Childhood sclerodermatomyositis with generalized morphea

Systemic sclerosis (SS) and dermatomyositis (DM) are both multisystem disorders and share some common clinical features. We report here an 11 year-old girl whose disease showed a changing clinical pattern from juvenile systemic sclerosis (JSS) to slowly progressing juvenile dermatomyositis (JDM) and had associated generalized morphea. Serological studies revealed antinuclear antibodies (ANA) with a speckled pattern. Topoisomerase-I (Scl-70), U1 RNP (ribonucleoprotein), anti-Ro, anti-La and anti Jo-1 antibody tests were negative. Electromyography (EMG) was suggestive of primary muscle disease and histopathological findings indicated scleroderma. The patient fulfilled the American College Rheumatology (ACR) diagnostic criteria for JSS as well as Bohan and Peter criteria for JDM separately and hence, was diagnosed to have sclerodermatomyositis (SDM). Mixed connective tissue disease (MCTD) and antisynthetase antibody syndrome (ASS) which share same clinical features with SS and DM were excluded by immunological studies

Bullous Lupus Erythematosus Manifesting As Erythema Multiforme

Bullous SLE has a distinctive clinical, histopathologic and immunopathologic features that together constitute a unique bullous disease phenotype. We report a 33 year old female presenting with multiple tense vesicles and bullae on normal and erythematous skin over the body and oral erosions. Palms and extremities showed typical target lesions. She had consumed NSAIDs intermittently for joint pains. She was diagnosed as bullous erythema multiforme and started on oral prednisolone but lesions failed to heal. Patient recollected a history of low grade fever and a photosensitive rash in the past. Investigations revealed positive ANA with a peripheral pattern. A skin biopsy of a vesicle showed a subepidemal blisher. Perilesional direct immunofluorescence studies showed a linear deposition of IgG, IgA and fibrin along the basement membrane zone and perivascular deposition of IgG. Lapus band test showed a linear deposition of IgG, C3, IgM and fibrin at BMZ clinching the diagnosis of bullous lupus erythematosus

Acroangiodermatitis of mali: A rare vascular phenomenon

Acroangiodermatitis (synonym pseudo-Kaposi sarcoma) is an unusual, benign condition which clinically presents as purple-colored patches, plaques or nodules, mostly on the extensor surfaces of lower extremities in patients with chronic venous insufficiency and arteriovenous malformations. It resembles aggressive conditions like Kaposi′s sarcoma and requires histopathological examination for its diagnosis. We report two such cases of acroangiodermatitis. Histopathology of both the cases showed dilated capillaries in the dermis with extravasated red blood corpuscles (RBCs), hemosiderin deposits, and hyperplastic granulation tissue. Both were treated with oral antibiotics and topical steroids. The ulcers showed a good response within 2 months of treatment

Penicillamine-induced elastosis perforans serpiginosa with abnormal "lumpy-bumpy" elastic fibers in lesional and non-lesional skin

Four types of elastosis perforans serpiginosa (EPS) have been described in literature: 1) idiopathic EPS, 2) reactive perforating elastosis associated with connective tissue disorders, 3) in some instances of pseudoxanthoma elasticum (PXE), disease-specific calcified elastic tissue is extruded, producing a clinical picture indistinguishable from other types, may also be seen in patients undergoing hemodialysis and 4) EPS induced by long-term treatment with D-penicillamine is observed in patients suffering from Wilson′s disease. Long term D-penicillamine therapy causes an alteration in the dermal elastic tissue. D-penicillamine induced EPS has a distinctive histopathologic feature - serrated appearance of elastic fibers due to perpendicular budding from their surface giving a "lumpy-bumpy" look. D-penicillamine induced elastic fiber alteration may not always manifest clinically as EPS. We report a case of D-penicillamine induced widespread alteration in skin elastic tissue with distinct histopathologic features

Regenerative Treatments: Microneedling and PRP

The treatment of hair disorders is an important part of clinical dermatology, given the prevalence of the problem and the great impact on patients’ quality of life. Many new treatments have been investigated in recent years, such as platelet-rich plasma (PRP) and microneedling, which have emerged as promising regenerative therapies. These techniques were initially used by the medical community in fields other than dermatology; however, they have recently started to be popular among dermatologists for stimulating follicular regeneration. Several studies and case reports have demonstrated the therapeutic effectiveness of PRP and microneedling in hair loss disorders, but more evidence-based studies are needed to establish their real benefits. Our experience leads us to suggest using these techniques in association with existing hair growth-promoting therapies, or alone when there are contraindications to medical treatments