PubMed Health⌕ Search

PubMed · 7657436

Hypopigmented macules in acantholytic disorders.

Abstract

BACKGROUND: Widespread hypopigmented macules are rarely seen in heavily pigmented patients with Darier's disease. Previous hypotheses concerning the cause of decreased pigmentation suggest it is a postinflammatory phenomenon or that the hypomelanosis is evidence of subclinical acantholysis. PATIENTS: This report presents 2 patients: a new case of disseminated guttate leukoderma in a black patient with Darier's disease and the first such case in a patient with transient acantholytic dermatosis (Grover's disease). Direct immunofluorescence and electron-microscopic studies were carried out on lesional biopsies. OBSERVATIONS: Numerous small hypopigmented macules were observed in two black patients followed for acantholytic disorders. Three biopsies of the hypopigmented macules revealed acantholysis, while one showed only decreased melanin. Direct immunofluorescence studies were negative. Electron-microscopic studies of the leukodermic macules showed sparse melanocytes and melanosomes that were mostly pigmented stage IV melanosomes. CONCLUSIONS: Disseminated guttate leukoderma can occur in transient acantholytic dermatosis, as well as in Darier's disease. It is readily apparent on darkly pigmented skin because of contrast. The etiology of this phenomenon is still unknown.

Explore related subjects

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

M J Rowley, L T Nesbitt, P R Carrington, C G Espinoza. 1995. Hypopigmented macules in acantholytic disorders.. https://doi.org/10.1111/j.1365-4362.1995.tb04438.x

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

Changes in desmoglein 1 expression and subcellular localization in cultured keratinocytes subjected to anti-desmoglein 1 pemphigus autoimmunity.

The complexity of pemphigus acantholysis together with the weak expression of desmoglein 1 (Dsg1) in cultured keratinocytes have made the study on the pathogenic action of anti-Dsg1 antibodies quite difficult. The pathophysiology of the acantholytic phenomenon could depend on the reduction of Dsg1 adhesion function occurring after its massive internalization or decrease of its synthesis. Here, we have investigated this hypothesis by using sera of patients having antibodies against Dsg1 or monoclonal anti-Dsg1 antibodies to simulate pemphigus autoimmunity in Dsg1-rich keratinocytes. Similar to pemphigus foliaceus (PF) and vulgaris (PV) sera, monoclonal anti-Dsg1 antibodies induced transient internalization of Dsg1 and reduced the adhesion strength among keratinocytes. However, binding of IgG to Dsg1 did not determine its early depletion from the adhesion complexes but reduced the amount of Dsg1 found in the Triton X-100 soluble pool of proteins. Taken together, our results represent the first demonstration that anti-Dsg1 antibodies induce similar alterations on the subcellular distribution of Dsg1 irrespective of the disease where they come from. Furthermore, the present study provides insight into the mechanisms underlying epithelial blistering observed in the skin type of pemphigus.

Acantholysis↗

Pathogenic human monoclonal antibody against desmoglein 3.

Pemphigus vulgaris (PV) is a potentially fatal autoimmune mucocutaneous disease associated with production of IgG autoantibodies to desmoglein 3 (Dsg3), a 130-kDa epidermal cadherin protein. The binding of pathogenic antibody to Dsg3 on epidermal keratinocytes leads to loss of intercellular adhesion and results in intraepithelial blister formation. Here, we describe a human monoclonal antibody, PVMAB786, a Dsg3-specific IgG4 antibody, from an untreated patient with active PV. The antibody reacts with a 130-kDa protein on keratinocyte cell surfaces and recombinant Dsg3 protein, but not desmoglein 1 protein. PVMAB786 induces acantholysis in normal human skin and mucous membranes and induces a clinical and histological profile similar to human PV when injected into neonatal mice. PVMAB786 will be a valuable tool in identifying the role of Dsg3 in epithelial cell adherence and acantholysis, mechanisms of Dsg3 processing/presentation and V gene and isotype usage in PV pathogenesis.

Acantholysis↗