Considered a new category of epidermolysis bullosa classification.
Hyperkeratosis, epidermal atrophy, basal vacuolar change, numerous telangiectatic vessels, keratotic warty lesions, subepidermal bullae. Telangiectatic, sun-sensitive facial rash; stunted growth; prone to respiratory and GI infections. Dilation of dermal capillaries; mild perivascular lymph infiltrate; basal vacuolar change but does not usually result in pigment incontinence. Genodermatosis with progressive bone marrow failure, and classic triad: 1.
Mild hyperkeratosis, epidermal atrophy, prominent telangiectasia of superficial vessels and numerous melanophages. Clinical DDx: 1. Red-to-brownish, reticular patches with irregular borders and symmetrical distribution. Etiology unknown, but considered due to cumulative effect of sun exposure exacerbated possibly by fragrances in combination with genetic predisposition and lighter skin types. Variable telangiectasia and melanin incontinence; mild epidermal atrophy is sometimes present along with mild vacuolar changes.
Luger, in Rheumatology Sixth Edition , Key dermatologic signs of DM include heliotrope rash, Gottron papules, Gottron sign, poikiloderma atrophicans vasculare, periungual telangiectases, dystrophic cuticles, and calcinosis cutis. Involvement of the skin is essential in making the diagnosis of DM because characteristic cutaneous lesions belong to the diagnostic criteria for DM according to Bohan and Peter.
This group of patients has been referred to as having amyopathic DM DM sine myositis. Although a variety of skin manifestations occur in DM, none of the skin signs allows discrimination between idiopathic DM and the paraneoplastic form. One highly specific skin sign that can be regarded as the cutaneous hallmark feature of DM is the heliotrope rash Fig.
Gentlelase Pigment Peel
This often pruritic, sometimes burning, violaceous, confluent erythema resembles the color of the heliotrope, a pinkish purple flower that tracks the course of the sun. The heliotrope rash of DM has a characteristic distribution that especially involves the periorbital area. DM also often affects the extensor surfaces of the extremities, the knuckles, the dorsal aspects of the interphalangeal joints, and the periungual area of the fingers in a symmetric fashion see Fig.
The characteristic violaceous color and the periorbital distribution of the heliotrope rash distinguish DM from the butterfly rash malar rash of ACLE. It is also important to recognize that the skin lesions of DM, when affecting the fingers, tend to be located over the joints and not on the interphalangeal areas, as in patients with generalized ACLE compare Figs.
Heliotrope rash on the face in a man with dermatomyositis. Depending on the intensity of inflammation, the violaceous macules of DM may evolve into plaques that are often covered with a fine silvery scale, especially on the knees and elbows. When such lesions occur over the knuckles, in the interphalangeal joints, and in the periungual area, they are known as Gottron papules see Fig. Violaceous macules developing over the knuckles and the elbows or knees have been referred to as the Gottron sign Fig.
The intensely inflamed skin lesions of DM may develop into erosions, subepidermal blisters, and ulcers Fig. Recently, the presence of antimelanoma differentiation-associated gene 5 MDA5 has been reported to identify the presence of characteristic skin lesions and musculoskeletal, pulmonary, and prognostic features in Japanese patients with DM.
Mortality was independently associated with the presence of anti-MDA5 antibodies. Violaceous plaques on the knees of a patient with dermatomyositis Gottron sign.
Skin ulcerations in a young female with paraneoplastic dermatomyositis. Furthermore, the term poikiloderma atrophicans vasculare has been coined to describe patients with DM and a combination of violaceous erythema, hyperpigmentation, hypopigmentation, telangiectasia, and atrophy. In addition to the aforementioned skin signs, many patients with DM have prominent nail-fold telangiectases see Fig. Moreover, gingival telangiectases have recently been identified as a sign of juvenile DM.
Other, less frequently encountered cutaneous manifestations of DM include panniculitis leading to lipotrophy, papular and pustular lesions, and centripetal flagellate erythema. The term mechanic's hands see Fig.
Another subset has anti-PM-SCL antibodies, indicative of a polymyositis-scleroderma overlap syndrome. However, recent observations have questioned the specific association of the previously mentioned antibodies with mechanic's hands, thus suggesting that these skin manifestations may also occur in related systemic connective tissue disorders.
Calcinosis cutis can be an extremely painful and devastating skin manifestation of DM. It occurs most often at sites of friction and trauma, such as on the elbows, trochanters, knees, and fingers, and consists of hard, irregular nodules that eventually drain chalky material to the skin surface.
It is more prevalent in juvenile DM than in the adult form. Of note, calcinosis is also a key feature of the c alcinosis, R aynaud phenomenon, e sophageal hypomotility, s clerodactyly, t elangiectasia CREST syndrome and can be a manifestation of overlap syndromes. Histopathologic examination of a heliotrope erythema typically reveals an interface dermatitis with a sparse lymphocytic infiltrate, epidermal atrophy, vacuolar alteration of the basal keratinocytes, basement membrane degeneration, and interstitial mucin deposition. More inflamed lesions i. The differential diagnosis of DM includes SLE, psoriasis, atopic dermatitis, photoallergic and phototoxic drug eruption, contact dermatitis, cutaneous T-cell lymphoma, SSc, and trichinosis.
The skin ulcers in DM need to be distinguished from hemorrhagic-necrotizing vasculitis and from a DM-like eruption with painful ulcerations induced by hydroxyurea. Epidermolysis bullosa simplex EBS , one of the four major subgroups of epidermolysis bullosa if Kindler's syndrome is accepted as the fourth category , is a mechanobullous disorder characterized by intraepidermal cleavage, usually through the basal layer of cells.
Update on Cosmetic Dermatology : Notes from the Doc Talks
The localized Weber—Cockayne form OMIM is an autosomal dominant type in which friction-induced blisters are predominantly found on the hands and feet. Epidermolysis bullosa Dowling—Meara , formerly called epidermolysis bullosa herpetiformis OMIM , is characterized by the development in the first few months of life of serous or hemorrhagic blisters on the trunk, face, and extremities. Mucosal lesions and nail involvement may occur, as may keratoderma of the palms and soles. This variant has distinct ultrastructural features with clumping of tonofilaments. The lethal acantholytic type OMIM is an exceedingly rare autosomal recessive form.
Wart-like or punctate keratotic lesions also develop. In epidermolysis bullosa simplex the cleavage is so low in the epidermis that in routine paraffin sections the blister may appear to be a cell-poor subepidermal blister Fig. Immunofluorescence or immunoperoxidase techniques can also be used to confirm the level of the split. These methods will show that the bullous pemphigoid antigen, laminin, type IV collagen, and the LDA-1 antigen a component of the lamina densa are all present in the base of the bulla.
Monoclonal antibodies to specific proteins such as keratin 5, keratin 14, and plectin are available in some laboratories. Epidermolysis bullosa simplex. A Cleavage in the base of the epidermis. B Fragments of the basal keratinocytes are present in the floor of the blister. There is no dermal inflammation.
C There is vacuolar change within keratinocytes, prior to cleavage. In the localized Weber—Cockayne subtype, the level of the split is usually in the mid or upper epidermis Fig. Intracytoplasmic homogenizations and inclusions dyskeratosis are often present in individual keratinocytes.
The expression of plectin is absent or markedly reduced. A Epidermolysis bullosa simplex localized, Weber—Cockayne type. B The split is in the mid epidermis. Transient intraepidermal blisters with the appearances of epidermolysis bullosa simplex can be seen at the donor sites of skin grafts taken for use in patients who have had toxic epidermal necrolysis.
There is some perinuclear edema and subnuclear cytolysis of the basal cells but organelles are usually intact. In epidermolysis bullosa Dowling—Meara the cytolysis is preceded by aggregation and clumping of the tonofilaments which are attached to the hemidesmosomes at the dermoepidermal junction. Jeffrey E. Ming, John M. Graham Jr , in Stiehm's Immune Deficiencies , Xeroderma pigmentosum XP is characterized by sensitivity to sunlight with development of carcinoma at an early age, freckle-like lesions, photophobia, and poikiloderma.
Neurologic complications are frequent, including progressive mental retardation, ataxia, microcephaly, and hearing loss. T cell number may be decreased, due to decreased CD4 cells, , and DTH response can be impaired. Bendsoe, K. Svanberg, in Lasers for Medical Applications , There are many examples of skin conditions related to age, sun tanning and smoking, such as wrinkles, solar elastosis, pigment changes, ecstatic vessels and poikiloderma of civatte.
The wider acceptance and increased accessibility of aesthetic medicine to an ageing population have led to a booming demand for rejuvenation treatments. With growing awareness follow higher demands from the patients for more credible and effective anti-ageing results but with minimal down-time.
Earlier variants of treatment could often be complicated by scarring, hypopigmentation, infection, post-treatment pain and outbursts of facial herpes simplex infections. Congenital poikiloderma with traumatic bulla formation and progressive cutaneous atrophy. Br J Dermatol ; Hereditary acrokeratotic poikiloderma. Arch Dermatol ; Loss of kindlin-1, a human homolog of the Caenorhabditis elegans actin-extracellular-matrix linker protein UNC, causes Kindler syndrome.
Am J Hum Genet ; Kindler syndrome in native Americans from Panama: Report of 26 cases. Kindler syndrome with squamous cell carcinoma of the leg. Indian J Dermatol ; Kindler syndrome. Indian J Dermatol Venereol Leprol ; Kindler's syndrome: A case series of three Indian children. Int J Dermatol ; Kindler syndrome: A case report and proposal for clinical diagnostic criteria.
Acta Dermatovenerol Alp Panonica Adriat ; Related articles Kindler syndrome photosensitivity poikiloderma traumatic blistering. Access Statistics.