanocytic nevi are able to induce reversible morphologic changes which simulate malignant melanoma. These changes are associated with an increased expression of HMB-45 antigen. In addition, an increased proliferation and repair activity is observed after UV exposure. The increased number of melanocytes located in suprabasal layers of the epidermis is accompanied by a change in keratinocyte adhesion molecule expression. The UV light is also able to influence the adhesive properties of melanocytes in vitro. Therefore, both keratinocyte-derived factors and direct influence of UV on the melanocyte system are probably responsible for the morphologic changes. While these exogenously evoked changes are reversible, the potential biologic relevance--especially a possible role in the initiation of the carcinogenesis cascade--requires clarification.